Pubmed abstracts for Protein-Protein Interaction search result :

Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
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Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
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Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
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Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
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Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
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Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
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Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
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Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
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Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
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Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.86031634
Thus CFTR , which has been demonstrated to interact with both Na+ and Cl channels in airway epithelium , modulates the function of renal ROMK 2 K+ channels . . 0.86031634^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :1.1990915
By using human airway epithelial cells , we show that CFTR associates with Na ( + ) / H ( + ) exchanger ( NHE ) type 3 kinase A regulatory protein ( E3KARP ) , an EBP50 / NHE regulatory factor ( NHERF ) related PDZ domain protein . 1.1990915^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
No difference in the Na+ , K+ ATPase activity in erythrocyte membranes from CF patients compared to control patients could be observed . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Nails of CF patients are here shown to have increased concentrations of Na , K and Cl , which will make the diagnosis of cystic fibrosis more reliable . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Most of the Ca efflux requires either external Na ( Na Ca exchange ) or external Ca plus in alkali metal ion ( Ca Ca exchange ; cf . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Erythrocytes from 15 patients with cystic fibrosis ( CF ) aged 8 mo to 22 y ( mean age 12 . 8 y ) were analyzed for Na+ , K ( + ) ATPase activity and sodium , potassium , and ATP concentrations . ^^^ Sodium concentrations and Na ( + ) K+ ratio of erythrocytes were statistically significantly lower in the CF patients [ 6 . 6 ( SD 1 . 9 ) versus 9 . 2 ( SD 1 . 1 ) mmol / L ( p less than 0 . 01 ) and 0 . 070 ( SD 0 . 023 ) versus 0 . 104 ( SD 0 . 016 ) mmol / L ( p less than 0 . 01 ) , respectively ] . ^^^ Intraerythrocyte sodium concentration and Na ( + ) K+ ratio were thus lower in relation to the recorded Na+ , K ( + ) ATPase activities in controls , indicating a change of the passive transmembrane movements of sodium ions in CF . ^^^ There was a rise of erythrocyte sodium and Na ( + ) K+ ratio despite unchanged Na+ , K ( + ) ATPase activity after regular infusion of a fat emulsion rich in essential fatty acids , inferring that an altered membrane composition by essential fatty acid deficiency could explain the low intracellular sodium concentration in CF . . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
The pH sensitive probe 2 ' , 7 ' bis ( 2 carboxyethyl ) 5 ( 6 ) carboxyfluorescein was used to measure intracellular pH ( pHi ) and test for Na ( + ) H+ exchange in single ciliated human nasal epithelial ( HNE ) cells from normal and cystic fibrosis ( CF ) subjects . ^^^ Utilizing the NH+4 loading technique to acid load cells , we found that pHi decreased from approximately 7 . 2 to approximately 6 . 6 ; subsequent alkalinization of normal and CF ciliated cells required Na+ and was independent of Cl but was blocked by amiloride ( 500 microM ) . ^^^ In the presence of extracellular Na+ , initial rates ( delta pHi / min ) of recovery from an acid load in normal and CF cells were 0 . 09 + / 0 . 03 and 0 . 12 + / 0 . 05 , respectively . ^^^ The results provide evidence in support of an amiloride sensitive PMA activated Na ( + ) H+ exchanger in ciliated HNE cells from normal and CF donors . . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Exposure of both normal and CF cells to ouabain resulted in decreased [ K ] and increased [ Na ] and [ Cl ] level . ^^^ Application of amiloride to the apical side of the cells resulted in a decrease of cellular [ Na ] in CF cells , whereas [ Na ] in control cells was not affected . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
This parameter , which does not directly represent noradrenaline release , but reflects release minus clearance , has been termed delta [ NA ] CF . ^^^ The effects of a number of pharmacological agents on the EJCs were examined both at 0 . 1 and 2 Hz , and the effects on the EJC response to 100 pulses at 2 Hz compared with that on the delta [ NA ] CF response . ^^^ However , clonidine and xylazine depressed at lower concentrations the EJC and delta [ NA ] CF responses to about the same extent ; these effects were partially or completely reversed by yohimbine . ^^^ Yohimbine or idazoxan did not affect the EJCs at 0 . 1 Hz but enhanced the EJC and delta [ NA ] CF responses to 100 pulses at 2 Hz to the same extent . ^^^ Under control conditions , both agents depressed the EJC but dramatically enhanced the delta [ NA ] CF response to 100 pulses at 2 Hz . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
The CFTR in all cases co localized with the Na+ , K ( + ) ATPase and the plasma membrane calcium ATPase , while the endoplasmic reticulum calcium ATPase and mitochondrial membrane markers were enriched at higher sucrose densities . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Additionally , we obtained the permeability coefficients for the anionic forms of CC and CF across EPC SUV , 0 . 6 . 10 ( 10 ) cm s 1 and 2 . 10 ( 10 ) cm s 1 , respectively , as compared to 2 . 5 . 10 ( 14 ) cm s 1 for the counterion Na+ ( Hauser , H , Oldani , D . and Phillips , M . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Cystic fibrosis ( CF ) is a lethal genetic disease resulting in a reduced Cl permeability , increased mucous sulphation , increased Na+ absorption and defective acidification of lysosomal vesicles . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
The utility of a transgenic murine model of cystic fibrosis ( CF ) lung disease will likely depend on whether the mouse ' s proximal airway epithelium is characterized by Na ( + ) and Cl ( ) conductive pathways comparable to those found in human airways . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Abnormal ion transport in airways of cystic fibrosis ( CF ) patients is manifest as a reduced Cl conductance and increased Na+ conductance . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Inner medullary cells may contain a class of Ba ( 2+ ) inhibitable , calmodulin dependent K+ conductive pathway which is activated in strongly hyperosmotic media by the operation of an inwardly directed Na ( + ) amino acid symport ( cf . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
In addition to the Cl channel abnormalities , Na+ absorption is increased in CF epithelia . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
However in contrast to control jejunum , net Na+ absorption in CF jejunum was higher in the IBMX ( 1 . 3 + / 0 . 5 mueq . cm 2 . h 1 ) compared with basal periods ( 0 . 6 + / 0 . 3 ; P less than 0 . 05 , paired t test ) . ^^^ In conclusion , CF jejunum failed to exhibit Cl secretion and also demonstrated abnormalities of Na+ absorption . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Cystic fibrosis ( CF ) airway epithelia exhibit raised transepithelial Na+ transport rates , as determined by open circuit isotope fluxes and estimates of the amiloride sensitive equivalent short circuit current ( Ieq ) . ^^^ To study the contribution of apical and basolateral membrane paths to raised Na+ transport in CF , CF nasal epithelial cultures were studied with double barreled Na ( + ) selective microelectrodes and the Ussing chamber technique . ^^^ It is concluded that 1 ) CF airway epithelia exhibit an increased apical membrane Na+ permeability , 2 ) acNa is regulated to a normal level in CF cells despite increased transcellular Na+ fluxes , 3 ) the abnormal increase in acNa in response to amiloride is dependent on luminal Na+ , 4 ) Na+ is transported across the basolateral membrane by a bumetanide sensitive cotransport mechanism , and 5 ) ouabain inhibits the basolateral Na ( + ) K ( + ) ATPase , causing slow dissipation of the chemical and electrical gradients across the cell membranes . . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Recent evidence suggests that abnormal epithelial transport of Na+ [ corrected ] and Cl may underlie pathogenesis , defects that may contribute to the markedly more negative transepithelial electrical potential differences ( PD ) recorded in CF airways compared with controls . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
The rise in SCC associated with Na ( + ) linked glucose absorption was greater in CF tissues than in controls , reflecting an enhanced active absorption of glucose . . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Control and CF saliva differed in that stimulation did not result in increased Na+ and Cl levels in CF saliva and that elevated levels of Ca2+ and PO 4 ( 2 ) were observed in CF . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Sweat Na+ , K+ , H+ , Cl , Ca2+ , and protein concentrations were reexamined with the use of three methods ; namely , in vitro sweat induction from isolated single sweat glands , intradermal methacholine induced sweating , and thermally induced sweating . [ Na+ ] and [ K+ ] in the primary sweat induced in vitro were nearly isotonic to the bath in both cystic fibrosis ( CF ) and control . ^^^ In CF the [ Na+ ] in both proximal ductal and skin surface sweat was always higher than 100 mmol / L . ^^^ Thus Na+ absorption never saturates , suggesting that the net NaCl absorption may increase with increasing sweat rate . pH of the primary sweat was 7 . 13 for CF and 7 . 29 for control , which corresponds to a [ HCO 3 ] of 7 . 3 and 10 . 5 mmol / L , respectively . [ Cl ] in the primary sweat was hypertonic ( 134 mmol / L for CF and 129 mmol / L for control ) to the bath ( 118 . 4 mmol / L ) . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Most NA units with characteristic frequencies ( CF ) above 1000 Hz respond to sound stimuli at CF as ' choppers ' , while units with CF ' s below 1000 Hz are ' primary like ' . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
The antimicrobial agents used were chloramphenicol ( C ) , gentamicin ( G ) , tetracycline ( T ) , ampicillin ( A ) , cephalothin ( Cf ) , trimethoprim sulfamethoxazole ( SXT ) , kanamycin ( K ) , nalidixic acid ( Na ) , streptomycin ( S ) , and furoxone ( F ) . 91 % of the salmonellae isolates contained resistance determinants , of which 89 . 6 % were resistant to more than one agent . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
In animals EFAD gives rise to symptoms similar to those in CF , e . g . defect Na transport , liver steatosis , increased caloric need , disturbed insulin release , increased bacterial colonization of airways , and decreased immune response . ^^^ Regular supplementation with fat emulsion ( Intralipid ) to CF patients normalizes the renal Na transport and prevents liver steatosis . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
The effect of the Na+ conductance blocker amiloride ( 10 ( 4 ) M ) on Vb was not significantly different in CF ( delta Vb = 26 . 4 + / 3 . 8 mV , n = 9 ) vs . normal ( delta Vb = 27 . 6 + / 2 . 5 mV , n = 30 ) cells . ( ABSTRACT TRUNCATED AT 250 WORDS ) . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
This was interpreted to show that at micromolar substrate , some low affinity , high turnover Na+ + K+ activity is possible , provided the Mg2+ concentration is low . ( 3 ) While small concentrations of K+ increased the hydrolysis rate ( cf . 2 ) they decreased the rate of ADP ATP exchange . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
We also investigated the effects of papaverine ( Pap ) , noradrenaline ( NA ) , acetylcholine ( ACh ) and absence of inorganic phosphate during Ca2+ free perfusion on coronary flow ( CF ) and cyclic nucleotide levels . ^^^ Ca2+ free perfusion alone and NA and absence of inorganic phosphate during the Ca2+ free perfusion period increased CF , whereas Pap and ACh decreased it . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
The level of Cai2+ achieved by this low Na+ maneuver was not significantly different in CF cells compared to normal cells . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Thus the diuretic efficiency was higher in CF for Na+ ( p = 0 . 02 ) , Cl ( p = 0 . 01 ) , K+ ( p = 0 . 07 ) , and volume ( p = 0 . 005 ) . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
The secretory cells of the fundus of sweat glands from cystic fibrosis ( CF ) patients had higher Na and Cl contents and showed more granule depletion , cellular disruption and dilated intercellular canaliculi than normal . ^^^ The elevated Na and fall in K in the fundus and raised Na and Cl in the coiled duct upon activation , were not observed in the CF glands in which no significant changes were detected . . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Na and Cl excretions in the urine decreased to very low levels in the CF adolescents during the run . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
In rats , clofibrate ( CF ) , 100 mg / kg / day , decreased TC and TG and increased HDL C , and nicotinic acid ( NA ) , 100 mg / kg / day , decreased and increased HDL C . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
The transepithelial permeability properties to Na , K , and Cl in microperfused segments of human eccrine sweat ducts from normal ( N ) subjects and patients with cystic fibrosis ( CF ) were examined . ^^^ The Vt responses to changes in Na concentration in the lumen and K concentration in the bath were similar in CF and N ducts and showed that the basolateral membrane of the duct is K permeable and the apical membrane ( in the absence of an anion shunt ) is an almost ideal Na electrode . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Under these conditions , the extracellular Na+ resonated from 10 to 20 ppm upfield relative to the intracellular Na+ , and no loss in the intensity of the extracellular Na+ resonance occurred due to the lability of dysprosium ( 3 ) tripolyphosphate ( cf . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
We studied the contribution of active Na+ absorption to the PD and attempted to increase the Cl permeability of CF epithelia . ^^^ The basal rate of Na+ absorption was raised in CF polyps compared with control tissues . ^^^ Whereas beta agonists induced Cl secretion in normal and atopic epithelia , beta agonists further increased the rate of Na+ absorption in CF epithelia without inducing Cl secretion . ^^^ We conclude that CF airway epithelia absorb Na+ at an accelerated rate . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Amiloride , a selective Na+ channel blocker , induced a larger apical membrane hyperpolarization and a greater increase in transepithelial resistance in CF epithelia . ^^^ Both reduced apical cell membrane Cl conductance and increased Na+ conductance appear to contribute to the abnormal function of respiratory epithelia of CF patients . . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Electron microprobe analysis of human labial gland secretory granules in cystic fibrosis . 10 ray microanalysis of freeze dried labial gland cryosections revealed that Na concentration was doubled and the Ca / S concentration ratio was decreased in secretory granules of labial glands from patients with cystic fibrosis ( CF ) when compared with glands from normal subjects . ^^^ These findings imply that mucous granules in labial saliva showed a CF related increase in Na and S content , and such changes would be expected to affect the rheology of the mucus after exocytosis . ^^^ In contrast with a previous study in human parotid glands , no evidence was found for CF related changes in cytoplasmic or nuclear Na , K , and Ca concentrations . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
We attempt to determine whether the decrease in Na+ reabsorption and the increase in K+ secretion in sweat of cystic fibrosis patients ( CF ) were associated with changes in glandular anaerobic metabolism evaluated by forehead sweat lactate excretion rate . 6 CF and 11 normal ( C ) children , 5 months to 14 years old , were exposed to external thermal load ( 45 degrees C ) . ^^^ The data showed that : 1 ) Na+ , K+ and Cl concentrations in CF are constant at any flow rate ( Qsw ) ; 2 ) In both groups the excretion rates of Na+ , K+ and Cl increased linearly with Qsw but the slopes in CF were significantly higher than in C ( p less than 0 . 001 ) ; 3 ) Lactate excretion rate increased with Qsw as in CF and C with the same slope . ^^^ We suggest that an increase in energy expenditure of Na+ K+ exchange and an active secretion of K+ by the duct could explain the normal energy metabolism that we observed in CF sweat glands . . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
The NA was characterized by estimation of the Michaelis constant , while the antigenicity of virus strains was assessed by induction of antibodies as detected in complement fixation ( CF ) tests . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Significantly lower relative Na concentrations and lower Na / K ratios were only found in CF fibroblasts ; the Na content of trisomy fibroblasts was normal . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
In the present study , we compared the kinetics of activation by Na , of red cell membrane Ca ATPase of cystic fibrosis ( CF ) patients and healthy volunteers ( controls ) . ^^^ On the other hand , the percent activation by Na of red cell Ca ATPase activity of the CF patients was approximately 40 % greater than that of the controls . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
In blocking uptake of [ 3H ] serotonin into cerebral cortical synaptosomes , methylaplysinopsin has a potency similar to that of imipramine ( Ki = 2 10 10 ( 7 ) mol / l cf . 5 10 10 ( 7 ) mol / l ) , but it is considerably less potent in blocking [ 3H ] NA uptake . 6 . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
For this purpose , the potential difference ( PD ) , which arises across the colonic mucosa from electrogenic sodium transport , was compared in CF and non CF subjects , together with the effect on this PD from bathing the mucosa with the specific Na+ channel blocker , amiloride . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Results indicate that in both normal and CF sweat ducts , Na+ is reabsorbed by an active process in which Cl follows passively while K+ is secreted . ^^^ Comparison of the differences in apparent electrochemical potential experienced by ions in the sweat duct during secretion indicates that Na+ is held out of the lumen of both groups of ducts against a large but similar gradient , but that Cl is held in the CF duct against a much larger gradient than in the normal duct . ^^^ These results indicate that the mechanism for Na+ reabsorption is not inhibited in the CF duct , but that the decreased NaCl transport in the defective duct is due to an abnormally low permeability to Cl . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
The ultrastructure of CF fibroblasts is basically normal . 10 ray microanalysis shows significantly lower Na and higher Ca levels in CF fibroblasts . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Cystic fibrosis ( CF ) patients may be at risk for heat illness because of their high sweat chloride and sodium concentrations ( [ Cl ] , [ Na+ ] ) , but it is not known if they can heat acclimate . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Two Na+ dependent intestinal transport mechanisms have been reported to be suppressed or missing in CF . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Vesicles with CF trapped inside were shown to be osmotically active and impermeable to Na+ and Co2+ ions . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
We characterized this abnormality further by measuring in the nasal epithelia of CF and normal subjects the concentration PD relationship for amiloride , an inhibitor of cell Na+ permeability , and PD responses to superfusion with solutions of different composition . ^^^ Na+ replacement by choline induced effects similar to those of amiloride , i . e . a greater depolarization in CF subjects . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
The resorption of Na+ and Cl across the duct of the human eccrine sweat gland is markedly decreased in individuals with cystic fibrosis ( CF ) . ^^^ No significant difference was observed between control and CF sweat glands in the freeze fracture morphology of the tight junctions of the duct , the segment where the net reabsorption of Na+ and Cl is abnormally decreased in CF . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Whereas the diuretic and natriuretic response to BFTZ was similar in groups AF , AV and CV , a supra additive response was observed in the CF group ( vs . group CV : delta urine flow rate : +78 % ; delta urinary Na excretion : +69 % ) . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Transepithelial Na+ absorption is increased two to three times in cystic fibrosis ( CF ) compared with normal ( NL ) airway epithelia . ^^^ Three Na+ channel related mechanisms may explain the increase in apical Na+ permeability in CF cells : increased number of channels , increased single channel conductance , and increased single channel open probability . ^^^ We compared the properties of Na ( + ) permeable channels in the apical membrane of confluent preparations of human NL and CF nasal epithelial cells cultured on permeable supports . ^^^ The same types of Na ( + ) permeable channels were recorded in CF and NL cells . ^^^ In excised patches , nonselective ( Na+ / K+ ) cation channels were recorded , and no differences between CF and NL were found in the properties , incidence , single channel conductance , and single channel open probability . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
When WT CFTR transfected cells were acidified ( pulsed with NH4Cl ) and incubated in Na ( + ) free ( N methyl D glucamine substitution ) solutions ( to block Na ( + ) dependent pHi regulatory mechanisms ) , pHi remained acidic ( pH approximately 6 . 5 ) until the cells were treated with 20 microM forskolin ( increases cellular [ cAMP ] ) ; pHi then increased toward ( but not completely to ) control level ( pHi 7 . 2 ) at a rate of 0 . 055 pH unit / min . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Hyperabsorption of Na+ and raised Ca ( 2+ ) mediated Cl secretion in nasal epithelia of CF mice . ^^^ We investigated the effect of homozygous genetic disruption of the murine cystic fibrosis transmembrane regulator ( CFTR ) gene on regulation of the rates of Na+ absorption and Cl secretion by nasal epithelia in cystic fibrosis ( CF ) mice . ^^^ The basal in vivo nasal potential difference ( PD ; 28 . 8 + / 1 . 8 mV , n = 10 ) and amiloride sensitive PD ( delta 13 . 8 + / 1 . 0 mV , n = 10 ) were raised in CF mice compared with controls [ 7 . 8 + / 0 . 8 mV , n = 14 ( basal ) ; delta 4 . 5 + / 0 . 7 mV , n = 14 ( amiloride ) ] , consistent with raised Na+ transport . ^^^ We conclude that CF murine nasal epithelia exhibit Na+ hyperabsorption , providing strong evidence for a regulatory link between CFTR and Na+ channel activity in airway epithelia . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Since the cAMP effects were dependent on Cl alone , not on Na , and since apical Cl conductance appears to be almost exclusively comprised of CFTR GCl , we surmise that this form of electrolyte absorption like secretion is also subject to acute control through CFTR GCl . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
We find that even repeated high doses can only partially ( 50 % ) correct the CF defect in Cl transport in vivo and do not correct the Na+ transport defect at all . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Cystic fibrosis ( CF ) is a recessive genetic disease with thickened airway secretions that result from abnormal airway epithelial ion transport , including defective cyclic AMP mediated Cl ( liquid ) secretion and excessive Na+ ( liquid ) absorption . ^^^ Amiloride aerosol inhibits excessive Na+ absorption , and pilot studies in adult patients with CF show improved biorheology and mucociliary clearance of airway secretions , as well as slowing of the decline in lung function . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Basal Na+ transport rates ( basal Ieq ) in CF airway epithelial sheets ( 78 . 5 + / 9 . 8 microA / cm2 ) were reduced to levels measured in normal epithelial sheets ( 30 . 0 + / 2 . 0 microA / cm2 ) by Ad 5 CBCFTR ( 36 . 9 + / 4 . 8 microA / cm2 ) , but not Ad 5 CMV lacZ ( 65 . 8 + / 6 . 1 microA / cm2 ) . ^^^ In conclusion , efficient transduction of CFTR corrects hyperabsorption of Na+ in primary CF airway epithelial cells and restores Ca ( 2+ ) mediated Cl secretion to levels observed in normal airway epithelial cells . ^^^ Dose response effects of Ad 5 CBCFTR on basal Na+ transport rates and Ca ( 2+ ) mediated Cl secretion suggest that the mechanism of regulation of these two ion transport functions by CFTR may be different . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Apical glucose elicited an electrogenic absorption of Na+ of identical magnitude in normal and CF jejuna . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
The cystic fibrosis transmembrane conductance regulator ( CFTR ) functions to regulate both Cl and Na+ conductive pathways ; however , the cellular mechanisms whereby CFTR acts as a conductance regulator are unknown . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
The pulmonary disease reflects mutations in the cystic fibrosis transmembrane conductance regulator ( CFTR ) gene , and associated abnormal epithelial ion transport , including defective cAMP mediated ( CFTR ) Cl secretion and an accelerated rate of basal Na+ transport . ^^^ Protocols are outlined to allow assessment of individual components of the electrolyte transport phenotype , i . e . , the magnitude of the basal and cAMP mediated ( CFTR ) Cl secretion , and the rate of Na+ transport . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Expression of complementary DNAs for rat epithelial Na+ channel ( rENaC ) alone in Madin Darby canine kidney ( MDCK ) epithelial cells generated large amiloride sensitive sodium currents that were stimulated by cAMP , whereas coexpression of human CFTR with rENaC generated smaller basal sodium currents that were inhibited by cAMP . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
However , a sustained and significant increase in NA efflux ( cf . basal efflux or handled controls ) was found when an unfamiliar conspecific was also present in the novel cage . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Re examination of all 239 sweat sodium values ( 80 . 9 + / 19 . 5 mmol / l ) in 80 newly diagnosed CF patients ( all : Na+ > 70 mmol / l ) revealed that 5 % of the values were below 50 mmol / l , the lowest sweat value obtained being 27 mmol / l . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
The availability of the newly cloned subunits ( alpha , beta , gamma ) of the epithelial Na+ channel ( ENaC ) permits molecular studies of the pathogenesis of the abnormal Na+ transport rates of cystic fibrosis ( CF ) airway epithelia . ^^^ These findings indicate that 1 ) Na+ transport defects in CF airways disease may be expressed in glandular acinar and ductal epithelium as well as superficial epithelium , and 2 ) the molecular pathogenesis of Na+ hyperabsorption in CF airways does not reflect increased levels of Na+ channel mRNAs , and probably number , but reflects an absence of the normal inhibitory regulation of Na+ channels by CF transmembrane conductance regulator proteins . . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Cl channel and Na ( + ) K ( + ) 2Cl cotransport activities were studied in various cystic fibrosis transmembrane conductance regulator ( CFTR ) expressing cells with the aim of assessing integrative patterns of regulation of Cl secretion . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
We now report an evaluation of the primary abnormalities in CF lung epithelia defective Cl secretion and Na hyperabsorption in xenografts following adenovirus mediated gene transfer . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
For comparison , the concentration of endogenous noradrenaline was monitored electrochemically by differential pulse amperometry with a carbon fibre microelectrode ; this signal is termed [ NA ] CF . [ NA ] alpha 2 and [ NA ] CF grew during the first 7 10 or 14 16 pulses , respectively , and then remained relatively constant throughout the stimulus train . ^^^ Cocaine caused [ NA ] alpha 2 and [ NA ] CF to continue to grow during the first 35 and 50 pulses , and enhanced their peak levels by 180 % and 320 % , respectively . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Our data suggest that AVP , via V 2 receptors , and the second messenger cAMP stimulate IscCl and that Cl secretion by mIMCD K 2 cells involves uptake of Cl across the basolateral membrane by Na ( + ) 2Cl ( ) K+ cotransport and Cl / HCO3 exchange and diffusion out of the cells across the apical membrane by cystic fibrosis transmembrane conductance regulator Cl channels . . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Aerosolized amiloride inhibits excessive Na+ absorption , and pilot studies in adult patients with CF show slowing of the disease associated decline in lung function . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
STUDY OBJECTIVE : It has been suggested that the defective cyclic AMP dependent Cl secretion and the increased Na+ absorption from airway epithelia in cystic fibrosis ( CF ) may lead to dehydrated secretions and impaired mucociliary clearance . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Cystic fibrosis ( CF ) airway epithelia exhibit defective transepithelial electrolyte transport : cAMP stimulated Cl secretion is abolished because of the loss of apical membrane cystic fibrosis transmembrane conductance regulator ( CFTR ) Cl channels , and amiloride sensitive Na+ absorption is increased two to threefold because of increased amiloride sensitive apical Na+ permeability . ^^^ Moreover , amiloride , which inhibits Na+ channels , failed to inhibit fluid absorption by CF epithelia . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
The electrochemical signal , which reflected the rise and fall in the NA concentration at the surface of the carbon fibre electrode following nerve stimulation ( termed delta [ NA ] CF ) , monitored on line and ' in real time ' , the changes in the NA concentration at the surface of the artery . ^^^ The total duration of the evoked EJC and delta [ NA ] CF responses indicated that the life time of the ATP and NA released by a single pulse , or by short trains ( 10 pulses ) at 50 Hz , was less than 100 ms or 3 sec , respectively . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Glucose and pyruvate concentrations in the CF declined , as did Na+ and Cl . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Mutations in the cystic fibrosis transmembrane conductance regulator gene ( CFTR ) manifest on the nasal epithelial surface of individuals with cystic fibrosis ( CF ) by Na+ hyperabsorption and diminished beta agonist induced Cl conductance , resulting in an abnormal bioelectric phenotype across the nasal epithelium . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Apart from the well described defect in adenosine 3 ' : 5 ' cyclic monophosphate ( cAMP ) dependent activation of Cl conductances in cystic fibrosis ( CF ) , an increased Na+ conductance has also been reported from transepithelial measurements . ^^^ The present experiments confirm the absence of a cAMP dependent Cl conductance in CF respiratory epithelial cells and support previous findings obtained in transepithelial and microelectrode studies which indicate an increased Na+ conductance in respiratory epithelial cells from CF patients . . ^^^ Reverse transcriptase polymerase chain reaction analysis of normal respiratory cells identified messenger RNA of both the cystic fibrosis transmembrane conductance regulator ( CFTR ) as well as the human epithelial Na+ channel ( hNaCh ) . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
We tested the hypothesis that the cystic fibrosis transmembrane conductance regulator ( CFTR ) directly affects epithelial Na+ channel activity by co incorporating into planar lipid bilayers immunopurified bovine tracheal CFTR and either heterologously expressed rat epithelial Na+ channel ( alpha , b eta , gamma rENaC ) or an immunopurified bovine renal Na+ channel protein complex . ^^^ CFTR also decreased the extent of elevation of the renal Na+ channel Po following PKA mediated phosphorylation . ^^^ Moreover , the presence of CFTR prohibited the inward rectification of the gating of this renal Na+ channel normally induced by PKA mediated phosphorylation , thus down regulating inward Na+ current . ^^^ This interaction between CFTR and Na+ channels occurs independently of whether or not wild type CFTR is conducting anions . ^^^ Our results indicate that CFTR can directly down regulate single Na+ channel activity , thus accounting , at least in part , for the observed differences in Na+ transport between normal and cystic fibrosis affected airway epithelia . . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Airway epithelial cells bearing mutations of the cystic fibrosis ( CF ) transmembrane conductance regulator ( CFTR ) possess an increased Na+ conductance along with their well described defect of cAMP dependent Cl conductance . ^^^ Currently it is not clear , how this occurs , and whether it is due to a CFTR control of epithelial Na+ conductances which might be defective in CF patients . ^^^ This may explain the higher Na+ conductance observed in airway epithelial cells of CF patients . . ^^^ Wild type but not deltaF 508 CFTR inhibits Na+ conductance when coexpressed in Xenopus oocytes . ^^^ In the present study , we have tried to identify possible interactions between both CFTR and the epithelial Na+ conductance by overexpressing respective cRNAs in Xenopus oocytes . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
By enhancing Cl secretion onto and blocking Na+ absorption from the airway surface , treatment with aerosolized uridine 5 ' triphosphate ( UTP ) plus amiloride may improve the rheology of airway secretions and enhance mucociliary clearance in patients with CF . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
In conclusion , this study has identified two antibacterial agents that also reduce the Na+ absorption found in CF . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
HCO 3 secretion involves a basolaterally placed Na ( + ) H+ exchanger and apical anion channel , most probably the cystic fibrosis transmembrane conductance regulator ( CFTR ) . ^^^ CFTR accounted for 70 % of HCO 3 secretion , while the Na ( + ) HCO 3 cotransporter accounted for 30 % . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Thus , like the apical Na+ channel and the H ( + ) K ( + ) adenosinetriphosphatase , CHIF is mineralocorticoid induced in the colon but constitutively expressed in kidney . . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
CFTR is required for cAMP inhibition of intestinal Na+ absorption in a cystic fibrosis mouse model . ^^^ Radioisotope flux measurements revealed that Na+ and Cl were absorbed equally across the cftr ( / ) jejunum under basal conditions . ^^^ In cftr ( + / + ) and cftr ( + / ) intestine , Na+ was absorbed at a similar rate , but net Cl absorption was reduced from that in cftr ( / ) intestine by an amount approximating the Isc . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
The characteristics of this secretory flux suggest that it is dependent upon the participation of an apical cystic fibrosis transmembrane conductance regulator ( CFTR ) like Cl channel and basolateral Na , K ATPase . ^^^ To test this hypothesis , we have immunolocalized the CFTR and Na , K ATPase proteins in intact cysts and in cyst epithelial cells cultured in vitro on permeable filter supports . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
CHIF exhibits significant homologies with proteins that are putatively regulatory ( phospholemman , gamma subunit of Na ( + ) K ( + ) ATPase , Mat 8 ) while it differs from the small conductance potassium channel Isk . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Three examples are discussed by which CFTR regulates other Cl channels , K+ channels , and epithelial Na+ channels . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Cystic fibrosis ( CF ) airway epithelial cells exhibit defective adenosine 3 ' , 5 ' cyclic monophosphate ( cAMP ) mediated chloride ( Cl ) secretion , abnormal hyperabsorption of sodium ( Na+ ) , and aberrant fluid transport . ^^^ Transduction of functional CFTR Cl channel activity reversed the abnormal Na+ hyperabsorption observed in CF cells in a dose dependent manner , suggesting that Na+ channel activity is downregulated by CFTR . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
CF airways demonstrated three discernible defects when compared with non CF , including 1 ) a lack of adenosine 3 ' , 5 ' cylic monophosphate ( cAMP ) inducible Cl secretion , 2 ) a fourfold higher basal fluid absorption rate , and 3 ) an altered regulation of fluid absorption in response to amiloride stimulated changes in Na+ transport . ^^^ The removal of apical Na+ from amiloride treated non CF xenografts was capable of ablating this amiloride induced increase in fluid absorption . ^^^ In light of the recent interactions demonstrated between CF transmembrane conductance regulator ( CFTR ) and the rat epithelial , amiloride sensitive Na+ channel , these findings implicate additional complexities between the Na+ conductance pathways and fluid transport in normal and CF proximal airways . ^^^ Such findings suggest that CFTR may also regulate amiloride insensitive Na+ channels . . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Increased Na+ transport rates are observed in CF airway epithelium , and recent studies suggest that this is due to an increase in Na+ channel open probability ( Po ) . ^^^ The Xenopus renal epithelial cell line , A 6 , expresses both cAMP activated 8 picosiemen ( pS ) Cl channels and amiloride sensitive 4 pS Na+ channels , and provides a model system for examining the interactions of CFTR and epithelial Na+ channels . ^^^ These data suggest that the regulation of Na+ channels by CFTR is not limited to respiratory epithelia and to epithelial cells in culture overexpressing CFTR and epithelial Na+ channels . . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
In intestinal bioelectric studies Colyte treated drinking water , compared with tap water , had no significant effect on basal short circuit current , cyclic AMP stimulated Cl secretion , Na+ coupled glucose absorption , or electrogenic Na+ absorption across intestinal sections from cftr ( + / + or + / ) mice . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
We demonstrate that cytosolic domains of human CFTR are sufficient to induce inhibition of rat epithelial Na+ currents ( rENaC ) when coexpressed in Xenopus oocytes and stimulated with 3 isobutyl 1 methylxanthine ( IBMX ) . ^^^ Enhanced Na+ transport as found in the airways of cystic fibrosis patients is probably due to a lack of CFTR dependent downregulation of ENaC . . ^^^ Inhibition of epithelial Na+ currents by intracellular domains of the cystic fibrosis transmembrane conductance regulator . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
All biliary epithelial cells expressed the membrane transporter Na+ K+ ATPase , restricted to the basolateral domain , and the apical transporters CFTR and MDR 1 . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Total ISC in CF cells was nearly completely inhibited by amiloride ( 92 + / 9 . 6 % ) , while in non CF tissue amiloride insensitive conductances mediated a considerable amount of the ISC ( 36 . 3 + / 6 . 1 % ) , indicating a lower activity of amiloride sensitive Na+ conductances in non CF cells . ^^^ Astonishingly , phenamil , a blocker which irreversibly blocks all epithelial Na+ channels hitherto described , inhibited the Na+ conductances of human nasal epithelium in a completely reversible way , but nevertheless with high affinity ( non CF : K1 / 2 = 12 . 5 + / 1 . 2 nM ; CF : K1 / 2 = 17 . 1 + / 1 . 1 nM ) . ^^^ From these findings , we conclude that the epithelial Na+ conductances of human CF nasal epithelium show modified regulation or are functionally different from those of other tissues . . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Patients with cystic fibrosis ( CF ) exhibit normal concentrations of sodium and chloride in spite of the disturbance of Cl and Na+ transport in epithelial cells . ^^^ To characterize compensatory mechanisms in the regulation of sodium homeostasis , erythrocytes of 13 CF patients were analysed for sodium lithium counter transport ( SLC ) , Na+ / K ( + ) ATPase activity and intracellular sodium content . ^^^ Red blood cells ' SLC and Na+ / K ( + ) ATPase activity were elevated in CF patients ( 381 + / 106 mumol / h / l RBCs vs 281 + / 64 ; p < 0 . 01 ) and ( 445 + / 129 mumol ATP mg prot / h vs 322 + / 84 , p < 0 . 01 ) . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
The initial increase by ATP ( 10 ( 4 ) M ) was significantly inhibited by a Cl ( ) channel inhibitor diphenylamine 2 carboxylate ( DPC , 5 10 10 ( 4 ) M ) and Cl ( ) substitution with gluconate in the bath solution , while a cystic fibrosis transmembrane regulator ( CFTR ) Cl ( ) channel inhibitor glibenclamide ( 10 ( 4 ) M ) , a Na ( + ) channel inhibitor amiloride ( 10 ( 4 ) M ) and a K ( + ) channel inhibitor quinidine ( 10 ( 4 ) M ) all failed to alter it . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Multiple dosing with recombinant adenoviral vectors containing the cystic fibrosis transmembrane conductance regulator ( CFTR ) cDNA to the nasal mucosa of cystic fibrosis ( CF ) transgenic mice reportedly results in only partial correction of the CF defect in chloride ( Cl ) secretion without normalizing sodium ( Na+ ) hyperabsorption , perhaps indicating inefficient gene transfer into the nasal airway epithelium in vivo . ^^^ No correction of either the Cl or Na+ transport defects were observed when 5 10 10 ( 9 ) IU of Ad2 / CFTR 5 were administered to the nasal passage of CF mice over a period of 5 20 min . ^^^ These results suggest that prolonging the contact between an adenoviral vector and the respiratory epithelium enhances the efficiency of gene transfer and can result in improved correction of the CF Na+ and Cl ion transport defects . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
AMP stimulates CFTR like Cl channels and inhibits amiloride sensitive Na+ channels in mouse CCD cells . ^^^ We conclude that , in addition to amiloride sensitive Na+ channels , CFTR like Cl channels are present in the apical membrane of confluent M 1 cells . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Because there are reports that electrogenic Na+ absorption is increased in colonic epithelia of cystic fibrosis ( CF ) subjects , we tested whether amiloride sensitive Na+ absorption was increased in the colonic epithelia of CF mice compared with normal mice on high or low Na+ diets . ^^^ However , when mice were placed on a low Na+ diet for 2 wk , all three intestinal regions from the CF mice exhibited a significant response to amiloride ( P < or = 0 . 05 ) . ^^^ Measurement of plasma aldosterone levels revealed that the CF mice on the low Na+ diet had significantly greater aldosterone levels than similarly treated controls [ 8 , 906 + / 1 , 039 ( n = 14 ) vs . 5 , 243 + / 1 , 410 pg / ml ( n = 14 ) , respectively ] . ^^^ If the presence of CF transmembrane conductance regulator ( CFTR ) down regulates Na+ absorption in the colonic tissue from normal mice , our data suggest that at least some CFTR may be colocalized with the Na+ channel . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Cystic fibrosis ( CF ) airway epithelia exhibit enhanced Na+ reabsorption in parallel with diminished Cl secretion . ^^^ These results indicate that CFTR can directly downregulate single Na+ channel activity , which may account for the observed differences between Na+ transport in normal and CF affected airway epithelia . ^^^ We tested the hypothesis that actin plays a role in the regulation of a cloned epithelial Na+ channel ( ENaC ) by the cystic fibrosis transmembrane conductance regulator ( CFTR ) . ^^^ Moreover , the presence of actin confers an enhanced modulatory ability of CFTR on Na+ channels . . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Abnormal regulation of ion channels by members of the ABC transport protein superfamily has been implicated in hyperinsulinemic hypoglycemia and in excessive Na+ absorption by airway epithelia in cystic fibrosis ( CF ) . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Thus , the direction and magnitude of baseline fluid movement depended on a balance between active absorption of Na+ and cAMP dependent secretion of Cl . 3 . 8 ( 4 Chlorophenylthio ) adenosine 3 ' , 5 ' cyclic monophosphate ( CPT cAMP ) , methacholine and luminal uridine 5 ' triphosphate ( UTP ) all induced or increased fluid secretion across non CF cultures . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
This study investigated the distribution ( apical / basolateral membrane ) of Na+ / H+ exchange in human nasal epithelial cell monolayers from cystic fibrosis ( CF ) and non CF individuals . ^^^ When cells were acid loaded ( NH4+ prepulse ) , subsequent alkalinization of non CF and CF cell monolayers required serosal Na+ and was blocked by serosal , but not mucosal , amiloride ( 500 microM ) . ^^^ In the presence of extracellular Na+ , initial rates ( delta pHi / min ) of recovery from an acid load in non CF and CF preparations were statistically identical ( approximately 0 . 08 ) . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Under some conditions when Na+ absorption across the apical membrane of principal cells in the CCD is stimulated and the apical membrane potential is depolarized the electrochemical gradient for Cl will support Cl absorption via CFTR Cl channels . ^^^ In addition to its function as a 3 ' , 5 ' cAMP activated Cl channel , CFTR may play a role in intracellular vesicle acidification , protein processing , protein trafficking , secretion of ATP and the regulation of the epithelial Na channel ( ENaC ) and the secretory K+ channel ( ROMK 2 ) which mediate Na+ and K+ transport , respectively , across the CCD . ^^^ Thus , CFTR may regulate Na+ and K+ excretion by the kidneys . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Furthermore , the murine CF intestinal tract also shows an inability to secrete HCO 3 , defective cAMP regulation of electroneutral NaCl absorption , and elevated electrogenic Na+ transport in the distal colon , as well as other ion transport perturbations . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
We characterized Na+ absorption in confluent monolayers of primary cultured epithelia derived from human nasal cystic fibrosis ( CF ) and non CF epithelium in modified Ussing chambers . ^^^ Amiloride sensitive Na+ channels in cells obtained from CF as well as from non CF patients showed properties different from all previously described epithelial Na+ channels ( ENaC ) . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
There was no difference in the electrogenic Na ( + ) coupled glucose absorption in the CF murine jejuna compared to jejuna from normal mice . ^^^ However , further studies are warranted to determine whether amiloride sensitive Na+ absorption is upregulated in the murine CF colon . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
At pH 5 . 5 ( untreated Rio Negro water ) , the four Rio Negro species tested ( piranha preta , Serrasalmus rhombeus ; piranha branca , Serrasalmus cf . holandi ; aracu , Leporinus fasciatus ; and pacu , Myleus sp . ) were at or near ion balance ; upon exposure to pH 3 . 5 , while Na+ and Cl loss rates became significant , they were relatively mild . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
We have compared responsiveness to secretin in pancreatic ducts isolated from healthy and diabetic Hartley guinea pigs and also have compared the expression of CFTR and Na+ , K ( + ) ATPase in these two groups , as the activities of these two proteins are essential for secretion of pancreatic juice . ^^^ The increases in cyclic AMP levels evoked by exposure to either 0 . 1 nM or 0 . 1 microM secretin were not significantly different in pancreatic ducts isolated from healthy and diabetic guinea pigs nor were levels of CFTR or Na+ , K ( + ) ATPase expression . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
This could explain the enhanced Na+ conductance found in CF . ^^^ Cl transport by cystic fibrosis transmembrane conductance regulator ( CFTR ) contributes to the inhibition of epithelial Na+ channels ( ENaCs ) in Xenopus oocytes co expressing CFTR and ENaC . 1 . ^^^ Epithelial Na+ channels ( ENaCs ) are inhibited by the cystic fibrosis transmembrane conductance regulator ( CFTR ) when CFTR is activated by protein kinase A . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
The raised nasal transepithelial potential difference ( PD ) in cystic fibrosis ( CF ) reflects accelerated active transport of Na+ , and is inhibited by topical administration of the Na+ channel blocker , amiloride . ^^^ These data suggest aerosolized benzamil is a candidate long duration Na+ channel blocker for CF . . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Cystic fibrosis ( CF ) airway cells , besides their well known defect in cAMP dependent Cl conductance , are characterized by an enhanced Na+ conductance . ^^^ In this study we have examined the Na+ conductance in human respiratory tract by measuring transepithelial voltage and resistance ( Vte , Rte ) and by assessing membrane voltages ( Vm ) of freshly isolated airway epithelial cells from CF and non CF patients . ^^^ This downregulation of epithelial Na+ channels is absent in CF airways , leading to hyperabsorption and to the characteristic increase in mucus viscosity . . ^^^ The amiloride inhibitable Na+ conductance is reduced by the cystic fibrosis transmembrane conductance regulator in normal but not in cystic fibrosis airways . ^^^ We conclude that cystic fibrosis transmembrane conductance regulator acts as a downregulator of epithelial Na+ channels in human airways . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Interrelationship between the Na+ / glucose cotransporter and CFTR in Caco 2 cells : relevance to cystic fibrosis . ^^^ Both the Na+ dependent glucose cotransporter ( SGLT 1 ) and the cystic fibrosis transmembrane conductance regulator ( CFTR ) modulate Na+ and fluid movement , although in opposite directions . ^^^ These results point to positive substrate cross regulation of SGLT 1 and CFTR and suggest that NaCl and glucose are important for not only Na+ absorption and fluid movement , but also for cAMP dependent Cl efflux , and glycoconjugate synthesis , functions that are known to be anomalous in cystic fibrosis . . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Elevated levels of Na and Cl in airway surface liquid may play a major role in the airway pathology of cystic fibrosis ( CF ) ( J . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
The ionic composition of CF ASL is still not entirely defined and has been speculated to be abnormally high or abnormally low in Na+ and Cl concentrations ; estimates of osmolarities have ranged from 200 ( hypo osmolar ) to 285 ( iso osmolar ) to > 300 meq / L ( hyper osmolar ) . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Microelectrode impalement of both non CF and CF spheroids revealed lumen positive transepithelial electrical potential differences ( PDs ) that were inhibited by amiloride , indicating that the spheroids were inflated due to amiloride sensitive Na+ absorption followed by water . ^^^ In response to hydrocortisone treatment , Na+ transport reflected by amiloride sensitive PD increased and more so in CF than in non CF spheres . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Ion transport proteins , which are modulated by NP and are thought to underlie the natriuretic and diuretic actions of NP , include : ( a ) calcium activated K+ channels ; ( b ) ATP sensitive K+ channels ; ( c ) inwardly rectifying K+ channels ; ( d ) outwardly rectifying K+ channels ; ( e ) L type Ca2+ channels ; ( f ) Cl channels including cystic fibrosis transmembrane conductance regulator Cl channels ; ( g ) Na+ K+ 2Cl co transporter ; ( h ) Na+ K+ ATPase ; ( 1 ) Na+ channels ; ( j ) stretch activated channels ; and ( k ) water channels . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
These results substantiate previously documented findings of elevated Na absorption in CF airways and also suggest that the slightly elevated [ Cl ] s found in this study of CF xenograft epithelia does not occur through a mechanism of decreased apical permeability to Cl . . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
In AL from non CF and CF samples , the ion concentrations were Na : 63 . 9 + / 7 . 6 , 79 . 7 + / 11 . 6 ; Cl : 64 . 9 + / 13 . 2 , 82 . 6 + / 15 . 7 ; Mg : 1 . 9 + / 0 . 3 , 2 . 2 + / 0 . 4 ; S : 4 . 9 + / 1 . 3 , 4 . 8 + / 0 . 5 ; K : 2 . 4 + / 0 . 5 , 3 . 2 + / 1 . 6 ; and Ca : 1 . 2 + / 0 . 3 , 2 . 6 + / 0 . 8 mmol / liter , respectively . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
As CF is also characterized by hyperabsorption of Na+ , we determined the effect of mucosal UTP on transepithelial Na+ transport in primary cultures of human bronchial epithelia ( HBE ) . ^^^ We conclude that increasing cellular Ca2+ induces a long term inhibition of transepithelial Na+ transport across normal and CF HBE at least partly due to downregulation of a basolateral membrane K+ conductance . ^^^ Thus UTP may have a dual therapeutic effect in CF airway : 1 ) stimulation of a Cl secretory response and 2 ) inhibition of Na+ transport . . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Analysis of the ion transport in CF airways and intestinal epithelium identified additional defects in Na+ transport . ^^^ It became clear recently that mutations of CFTR may also affect the activity of other membrane conductances including epithelial Na+ channels , KvLQT 1 K+ channels and aquaporins ( Fig . 7 ) . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
The cystic fibrosis transmembrane conductance regulator ( CFTR ) functions as a cAMP regulated Cl channel and as a regulator of other membrane conductances . cAMP dependent activation of CFTR inhibits epithelial Na+ channels ( ENaC ) . ^^^ The first nucleotide binding domain of the cystic fibrosis transmembrane conductance regulator is important for inhibition of the epithelial Na+ channel . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Cystic fibrosis ( CF ) is characterized by defective Cl and enhanced Na+ conductance , both due to malfunction of the cystic fibrosis transmembrane conductance regulator ( CFTR ) protein in airway epithelial cells . ^^^ Moreover , we tried to correlate differences in epithelial Cl and Na+ conductance with the level of CFTR mRNA expression and studied whether these properties correlate to the clinical phenotype of CF patients . ^^^ As expected , epithelial Na+ conductance was enhanced and CFTR Cl conductance was absent in airway cells from CF patients . ^^^ Lack of correlation between CFTR expression , CFTR Cl currents , amiloride sensitive Na+ conductance , and cystic fibrosis phenotype . ^^^ To that end , CFTR mRNA was determined by means of quantitative reverse transcriptase polymerase chain reaction ( RT PCR ) and cyclic adenosine monophosphate ( cAMP ) activated Cl and epithelial Na+ conductances were examined in airway epithelial cells using microelectrode techniques . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
We confirm and expand our previous observations that Na+ absorption in human nasal epithelium is the dominant ion transport process and that Cl secretion is detectable in both CF and non CF tissue . ^^^ We further give evidence that in human nasal CF and non CF epithelium Na+ absorption is mediated by epithelial Na+ channels ( ENaC ) that are either different from those of other epithelia or which exhibit altered regulation . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Epithelial Na+ channels ( ENaC ) are inhibited by the cystic fibrosis transmembrane conductance regulator ( CFTR ) upon activation by protein kinase A . ^^^ Antibodies to dynamin or ubiquitin enhanced alpha , beta , gamma rENaC whole cell Na+ conductance but did not interfere with inhibition of ENaC by CFTR . ^^^ Another mutant , beta T592M , T593A ENaC , also showed enhanced Na+ currents , which were down regulated by CFTR . ^^^ Cystic fibrosis transmembrane conductance regulator inhibits epithelial Na+ channels carrying Liddle ' s syndrome mutations . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Na+ and Cl currents were studied in primary cultures of human nasal epithelium derived from non cystic fibrosis ( non CF ) and cystic fibrosis ( CF ) patients . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Defective regulatory interactions between the cystic fibrosis conductance regulator ( CFTR ) and the epithelial sodium channel ( ENaC ) have been implicated in the elevated Na+ transport rates across cystic fibrosis airway epithelium . ^^^ To study the mechanisms of this downregulation we have measured amiloride inhibitable Na+ current ( Iamil ) in oocytes co expressing rat ENaC and human wild type CFTR . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
It has been hypothesized that lack of Cl ( ) secretion and possibly elevated Na ( + ) absorption contribute to the gut problems in CF neonates . ^^^ The neonatal distal colon exhibited a high rate of amiloride sensitive electrogenic Na ( + ) absorption , which did not differ between the normal and CF preparations . ^^^ The neonatal proximal colon and ileum also exhibited electrogenic Na ( + ) glucose cotransport , which was significantly greater in the normal compared with the CF ileum . ^^^ In addition , all three intestinal regions exhibited electrogenic Na ( + ) alanine cotransport , which was significantly reduced in two of the regions of the CF neonatal intestine . ^^^ It is speculated that : 1 ) the reduced rate of Na ( + ) nutrient cotransport in the CF intestine contributes to the lower rate of growth in CF pups , whereas 2 ) the elevated electrogenic Na ( + ) absorption in the neonatal intestine , coupled with an inability to secrete Cl ( ) , contributes to the intestinal obstruction in the CF pups . . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Cystic fibrosis ( CF ) airway epithelial cells have a reduced cAMP dependent Cl ( ) conductance channel ( CFTR ) function but an increased level of amiloride sensitive Na ( + ) channel ( ENaC ) activity . ^^^ Treatment of JME / CF 15 cells with rhHGF at concentrations of 100 ng / ml and above was found to dramatically decrease the activity of amiloride sensitive Na ( + ) transport . ^^^ These observations are consistent with the possible application of an extracellular signalling molecule , such as the cytokine HGF , to reduce the abnormally high activity of amiloride sensitive Na ( + ) ion channels observed in CF airway cells . . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Because the cystic fibrosis transmembrane conductance regulator ( CFTR ) also functions as a downregulator of epithelial Na+ channels ( ENaC ) , enhanced Na+ conductance was found in the airways of CF patients . ^^^ Here , we examined whether enhanced epithelial Na+ conductance is also present in the colonic epithelium of CF patients and examined the underlying mechanisms . ^^^ Therefore , enhanced epithelial Na+ conductance is present in the CF colon and is probably due to missing downregulation by CFTR . . ^^^ CFTR mediated inhibition of epithelial Na+ conductance in human colon is defective in cystic fibrosis . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
PBA reduced CFTR Cl ( ) currents across the apical membrane but had no effect on Na ( + ) K ( + ) ATPase activity in the basolateral membrane . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Besides the well documented short term effect of AVP on chloride transport , these results provide evidence that in RCCD ( 1 ) cells , AVP induces a delayed increase in transepithelial net chloride secretion that is mediated by a Na ( + ) / K ( + ) / 2Cl ( ) co transporter and CFTR . . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
We recently showed that the COOH terminus of the cystic fibrosis transmembrane conductance regulator associates with the submembranous scaffolding protein EBP 50 ( ERM binding phosphoprotein 50 kD ; also called Na ( + ) / H ( + ) exchanger regulatory factor ) . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Activation of the epithelial Na+ channel ( ENaC ) requires CFTR Cl channel function . ^^^ The only frequently cited explanation for this hypertransport is that the activity of an epithelial Na+ channel ( ENaC ) is inversely related to the activity of the CFTR Cl channel . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
CFTR upregulates the expression of the basolateral Na ( + ) K ( + ) 2Cl ( ) cotransporter in cultured pancreatic duct cells . ^^^ The purpose of the current experiments was 1 ) to assess basolateral Na ( + ) K ( + ) 2Cl ( ) cotransporter ( NKCC 1 ) expression and 2 ) to ascertain the role of cystic fibrosis transmembrane conductance regulator ( CFTR ) in the regulation of this transporter in a prototypical pancreatic duct epithelial cell line . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Sodium channel inhibitors block the enhanced Na+ reabsorption in cystic fibrosis ( CF ) . ^^^ Amiloride and loperamide reduced the PD in cftr ( tm1HGU ) CF mice ( deltaPD 13 mV & 15 mV respectively ) suggesting Na+ blockade . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Gating of amiloride sensitive Na ( + ) channels : subunit subunit interactions and inhibition by the cystic fibrosis transmembrane conductance regulator . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
The CFTR mediated anion secretion is transient and is followed , in time , by a verapamil and Ba ( + ) sensitive anion secretion or cation absorption and , finally , by Na+ reabsorption via epithelial Na+ channels ( ENaC ) . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
CFTR inhibits epithelial Na ( + ) channel ( ENaC ) currents in many epithelial and nonepithelial cells . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
These findings suggest that cultured ovine vas deferens and epididymis cells absorb Na ( + ) via amiloride sensitive epithelial Na ( + ) channels ( ENaC ) and secrete Cl ( ) and HCO ( ) ( 3 ) via apical cystic fibrosis transmembrane conductance regulator ( CFTR ) Cl ( ) channels . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
We retrospectively reviewed sweat test results reported to a neonatal screening program for CF with respect to completeness of reported results and the values recorded for sweat chloride ( Cl ( ) ) and sodium ( Na ( + ) ) concentrations and the Cl ( ) : Na ( + ) ratio in screened infants . ^^^ The Cl ( ) : Na ( + ) ratio does not appear useful in establishing a diagnosis of CF in infants . . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Whole cell macroscopic sodium currents revealed that wild type ( wt ) alphabetagamma rENaC induced Na ( + ) current was inhibited by co expression of CFTR , and further inhibited when CFTR was activated with a cAMP raising mixture ( CKT ) . ^^^ Interestingly , down regulation of rENaC by activated CFTR was Cl ( ) dependent , while stimulation of CFTR by rENaC was not dependent on either cytoplasmic Na ( + ) or a depolarized membrane potential . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
These findings have been clearly demonstrated by Ussing chamber experiments in colon epithelium biopsies of CF and normal individuals : Colonic Cl secretion in CF patients is variable and reflects the genotype ; a complete defect of CFTR is paralleled by the absence of Cl secretion and unmasks Ca ( 2+ ) regulated K+ channels in the luminal membrane ; overabsorption of Na+ in CF reflects the absence of ENaC inhibition by CFTR ; and the functional status of CF colon can be mimicked by the complete suppression of cAMP stimulation in enterocytes of healthy individuals . . ^^^ Activation of CFTR leads to coactivation of basolateral KVLQT 1 type K+ channels and inhibition of luminal Na+ channels ( ENaC ) . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
The cystic fibrosis transmembrane conductance regulator ( CFTR ) is essential for epithelial electrolyte transport and has been shown to be a regulator of epithelial Na ( + ) , K ( + ) , and Cl ( ) channels . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
The aims of this study were to investigate ( a ) if renal Na ( + ) handling was normal in Cftr ( tm2cam ) delta F 508 cystic fibrosis mice , ( b ) whether adaptation to dietary salt depletion was preserved and ( c ) whether Cftr ( tm2cam ) delta F 508 mice exhibited enhanced amiloride sensitive Na ( + ) absorption . ^^^ In Na ( + ) replete animals ( maintained on a 0 . 32 % NaCl diet ) given a 150 mM NaCl i . v . maintenance infusion , there was no difference in fractional Na ( + ) excretion ( FE ( Na ) ) between wild type ( 0 . 42 + / 0 . 06 % , n = 12 ) and Cftr ( tm2cam ) delta F 508 mice ( 0 . 47 + / 0 . 13 % , n = 7 ) . ^^^ Amiloride infusion significantly increased FE ( Na ) in both wild type ( 3 . 14 + / 0 . 83 % , n = 6 ) and Cftr ( tm2cam ) delta F 508 mice ( 3 . 47 + / 0 . 63 % , n = 9 ) , though with no significant difference between genotypes . ^^^ A 14 day dietary salt restriction ( animals maintained on a 0 . 03 % NaCl diet ) and maintenance infusion with a 15 mM NaCl vehicle caused a reduction in FE ( Na ) to 0 . 14 + / 0 . 05 % , n = 8 in wild type mice and 0 . 14 + / 0 . 04 % , n = 8 in Cftr ( tm2cam ) delta F 508 mice . ^^^ Treatment of salt restricted mice with amiloride resulted in a blunted natriuresis in both wild type mice ( FE ( Na ) = 1 . 10 + / 0 . 16 % , n = 7 ) and Cftr ( tm2cam ) delta F 508 mice ( FE ( Na ) = 1 . 97 + / 0 . 29 % , n = 9 ) . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
In the branchial epithelium , Na ( + ) / H ( + ) exchangers ( both NHE 2 and NHE 3 like isoforms ) , a cystic fibrosis transmembrane regulator ( CFTR ) like anion channel , a vacuolar type H ( + ) ATPase ( 5 ATPase ) and carbonic anhydrase immunoreactivity are associated with the apical crypt region of MR cells . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Evidence of absorptive or secretory ion transport in different respiratory regions of the mouse was sought by assessing the regional distribution of alpha , beta , and gamma epithelial sodium channel ( ENaC ; Na ( + ) absorptive ) , cystic fibrosis transmembrane conductor regulator ( CFTR ) , and Na ( + ) K ( + ) 2Cl ( ) cotransporter mRNAs . ^^^ The low abundance of CFTR mRNA throughout murine pulmonary epithelium is consistent with functional data that attributes Cl ( ) secretion predominantly to an alternative Cl ( ) channel . alpha ENaC as the only mRNA found in all regions of airway epithelia is consistent with the alpha subunit as requisite for Na ( + ) absorption , and the increased expression of alpha , beta , and gamma ENaC in distal airways suggests a greater absorptive capability in this region . . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Therefore , it is unclear what is pathophysiologically more important for the development of CF lung disease , the impaired secretion of Cl or the enhanced reabsorption of Na+ and consecutive hyperabsorption of electrolytes . ^^^ Apart from its secretory function , CFTR also has a task in regulating the reabsorption of electrolytes by controlling the activity of the epithelial Na+ channel , ENaC . ^^^ Control of epithelial Na+ conductance by the cystic fibrosis transmembrane conductance regulator . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
CFTR Mediated anion conductance regulates Na ( + ) K ( + ) pump activity in Calu 3 human airway cells . ^^^ We studied the role of CFTR in the Na ( + ) K ( + ) pump activity of Calu 3 human airway cells . ^^^ The Na ( + ) K ( + ) pump activity was diminished by a selective CFTR blocker ( glybenclamide ) or nonspecific Cl ( ) channel inhibitors ( NPPB and DPC ) but not by outwardly rectifying Cl ( ) channel blockers ( DNDS , DIDS ) . ^^^ Augmentation of anion conductance by 8 bromo cyclic AMP ( 8Br cAMP , 1 mM ) potentiated the Na ( + ) K ( + ) pump activity that was reduced by blocking CFTR or by the replacement of Cl ( ) with gluconate , a less membrane permeant anion . ^^^ The Na ( + ) K ( + ) pump activity was unaffected by the replacement of Cl ( ) with NO ( ) ( 3 ) that has equal permeability through the CFTR . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
In DeltaF HBE , baseline 1 ( sc ) was 33 . 8 + / 1 . 2 microA / cm ( 2 ) ( n = 200 ) , and amiloride reduced this by 29 . 6 + / 1 . 5 microA / cm ( 2 ) ( n = 116 ) , demonstrating the characteristic hyperabsorption of Na ( + ) associated with cystic fibrosis ( CF ) . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
CF tissues showed typical alterations of short circuit currents with enhanced amiloride sensitive Na ( + ) conductance and defective cAMP mediated Cl ( ) conductance . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Suppression of CFTR mediated Cl ( ) secretion by enhanced expression of epithelial Na ( + ) channels in mouse endometrial epithelium . ^^^ The present study investigated the effect of enhanced expression of epithelial Na ( + ) channels ( ENaC ) on the cystic fibrosis transmembrane conductance regulator ( CFTR ) mediated Cl ( ) secretion in the mouse endometrium using the short circuit current technique . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
More than 900 different mutations in the CFTR gene are the cause for defective transport of Cl ( ) and Na ( + ) and impaired secretion or absorption of electrolytes in cystic fibrosis . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Several studies have demonstrated that CFTR does not only transport Cl but also secretes ATP and , thus , controls other conductances such as Na+ ( ENaC ) and K+ ( ROMK 2 ) channels , especially in CCD . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
When produced in Xenopus oocytes , both proteins exhibited Na ( + ) dependent cib type functional activities . hCNT 3 was electrogenic , and a sigmoidal dependence of uridine influx on Na ( + ) concentration indicated a Na ( + ) : uridine coupling ratio of at least 2 : 1 for both hCNT 3 and mCNT 3 ( cf 1 : 1 for hCNT1 / 2 ) . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Both cell preparations express mRNA for CFTR , Na ( + ) K ( + ) 2Cl ( ) cotransporter ( NKCC 1 ) , GCC and PKG 2 as detected by RT PCR . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Cystic fibrosis ( CF ) airway epithelia are characterized by enhanced Na ( + ) absorption probably due to a lack of downregulation of epithelial Na ( + ) channels by mutant CF transmembrane conductance regulator . ^^^ In this study we examined the role of mucosal ATP and UTP in regulating Na ( + ) transport in native human upper airway tissues from patients with 16 patients with CF and 32 non CF control subjects . ^^^ Both basal Na ( + ) transport and nucleotide dependent inhibition of amiloride sensitive 1 ( SC ) were significantly enhanced in CF airways compared with non CF . ^^^ These data demonstrate sustained inhibition of Na ( + ) transport in non CF and CF airways by mucosal ATP and UTP and suggest that this effect is mediated by an increase of intracellular Ca ( 2+ ) . ^^^ Because ATP and UTP inhibit Na ( + ) absorption and stimulate Cl ( ) secretion simultaneously , extracellular nucleotides could have a dual therapeutic effect , counteracting the ion transport defect in CF lung disease . . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
We assessed concentrations of eosinophil cationic protein ( ECP ) , eosinophil protein 10 ( EPX ) , major basic protein ( MBP ) and ions ( Na+ , Cl , Ca2+ , Mg2+ ) in sputum samples of 29 children with CF as well as in 10 controls with bronchial asthma . ^^^ Patients with CF demonstrated significantly higher levels of ECP , Na+ , Cl and Ca2+ levels than asthmatics ( P < 0 . 04 , P < 0 . 0001 , P < 0 . 0001 , P < 0 . 02 ) . ^^^ In CF , eosinophil granule proteins correlated significantly with Ca2+ and Mg2+ concentrations ( ECP , P < 0 . 0001 , r = 0 . 65 , P < 0 . 0001 , r = 0 . 66 ; MBP , P < 0 . 03 , r = 0 . 41 , P < 0 . 03 , r = 0 . 42 ) , furthermore inversely with Cl concentrations ( ECP , P < 0 . 0003 , r = 0 . 63 ; EPX , P < 0 . 02 , r = 0 . 45 ; MBP , P < 0 . 03 , r = 0 . 41 ) but not with Na+ levels . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
The dDAVP dependent 1 ( sc ) measured under apical Na ( + ) free condition was reduced by Cl ( ) channel blockers with a profile ( NPPB > glibenclamide > DIDS ) , similar to that for rat CFTR . dDAVP stimulated the apical to basal ( 36 ) Cl ( ) flux and to a lesser extent the basal to apical ( 36 ) Cl ( ) flux under open circuit condition in all three cultured cell lines . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
The Na ( + ) / H ( + ) exchanger regulatory factor ( NHERF ) binds to the cytoplasmic tail of CFTR through either of its two PDZ ( PDZ 1 and PDZ 2 ) domains . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
These ion transport systems are defective in cystic fibrosis ( CF ) : Cl secretion by CFTR is impaired and Na+ absorption by ENaC is dramatically increased . ^^^ Measurements of transepithelial short circuit current ( ISC ) showed that NO has no inhibitory potency against amiloride sensitive nasal ENaC ( nENaC ) or amiloride insensitive Na+ absorbing mechanisms in non CF and CF epithelia . ^^^ Nitric oxide ( NO ) has been reported to activate Cl secretion via the cystic fibrosis transmembrane conductance regulator ( CFTR ) and inhibit epithelial Na+ absorption mediated by amiloride sensitive epithelial Na+ channels ( ENaC ) . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
We found that uptake of ( 22 ) Na ( + ) through ENaC is modulated by activation of CFTR in oocytes , coexpressing CFTR and ENaC , depending on extracellular chloride concentration . ^^^ We conclude that the observed modulation of ( 22 ) Na ( + ) uptake by activated CFTR is due to the effect of CFTR mediated chloride conductance on the membrane potential . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
PCs in SCA 1 mice had Na ( + ) spikes , Ca ( 2+ ) spikes , climbing fiber ( CF ) electrical responses , parallel fiber ( PF ) electrical responses , and metabotropic glutamate receptor ( mGluR ) mediated , PF evoked Ca ( 2+ ) release from intracellular stores that were qualitatively similar to those recorded from WT mice . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
The PDZ 1 domain of the Na ( + ) / H ( + ) exchanger regulatory factor ( NHERF ) binds with nanomolar affinity to the carboxyl terminal sequence QDTRL of the cystic fibrosis transmembrane conductance regulator ( CFTR ) and plays a central role in the cellular localization and physiological regulation of this chloride channel . ^^^ Structural basis of the Na+ / H+ exchanger regulatory factor PDZ 1 interaction with the carboxyl terminal region of the cystic fibrosis transmembrane conductance regulator . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Renal Na ( + ) clearance was similar in wild type ( 1 . 4 + / 0 . 3 microl min ( 1 ) , number of animals , N = 12 ) and CF mice ( 1 . 6 + / 0 . 4 microl min ( 1 ) , N = 7 ) under control conditions . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
In contrast , cystic fibrosis transmembrane conductance regulator or the Na+ pump beta subunit were unchanged . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Pure , highly explosive CF ( 3 ) C ( O ) OOC ( O ) CF ( 3 ) is prepared for the first time by low temperature reaction between CF ( 3 ) C ( O ) Cl and Na ( 2 ) O ( 2 ) . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
We conclude that water freely permeates porcine tracheal epithelium and that absorption of liquid is normally driven by active transcellular Na ( + ) transport and does not require the CFTR . . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Mechanisms of the inhibition of epithelial Na ( + ) channels by CFTR and purinergic stimulation . ^^^ The epithelial Na+ channel ENaC is inhibited when the cystic fibrosis transmembrane conductance regulator ( CFTR ) coexpressed in the same cell is activated by the cyclic adenosine monophosphate ( cAMP ) dependent pathway . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
CFTR is in charge of proper secretion and absorption of electrolytes , and thus the disease is characterized by defective epithelial Cl ( ) secretion and enhanced Na ( + ) absorption . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Sum frequency generation ( SFG ) vibrational spectra of D ( 2 ) O and / or acetonitrile ( CH ( 3 ) CN ) on a Pt ( 111 ) single crystal electrode were obtained as a function of applied potential in a 5 mol % water / acetonitrile mixed solvent with different 0 . 1 molar MSO ( 3 ) CF ( 3 ) salts ( M = H ( + ) , Li ( + ) , Na ( + ) , K ( + ) , and Cs ( + ) ) . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
CHIF , a member of the FXYD protein family , is a regulator of Na , K ATPase distinct from the gamma subunit . ^^^ Here , we report that , similarly to gamma ( a ) and gamma ( b ) splice variants , the FXYD protein CHIF ( corticosteroid induced factor ) is a type 1 membrane protein which is associated with NKA in renal tissue , and modulates the Na , K pump transport when expressed in Xenopus oocytes . ^^^ In contrast to gamma ( a ) and gamma ( b ) , which both decrease the apparent Na+ affinity of the Na , K pump , CHIF significantly increases the Na+ affinity and decreases the apparent K+ affinity due to an increased Na+ competition at external binding sites . ^^^ These data document that CHIF is a new tissue specific regulator of NKA which probably plays a crucial role in aldosterone responsive tissues responsible for the maintenance of body Na+ and K+ homeostasis . . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NOF ( 0 . 1 50 microM ) produced a small but not concentration related vasodilation of the NA preconstricted ( i . e . `` high tone ' ' ) perfused rat mesentery preparation ( cf . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
The aim of this study was to determine the effects of a single exercise bout on luminal Cl ( ) and Na ( + ) conductance in the respiratory epithelium of patients with cystic fibrosis ( CF ) . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
To study the potential influence of cystic fibrosis conductance regulator ( CFTR ) on intracellular pH regulation during apoptosis induction , we used PS 120 Chinese hamster lung fibroblasts devoid of the Na ( + ) / H ( + ) exchanger ( NHE 1 isoform ) transfected with constructs , allowing the expression of CFTR and / or NHE 1 . ^^^ In PS 120 NHE1 cells , the transfection with CFTR did not modify the percentage of apoptotic cells after 40 h ( control : 19 + / 3 % , n = 8 ; CFTR : 17 + / 1 % , n = 8 ) , indicating that blocking intracellular acidification by overexpressing the Na ( + ) / H ( + ) exchanger inhibited the enhancement of apoptosis induced by CFTR . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Here , we investigated the effects of deamino 8 D arginine vasopressin ( dDAVP ) and isoproterenol ( ISO ) on NaCl transport in primary cultured CCDs microdissected from normal [ CFTR ( + / + ) ] and CFTR knockout [ CFTR ( / ) ] mice . dDAVP stimulated the benzamyl amiloride ( BAm ) sensitive transport of Na ( + ) assessed by the short circuit current ( 1 ( sc ) ) method in both CFTR ( + / + ) and CFTR ( / ) CCDs to a very similar degree . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Cystic fibrosis ( CF ) is characterized by impaired Cl ( ) secretion and increased Na ( + ) reabsorption in several tissues including respiratory epithelium . ^^^ In summary , enhanced expression of h SGK 1 in epithelial cells of CF lung tissue may be a novel pathophysiological factor contributing to increased Na ( + ) channel activity and thus to increased Na ( + ) transport in CF . . ^^^ Effects of the serine / threonine kinase SGK 1 on the epithelial Na ( + ) channel ( ENaC ) and CFTR : implications for cystic fibrosis . ^^^ In addition CFTR mediated currents were also stimulated . h SGK 1 accelerated the expression of the amiloride sensitive Na ( + ) current in Xenopus oocytes paralleled by increased ENaC protein abundance in the oocyte membrane , an effect which was reversed by a h SGK 1 ( K127R ) mutation lacking the ATP binding site . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
In the kidney the epithelial Na+ channel ( ENaC ) is co expressed with the sulfonylurea receptor ( SUR ) , an ABC protein that shares a high degree of homology with the cystic fibrosis transmembrane conductance regulator ( CFTR ) and reportedly modifies ENaC in various preparations . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Focal injections of horseradish peroxidase or neurobiotin were placed either in the NA or in the cochlear nucleus magnocellularis , labeling small groups of auditory nerve fibers of known characteristic frequency ( CF ) from 0 . 25 to 9 . 6 kHz . ^^^ Auditory nerve fibers of a given CF always entered the nucleus angularis within a well restricted area and then traveled along their isofrequency band within the NA while branching off terminals . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Forskolin treatment , endogenous production of cAMP by activation of coexpressed beta adrenergic receptors , or intracellular perfusion with cAMP did not increase the amiloride sensitive Na current , even though these maneuvers stimulated CFTR ( cystic fibrosis transmembrane conductance regulator ) mediated Cl currents . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Role of CFTR ' s PDZ 1 binding domain , NBF 1 and Cl ( ) conductance in inhibition of epithelial Na ( + ) channels in Xenopus oocytes . ^^^ The cystic fibrosis transmembrane conductance regulator ( CFTR ) inhibits epithelial Na ( + ) channels ( ENaC ) . ^^^ We eliminated the PDZ BD of CFTR and coexpressed Na ( + ) / H ( + ) exchange regulator factors together with CFTR and ENaC . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Activation of the CFTR Cl channel inhibits epithelial Na+ channels ( ENaC ) , according to studies on epithelial cells and overexpressing recombinant cells . ^^^ Similar to CFTR , ClC 0 Cl currents also inhibit ENaC , as well as high extracellular Na+ and Cl in partially permeabilized oocytes . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
More than 1300 different mutations in the cystic fibrosis transmembrane conductance regulator ( CFTR ) cause cystic fibrosis ( CF ) , a disease characterized by deficient epithelial Cl secretion and enhanced Na+ absorption . ^^^ Apart from that , Na+ channel blockers , such as phenamil and benzamil , are being explored , which counteract the hyperabsorption of NaCl in CF airways . 3 . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
The Na ( + ) / H ( + ) exchanger regulatory factor , NHE RF , a subplasma membrane PDZ domain protein , restores cell surface expression of CFTR and chloride currents . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Cystic fibrosis transmembrane conductance regulator ( CFTR ) functions as both a chloride channel and an epithelial transport regulator , interacting with Na ( + ) ( epithelial sodium channel ) , Cl ( ) , renal outer medullary potassium channel ( + ) , and H ( 2 ) O channels and some exchangers ( i . e . ^^^ Our observations show that the proton gated Na ( + ) current formed by the heteromultimeric ASIC1a / 2a channel was up regulated by wild type but not by Delta F 508 CFTR . ^^^ In contrast , the acid gated Na ( + ) current associated with either the homomultimeric ASIC1a or ASIC2a channel was not influenced by wild type CFTR . ^^^ The apparent equilibrium dissociation constant for extracellular Na ( + ) for ASIC1a / 2a was increased by CFTR , but CFTR had no effect on the gating behavior or acid sensitivity of ASIC1a / 2a . ^^^ We conclude that wild type CFTR elevates the acid gated Na ( + ) current of ASIC1a / 2a in part by altering the kinetics of extracellular Na ( + ) interaction . . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
The cystic fibrosis transmembrane conductance regulator ( CFTR ) , in addition to its Cl ( ) channel properties , has regulatory interactions with other epithelial ion channels including the epithelial Na ( + ) channel ( ENaC ) . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
As a consequence , mutations in CFTR result in both impaired Cl ( ) secretion and enhanced Na ( + ) absorption in the colon of cystic fibrosis ( CF ) patients . ^^^ Acute regulation of absorption may occur by a Na ( + ) feedback mechanism and the cystic fibrosis transmembrane conductance regulator ( CFTR ) . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
RT PCR experiments show that IL 4 elicits a 7 fold decrease in the level of the gamma amiloride sensitive Na ( + ) channel mRNA , one of the subunits of the amiloride sensitive Na ( + ) channel , and an increase in CFTR mRNA . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Significant increases in the expression of mRNAs for alpha and beta subunits of the Na , K ATPase , the Na K Cl cotransporter and CFTR chloride channel were detected but not until 1 2 days after the feeding event . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
In cystic fibrosis , lack of the functional cystic fibrosis transmembrane conductance regulator ( CFTR ) causes failure of transcellular Cl absorption , resulting in an elevation of [ Na ] and [ Cl ] of airway surface liquid to approximately 100 mM . . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
No significant differences in renal NaCl handling between WT mice ( C ( Na ) =1 . 2 + / 0 . 3 microl 10 min ( 1 ) , C ( Cl ) =4 . 0 + / 0 . 5 microl 10 min ( 1 ) ) and DeltaF 508 CFTR mice ( C ( Na ) =1 . 7 + / 0 . 5 microl 10 min ( 1 ) , C ( Cl ) =4 . 1 + / 0 . 8 microl 10 min ( 1 ) ) were observed under control conditions . ^^^ Volume expansion resulted in large significant increases in NaCl clearance in both WT mice ( C ( Na ) =7 . 0 + / 0 . 9 microl 10 min ( 1 ) , C ( Cl ) =12 . 0 + / 0 . 6 microl 10 min ( 1 ) ) and DeltaF 508 CFTR mice ( C ( Na ) =7 . 2 + / 1 . 6 microl 10 min ( 1 ) , C ( Cl ) =11 . 0 + / 2 . 2 microl 10 min ( 1 ) ) . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Inhibition of amiloride sensitive Na ( + ) absorption by activation of CFTR in mouse endometrial epithelium . ^^^ Previous studies have demonstrated amiloride sensitive Na ( + ) absorption under basal conditions and cystic fibrosis transmembrane conductance regulator ( CFTR ) mediated Cl ( ) secretion following neurohormonal stimulation in the mouse endometrial epithelium . ^^^ The present study investigated the inhibition of amiloride sensitive Na ( + ) absorption accompanying activation of CFTR in the mouse endometrium using the short circuit current ( 1 ( sc ) ) technique . ^^^ RT PCR demonstrated the co expression of CFTR and epithelial Na ( + ) channels ( ENaC ) in primary cultured mouse endometrial epithelia and cultured endometrial monolayers exhibited a basal amiloride sensitive 1 ( sc ) of 5 . 4 + / 0 . 6 microA / cm ( 2 ) . ^^^ Our data suggest an interaction between CFTR and ENaC , which may be the underlying mechanism for balancing Na ( + ) absorption and Cl ( ) secretion across the mouse endometrial epithelium . . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
In pancreatic ducts HCO ( 3 ) ( ) secretion is mediated by cystic fibrosis transmembrane conductance regulator ( CFTR ) activated luminal Cl ( ) / HCO ( 3 ) ( ) exchange activity and HCO ( 3 ) ( ) absorption is achieved by Na ( + ) dependent mechanisms including Na ( + ) / H ( + ) exchanger 3 ( NHE 3 ) . ^^^ In a chronic mechanism , CFTR increases the luminal expression of Na ( + ) / H ( + ) exchange in pancreatic duct cells . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
The HCO ( 3 ) ( ) exits at the lumen predominantly via two recently discovered apical HCO ( 3 ) ( ) transporters . cAMP , which mediates the stimulatory effect of secretin on pancreatic ductal HCO ( 3 ) ( ) secretion , potentiates the basolateral Na ( + ) : HCO ( 3 ) ( ) cotransporter due to generation of a favorable electrogenic gradient as a result of membrane depolarization by Cl ( ) secreting cystic fibrosis transmembrane conductance regulator ( CFTR ) . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Activity of a Cl ( ) dependent anion exchanger provides near constant HCO ( 3 ) ( ) secretion in CF intestine , but under basal conditions the magnitude of secretion is lessened by simultaneous activity of a Na ( + ) / H ( + ) exchanger ( NHE ) . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
The present study investigated the regional distribution and cyclic changes in the mRNA expression of epithelial Na+ channel ( ENaC ) subunit and cystic fibrosis transmembrane conductance regulator ( CFTR ) , a cAMP activated Cl channel , in adult female mouse reproductive tract . ^^^ The involvement of ENaC and CFTR in Na+ absorption and Cl secretion was demonstrated in cultured endometrial epithelia using the short circuit current technique and found to be influenced by ovarian hormones . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Cystic fibrosis transmembrane conductance regulator ( CFTR ) was immunocytochemically localized with a monoclonal mouse antibody to the carboxy terminus and found to be present in the cytoplasm and basolateral membranes of all enterocytes and in the brush border membrane of some cells , whereas NKCC immunofluorescence , demonstrating the presence of the Na ( + ) / K ( + ) / 2Cl ( ) cotransporter , was present in the cytoplasm and brush border membrane . ^^^ Cystic fibrosis transmembrane conductance regulator ( CFTR ) was immunocytochemically localized with a monoclonal mouse antibody to the carboxy terminus and found to be present in the cytoplasm and basolateral membranes of all enterocytes and in the brush border membrane of some cells , whereas NKCC immunofluorescence , demonstrating the presence of the Na ( + ) / K ( + ) / 2Cl ( ) cotransporter , was present in the cytoplasm and brush border membrane . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
In the intestine , activation of GC C results in a dual action : stimulation of Cl and HCO 3 secretion , through the opening of apical CFTR Cl channels ; and inhibition of Na absorption , through blockade of an apical Na / H exchanger . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Using overlay assay , immunoprecipitation , pulldown and binding assays , we show that PKC epsilon does not bind to CFTR , but does bind to a receptor for activated C kinase ( RACK 1 ) , a 37 kDa scaffold protein , and that RACK 1 binds to Na ( + ) / H ( + ) exchange regulatory factor ( NHERF 1 ) , a binding partner of CFTR . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
In LC , peak NA efflux on stimulation ( 99 pulses , 100 Hz ) was higher in MAO A KO than C3H mice ( 938 + / 58 nm cf . 511 + / 42 nm ; P < 0 . 001 ) . ^^^ The NA uptake half time ( t ( 1 / 2 ) ) was longer in MAO A KO than in C3H mice ( 6 . 0 + / 0 . 9 s cf . 1 . 9 + / 0 . 3 s ; P < 0 . 001 ) and the selective NA reuptake inhibitor desipramine ( 50 nm ) had a smaller effect in MAO A KO mice . ^^^ The alpha 2 agonist dexmedetomidine ( 10 nm ) decreased stimulated NA efflux more in C3H than in MAO A KO mice ( 73 . 3 % cf . 29 . 6 % inhibition , P < 0 . 001 ) . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Simultaneous measurement of uridine evoked currents and radiolabeled uridine uptake under voltage clamp conditions gave a Na+ to uridine coupling ratio of 2 : 1 ( cf . 2 : 1 for hCNT 3 and 1 : 1 for hCNT1 / 2 ) . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Recently , corticosteroid hormone induced factor ( CHIF ) and the gamma subunit , two members of the FXYD family of small proteins , have been identified as regulators of renal Na , K ATPase . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
METHODS : In 119 surgically resected T 2 rectal cancers , the following parameters of the cancer cell nuclei were microscopically measured : mean nuclear area ( NA ) , mean perimeter ( PM ) , largest to smallest diameter ratios ( LS ) , circularity factor ( CF ) , and coefficient of variation of nuclear area ( NACV ) . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
The secondary active Cl ( ) secretion in seawater ( SW ) teleost fish gills and elasmobranch rectal gland involves basolateral Na ( + ) , K ( + ) ATPase and NKCC , apical membrane CFTR anion channels , and a paracellular Na ( + ) selective conductance . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
The data are consistent with an effect of CHIF on the Na ( + ) K ( + ) ATPase that is specific to the outer and inner medullary duct , its major expression site . . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
A functional interaction between CHIF and Na K ATPase : implication for regulation by FXYD proteins . ^^^ Like the gamma subunit of Na K ATPase , the corticosteroid hormone induced factor ( CHIF ) is a member of the FXYD family of one transmembrane segment proteins . ^^^ CHIF has been expressed in HeLa cells harboring the rat alpha ( 1 ) subunit of Na K ATPase . ( 86 ) Rb flux experiments demonstrate that CHIF induces a two to threefold increase in apparent affinity for cytoplasmic Na ( K ' ( Na ) ) but does not affect affinity for extracellular K ( Rb ) ions ( K ' ( K ) ) or 5 ( max ) . ^^^ Measurements of Na K ATPase using isolated membranes show similar but smaller effects of CHIF on K ' ( Na ) , whereas K ' ( K ) and K ' ( ATP ) are unaffected . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
IFN gamma inhibits intestinal Cl ( ) secretion , in part via downregulation of CFTR and Na ( + ) K ( + ) ATPase activity and expression , but the proximal signaling events were unknown . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Both purinergic stimulation and activation of cystic fibrosis transmembrane conductance regulator ( CFTR ) increases Cl ( ) secretion and inhibit amiloride sensitive Na ( + ) transport . ^^^ Therefore , a possible mechanism on how CFTR controls the activity of epithelial Na ( + ) channels ( ENaC ) could be by release of ATP or uridine 5 ' triphosphate ( UTP ) , which would then bind to P2Y receptors and inhibit ENaC . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Tracheas of both normal and CFTR ( / ) mice showed a dominant amiloride sensitive Na+ absorption under both , control conditions and after cAMP dependent stimulation . ^^^ Thus , the present study demonstrates Cl ( ) secretion in mouse airways , which depends on basolateral Na ( +2 ) Cl ( ) K ( + ) cotransport and luminal CFTR and non CFTR Cl ( ) channels . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
In CF , the impaired Cl ( ) and HCO ( 3 ) ( ) secretion associated with increased epithelial Na ( + ) absorption results in dehydration of airway mucus , decreased antimicrobial functions and impaired mucociliary clearance . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
CFTR inhibits epithelial Na ( + ) channel ( ENaC ) currents in many epithelial and non epithelial cells , whereas the presence of ENaC increases CFTR functional expression . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
To investigate whether the cAMP induced Na ( + ) HCO ( 3 ) ( ) cotransport activation was secondary to secretion associated changes in HCO ( 3 ) ( ) or cell volume , we measured potential forskolin induced changes in intracellular pH and assessed Na ( + ) HCO ( 3 ) ( ) transport activity in CFTR / crypts ( in which no forskolin induced cell shrinkage occurs ) . ^^^ We found 30 % reduced Na ( + ) HCO ( 3 ) ( ) transport rates in CFTR / compared with CFTR + / + crypts but similar Na ( + ) HCO ( 3 ) ( ) cotransport activation by forskolin . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
The cystic fibrosis transmembrane conductance regulator interacts with and regulates the activity of the HCO 3 salvage transporter human Na+ HCO 3 cotransport isoform 3 . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Many epithelia express specific Na ( + ) channels ( ENaC ) together with the cystic fibrosis regulator ( CFTR ) Cl ( ) channels . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
This stimulation of alveolar liquid clearance is related to activation or increased expression of sodium transport molecules such as the epithelial sodium channel or the Na ( + ) K ( + ) ATPase pump and may also involve the cystic fibrosis transmembrane conductance regulator . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
A sequence is developed for CF whereby there is a depletion of the PCL that reflects the combined dysfunctions of accelerated Na ( + ) dependent volume absorption and failure to secrete Cl ( ) . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Using micromethod of complement fixation ( CF ) test with genus specific antigen Ch . psittaci ( Bioveta , Ivanovice na Han , Czech Republic ) , the seropositivity in pheasants oscillated between 31 . 5 40 . 4 % . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
We also demonstrate that cofilin interacts with the large cytoplasmic domains of the alpha 1 , alpha 2 and alpha 3 isoforms of Na , K ATPase , but not with those of glucose transporter 1 , glucose transporter 4 , cystic fibrosis transmembrane conductance regulator and plasma membrane Ca ATPase . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Phospholemman ( FXYD 1 ) in heart and skeletal muscle , the gamma subunit of Na , K ATPase ( FXYD 2 ) and corticosteroid hormone induced factor ( FXYD 4 , also known as CHIF ) in the kidney , and FXYD 7 in the brain associate preferentially with the widely expressed Na , K ATPase alpha 1 beta1 isozyme and modulate its transport activity in a way that conforms to tissue specific requirements . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Lack of CFTR Cl ( ) conductance ( g ( CFTR ) ) in the plasma membranes was seen to enhance ENaC conductance ( g ( ENaC ) ) and Na ( + ) absorption from the airway surface liquid causing airway pathology in cystic fibrosis ( CF ) . ^^^ These results showed that lack of CFTR in the plasma membranes is not characteristically coupled to elevated ENaC activity or to increased Na ( + ) absorption in CF epithelial cells . ^^^ Early studies suggested that in absorption significant cross talk occurs between CFTR Cl ( ) channels and epithelial Na ( + ) channels ( ENaC ) . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Activation of the CFTR Cl ( ) channel inhibits epithelial Na ( + ) absorption , according to studies on native epithelia derived from airways , colon and kidney , and can also be demonstrated in overexpressing cells . ^^^ However , Na ( + ) absorption is not inhibited by CFTR in the native sweat duct epithelium . ^^^ Similar to CFTR , ClC 0 Cl ( ) currents also inhibit ENaC , as well as high extracellular Na ( + ) and Cl ( ) in partially permeabilized oocytes . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
CFTR and the epithelial Na channel ( ENaC ) are two membrane proteins expressed in the apical membrane of several epithelia . ^^^ Second , concerning the influence of CFTR on ENaC , the simulations show that : ( 1 ) under current clamp conditions , the amplitude of the apical membrane Cl ( ) conductance has a strong influence on the transepithelial depolarization induced by inhibition of the apical Na ( + ) conductance , ( 2 ) under voltage clamp conditions , the decrease of short circuit current induced by inhibition of the apical Na ( + ) conductance is also significantly decreased by the presence of a large apical membrane Cl ( ) conductance . ^^^ Our results show that when ENaC and CFTR are expressed in the same apical membrane , the electrical coupling of these two ion channels can explain at least part of the effect of CFTR activation on the Na ( + ) current flowing through ENaC . . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
By altering or reproducing the ouabain induced changes in intracellular ionic activities ( decreasing in external Na ( + ) or K ( + ) or using Na ( + ) ionophore ) , we show that the ouabain induced regulations of both CFTR and MDR 1 transcripts depend on the Na ( + ) / K ( + ) pump inhibition but that the decrease in CFTR mRNAs also proceeds from cytoplasm reactions simultaneously activated by ouabain . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
In a final series of studies , normal and CF cells acutely alkaline loaded by replacing bilateral Krebs bicarbonate Ringer ( KBR ) with Hepes buffered Ringer solution exhibited basolateral , but not apical , recovery from an alkaline challenge that was dependent on Cl , independent of Na+ and blocked by H2DIDS . ^^^ We conclude that : ( 1 ) normal , but not CF , nasal epithelia have a constitutively active DPC sensitive HCO 3 influx / efflux pathway across the apical membrane of cells , consistent with the movement of HCO 3 via CFTR ; and ( 2 ) both normal and CF nasal epithelia have Na+ independent , H2DIDS sensitive AE at their basolateral domain . . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Corticosteroid hormone induced factor ( CHIF ) and the gamma subunit of the Na , K ATPase ( gamma ) are two members of the FXYD family whose function has been elucidated recently . ^^^ CHIF and gamma interact with the Na+ pump and alter its kinetic properties , in different ways , which appear to serve their specific physiological roles . ^^^ The transmembrane segments also determine the opposite effects of CHIF and gamma on the Na+ affinity of the pump , but the amino acids involved in this functional effect are different from those responsible for stable interactions with alpha . . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
The serum and glucocorticoid inducible kinase SGK 1 stimulates the Na+ channels ENaC and SCN5A , the K+ channels ROMK 1 , Kv1 . 3 , and KCNE1 / KCNQ1 , the cation conductance induced by 4F2 / LAT1 and the chloride conductance induced by CFTR . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Effects of purinergic stimulation , CFTR and osmotic stress on amiloride sensitive Na+ transport in epithelia and Xenopus oocytes . ^^^ Both stimulation of purinergic receptors by ATP and activation of the cystic fibrosis transmembrane conductance regulator ( CFTR ) inhibit amiloride sensitive Na+ transport and activate Cl secretion . ^^^ We therefore examined whether cell shrinkage or cell swelling do affect amiloride sensitive Na+ transport in epithelial tissues or Xenopus oocytes and whether osmotic stress interferes with regulation of Na+ transport by ATP or CFTR . ^^^ When coexpressed in Xenopus oocytes , activation of CFTR by IBMX and forskolin inhibited the epithelial Na+ channel ( ENaC ) in a Cl dependent fashion . ^^^ We conclude that amiloride sensitive Na+ absorption in mouse airways and colon is inhibited by cell shrinkage by a mechanism that does not interfere with purinergic and CFTR mediated inhibition of ENaC . . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Among the antimicrobial agents tested , the highest resistance rates in the penicillin group were found for Ampicillin ( AM ) ( up to 18 % ) and Piperacillin ( PIP ) ( up to 12 % ) ; in the cephalosporin group for Cefalothin ( CF ) ( up to 35 % ) and Cefuroxime Axetil ( CXMAX ) ( up to 11 % ) ; in the group of quinolones for Nalidixic acid ( NA ) ( up to 15 % ) ; and for Trimethoprime / Sulfamethoxazole ( SXT ) ( up to 13 % ) and for Tetracycline ( TE ) ( 57 % ) . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
The nonoverlapping distribution of gamma and CHIF ( FXYD 4 ) in kidney helps to explain physiological differences in Na ( + ) affinity among nephron segments . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
We have investigated whether other members of the FXYD family may have a similar role as the gamma subunit and have found that CHIF ( corticosteroid hormone induced factor , FXYD 4 ) , FXYD 7 , as well as phospholemman ( FXYD 1 ) specifically associate with Na , K ATPase and preferentially with alpha 1 beta isozymes in native tissues , and produce distinct effects on the transport properties of Na , K ATPase that are adapted to the physiological demands of the tissues in which they are expressed . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
A specific functional interaction between CHIF and Na , K ATPase : role of FXYD proteins in the cellular regulation of the pump . ^^^ CHIF ( corticosteroid hormone induced factor ) is a member of the FXYD family that shares approximately 50 % homology with the gamma subunit of Na , K ATPase . ^^^ Both CHIF and gamma are coimmunoprecipitated by an anti alpha subunit antibody , and alpha is immunoprecipitated by anti gamma and anti CHIF antibodies . ( 86 ) Rb ( + ) flux experiments in CHIF transfected HeLa cells demonstrate that CHIF increases the affinity for cytoplasmic Na ( + ) , but does not affect the affinity for extracellular K ( Rb ) . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Immunocytochemical localization of Na , K ATPase gamma subunit and CHIF in inner medulla of rat kidney . ^^^ The gamma subunit of Na , K ATPase and CHIF both belong to the FXYD single membrane spanning protein family and have been suggested to have regulatory functions in kidney tubules . ^^^ The present observations demonstrate that the Na , K ATPase gamma subunit and CHIF are strategically located in the inner medulla to participate in the fine tuning of urine ion composition through the regulation of the Na , K ATPase activity in the IMCD . . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
The FXYD protein family has recently been defined as a result of the search for homologues of the Na , K ATPase gamma subunit , CHIF , and phospholemman in EST and gene data banks . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
AIM : We compared the distribution and putative association of Cl channel transport , CFTR mRNA transcripts , and Na+ channel ( ENaC ) alpha and beta subunit mRNA transcripts in villus and crypt epithelial cells of duodenum , with corresponding surface and crypt cells of colon from sodium depleted rats . ^^^ Possible interaction between CFTR and Na+ channels is apparently limited to parts of the colonic crypt . ^^^ Cystic fibrosis transmembrane conductance regulator and Na+ channel subunits mRNA transcripts , and Cl efflux , show a different distribution in rat duodenum and colon . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
The hIK 1 mediated ATP release was inhibited by a hIK 1 blocker ( charybdotoxin ) , and an Na ( + ) K ( + ) 2Cl ( ) cotransport blocker ( bumetanide ) without interruption by GdCl ( 3 ) , an inhibitor of stretch activated nonselective cation ( SA ) channels , or glybenclamide , a blocker of the cystic fibrosis transmembrane conductance regulator ( CFTR ) . ^^^ These results suggest that a cell volume decrease via the hIK 1 mediated KCl loss and the resultant induction of a regulatory volume increase via the Na ( + ) K ( + ) 2Cl ( ) transporter may trigger release of ATP , which causes P2Y ( 1 ) mediated Ca ( 2+ ) mobilization , through mechanisms unrelated to the CFTR and SA channels . . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
AMP inhibition of murine intestinal Na / H exchange requires CFTR mediated cell shrinkage of villus epithelium . ^^^ Because CFTR mediated anion secretion has been associated with changes in crypt cell volume , we hypothesized that CFTR mediated cell volume reduction in villus epithelium is required for intracellular cyclic adenosine monophosphate inhibition of Na ( + ) / H ( + ) exchanger ( primarily Na ( + ) / H ( + ) exchanger 3 ) activity in the proximal small intestine . ^^^ METHODS : Transepithelial ( 22 ) Na flux across the jejuna of CFTR ( + ) , CFTR ( ) , the basolateral membrane Na ( + ) / K ( + ) / 2Cl ( ) co transporter protein NKCC 1 ( + ) , and NKCC 1 ( ) mice were correlated with changes in epithelial cell volume of the midvillus region . ^^^ RESULTS : Stimulation of intracellular cyclic adenosine monophosphate resulted in cessation of Na ( + ) / H ( + ) exchanger mediated Na ( + ) absorption ( J ( ms ) ( NHE ) ) in CFTR ( + ) jejunum but had no effect on J ( ms ) ( NHE ) across CFTR ( ) jejunum . ^^^ Bumetanide treatment to inhibit Cl ( ) secretion by blockade of the Na ( + ) / K ( + ) / 2Cl ( ) co transporter , NKCC 1 , of stimulated CFTR ( + ) jejunum prevented maximal volume reduction of villus epithelium and recovered approximately 40 % of J ( ms ) ( NHE ) . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
The change of DeltaH ( HH ) of CF ( 2 ) unit in F ( CF ( 2 ) ) ( n ) COONH ( 4 ) shows the similar tendency to H ( CH ( 2 ) ) ( n ) SO ( 4 ) Na . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
ASL volume changes reflected altered ion transport , including an upregulation of Cl ( ) secretory currents ( via CFTR and Ca ( 2+ ) activated Cl ( ) conductance ) and an inhibition of epithelial sodium channel ( ENaC ) mediated absorptive Na ( + ) currents . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
At high altitude , the amount of mRNA of Na K ATPase , CFTR , and beta actin of brush biopsies did not change in controls but decreased significantly ( 60 % ) in HAPE susceptible subjects . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
ASL [ Na+ ] was 122 + / 2 mM , [ Cl ] was 123 + / 4 mM and pH was 7 . 28 + / 0 . 07 , and not dependent on airway size ( < 100 to > 250 mum diameter ) , ENaC inhibition by amiloride , or CFTR inhibition by the thiazolidinone CFTRinh 172 . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
MCT in the CF mouse nasal cavity ( 1 . 4 + / 0 . 31 , n = 8 ) , a region in which the CF mouse exhibits bioelectric properties similar to the human CF patient , was , again , not different from the normal mouse , perhaps reflecting copious gland secretion offsetting Na ( + ) and liquid hyperabsorption . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
The amiloride sensitive epithelial sodium channel ( ENaC ) , a multimeric plasma membrane protein composed of alpha , beta , and gamma ENaC subunits , mediates Na ( + ) reabsorption in epithelial tissues , including the distal nephron , colon , lung , and secretory glands , and plays a critical role in pathophysiology of essential hypertension and cystic fibrosis ( CF ) . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Pharmacological profiling of the channels and transporters involved in fluid and electrolyte transport showed that net fluid transport requires Cl uptake across the basolateral membrane through a Na ( + ) K ( + ) 2Cl cotransporter ( NKCC 1 ) and its subsequent exit across an apical cystic fibrosis transmembrane conductance regulator ( CFTR ) Cl channel . ^^^ Expression of chloride channels ( CFTR and the Ca ( 2+ ) dependent Cl channel , ClCa 1 ) , potassium channels ( KCNN 4 and KCNQ 1 ) , epithelial Na+ channel ( ENaC ) alpha , beta and gamma subunits and ion transporters ( NKCC 1 ; anion exchanger , AE 2 ; Na+ / H+ exchangers , NHE 1 3 ) was detected by RT PCR and Western blot in the case of ENaC . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
In ' Deconsolidation ' , the fluid uptake process can be reversed and amplified by agents that increase cAMP , reactivating the Na+ , K+ ATPase and expressing CFTR or equivalent Cl secretory channels that force the extrusion of fluid from the platelets , with rehydration of the intercellular polymer and a large increase in the interplatelet space . . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
CF is characterized by enhanced airway Na ( + ) absorption , mediated by epithelial Na ( + ) channels ( ENaC ) , and deficient Cl ( ) transport . ^^^ In other epithelial tissues , epithelial Na ( + ) conductance is either increased ( intestine ) or decreased ( sweat duct ) in CF . ^^^ Activation of the dominant class of metabotropic purinergic receptors , P2Y ( 2 ) receptors , appears to have a 2 fold benefit on ion transport in CF airways ; excessive Na ( + ) absorption is attenuated , most likely by inhibition of the ENaC and , simultaneously , an alternative Ca ( 2+ ) dependent Cl ( ) channel is activated that may compensate for the CFTR Cl ( ) channel defect . ^^^ Administration of these compounds alone , or in combination with other drugs that inhibit accelerated Na ( + ) transport and help recover or increase residual activity of mutant CFTR , is most promising as successful therapy to counteract the ion transport defect in the airways of CF patients . . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Recent data have linked the abnormal ion transport properties of CF airway epithelia to depleted airway surface liquid ( ASL ) volume , reflecting the combined defects of accelerated Na+ transport and the failure to secrete Cl . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Recent data have suggested that CHIF interacts with the a subunit of the Na+ K+ ATPase and increases the pump ' s affinity to cell Na+ . ^^^ CHIF knockout ( KO ) mice have mild renal phenotype under low Na+ or high K+ diets . ^^^ The present findings support a role of CHIF as an indirect modulator of several different ion transport mechanisms and are consistent with regulation of the Na+ K+ ATPase as the common denominator . . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
The perfluorinated heptanol [ CF ( 3 ) ( CF ( 2 ) ) ( 5 ) CH ( 2 ) OH ] , which produces anesthesia , inhibited the Na ( 5 ) 1 . 2 channel like other alcohols tested ( ethanol , heptanol , and CF ( 3 ) CH ( 2 ) OH ) , even though this compound does not affect GABA , glycine , alpha amino 3 hydroxy 5 methyl 4 isoxazole propionic acid , or kainate receptors . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
A combination of laser flash photolysis and competitive kinetic methods have been used to measure the absolute bimolecular rate constants for hydrogen atom abstraction in water from a variety of organic substrates including alcohols , ethers , and carboxylic acids by the perfluoroalkyl radical , * CF ( 2 ) CF ( 2 ) OCF ( 2 ) CF ( 2 ) SO ( 3 ) ( ) Na ( + ) . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Na ( + ) dependent uptake by BBMVs from CF ( Cftr ( tm1Eur ) ) mice was not reduced compared with wild type and Swiss BBMVs . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
However , this higher order complex is predominantly CFTR dimers , and the PDZ interacting partners ( Na ( + ) / H ( + ) exchanger regulatory factor 1 ( NHERF 1 ) and NHERF 2 ) constitute approximately 2 % of this complex . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
In past studies , we demonstrated regulation of CFTR Cl channel function by protein kinase C ( PKC ) epsilon through the binding of PKC epsilon to RACK 1 ( a receptor for activated C kinase ) and of RACK 1 to human Na ( + ) / H ( + ) exchanger regulatory factor ( NHERF 1 ) . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Spring wheat cultivated in NF OTC showed only minor differences in the P , K , Ca , Mg , Mn and Na concentrations of the different plant parts at final harvest , as well as organic and inorganic S fractions , compared to those of the plants grown in CF air . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
The complexes [ Na ( ctv ) ( H ( 2 ) O ) ( CB ( 11 ) H ( 6 ) 10 ( 6 ) ) ] ( CF ( 3 ) CH ( 2 ) OH ) feature chiral Na CTV coordination chains with complexation of the [ CB ( 11 ) H ( 6 ) 10 ( 6 ) ] ( ) ion by the Na ( + ) ion , together with the CTV molecular cavity . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Mutations in the cystic fibrosis transmembrane conductance regulator ( CFTR ) gene result in defective epithelial cAMP dependent Cl ( ) secretion and increased airway Na ( + ) absorption . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Treatment with cAMP ( 100 microM 8 cpt cAMP plus 100 microM IBMX ) stimulated ENaC mediated Na+ absorption as well as Cl secretion via cystic fibrosis transmembrane conductance regulator , which was characterized in alpha toxin permeabilized monolayers to have the anion selectivity sequence NO 3 > Br > Cl > 1 . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Under basal conditions , electroneutral Na ( + ) absorption mediated by Na ( + ) / H ( + ) exchanger 3 ( NHE 3 ) predominates ; under stimulated conditions , increased anion secretion mediated by CFTR occurs concurrently with inhibition of Na ( + ) absorption . ^^^ To test this hypothesis , we measured electrogenic anion secretion ( indexed by the short circuit current ) across NHE 3 null [ NHE 3 ( ) ] murine small intestine and electroneutral Na ( + ) absorption ( by radioisotopic flux analysis ) across small intestine of mice with gene targeted disruptions of the anion secretory pathway , i . e . , CFTR null [ CFTR ( ) ] or Na ( + ) K ( + ) 2Cl ( ) cotransporter null [ NKCC 1 ( ) ] . ^^^ In CFTR ( ) and NKCC 1 ( ) intestine , Na ( + ) absorption was significantly reduced compared with wild type . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
The abundant CFTR mRNAs and proteins around the ovulatory period may drive Na ( + ) and fluid from plasma into the uterine lumen to make optimal electrolyte composition and sufficient fluid volume for sperm migration . . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Inhibition of epithelial Na+ channels ( ENaC ) by the cystic fibrosis transmembrane conductance regulator ( CFTR ) has been demonstrated previously . ^^^ In the present study we demonstrate that the Na+ / H+ exchanger regulator factor ( NHERF ) controls expression of CFTR in mouse collecting duct cells . ^^^ Inhibition of NHERF largely attenuates CFTR expression , which is paralleled by enhanced Ca ( 2+ ) dependent Cl secretion and augmented Na+ absorption by the ENaC . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
RESULTS : The concentrations of the major components Na and Cl did not differ , Zn was higher and NO 3 was lower in CF patients . ^^^ During a given time period , CF patients produced a slightly larger volume of breath condensate and they exhaled more Na , K and Zn . ^^^ CONCLUSIONS : These data detail the composition of exhaled breath condensate and suggest a similar Na and Cl concentration in CF airway surface fluid as in healthy subjects . . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Inhibition of the dysregulated epithelial Na channel ( ENaC ) is one pharmacologic approach to enhance airway clearance in CF . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Zn had no effect on bumetanide sensitive 86Rb uptake , Na K ATPase , or CFTR . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
CFTR ( / ) mice had significantly higher concentrations of Na and Cl in their ASL than age matched controls . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Neutrophil elastase is a serine protease that is abundant in the airways of individuals with cystic fibrosis ( CF ) , a genetic disease manifested by excessive airway Na ( + ) absorption and consequent depletion of the airway surface liquid layer . ^^^ The hNE induced increase of near silent ENaC activity in CF airways could contribute to Na ( + ) hyperabsorption , reduced airway surface liquid height , and dehydrated mucus culminating in inefficient mucociliary clearance . . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
The pharmacology of 1 ( plateau ) suggests that CFTR channels are involved and that Na ( + ) / K ( + ) / 2Cl ( ) is the only transporter associated with 1 ( plateau ) . dDAVP , a V 2 receptor agonist induced 1 ( plateau ) with the same potency as AVP , suggesting the involvement of V 2 receptors in the AVP induced 1 ( plateau ) . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
As an inhibitor of the sarcoplasmic reticulum Ca ( 2+ ) ATPase , curcumin was shown to correct cystic fibrosis ( CF ) defects in some model systems , whereas others have reported no or little effects on CF after curcumin treatment , suggesting that curcumin effect is not due to simple inhibition of the Ca ( 2+ ) ATPase . 2 We tested the hypothesis that curcumin may modulate other members of the P ( 2 ) type ATPase superfamily by studying the effects of curcumin on the activity and kinetic properties of the Na , K ATPase . 3 Curcumin treatment inhibited Na , K ATPase activity in a dose dependent manner ( K ( 0 . 5 ) approximately 14 . 6 microM ) . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Interdependency of beta adrenergic receptors and CFTR in regulation of alveolar active Na+ transport . ^^^ Alveolar fluid clearance ( AFC ) , an index of alveolar active Na+ transport , was 92 % greater in CFTR overexpressing lungs than controls . ^^^ These studies indicate that upregulation of alveolar CFTR function speeds clearance of excess fluid from the airspace and that CFTRs effect on active Na+ transport requires the betaAR . ^^^ These studies reveal a previously undetected interdependency between CFTR and betaAR that is essential for upregulation of active Na+ transport and fluid clearance in the alveolus . . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
The ability of 1 to recognize anions , including CF ( 3 ) SO ( 3 ) ( ) and BF ( 4 ) ( ) , remarkably increases in a stepwise manner using Na ( + ) and Ag ( + ) as effectors . ^^^ The enhancement of the K ( a ) eventually reaches factors of 1500 and 2000 for NO ( 3 ) ( ) and CF ( 3 ) SO ( 3 ) ( ) , respectively , in the presence of both Na ( + ) and Ag ( + ) , compared to the free 1 . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Amiloride sensitive , epithelial Na ( + ) channel ( ENaC ) mediated , active absorption of Na ( + ) is elevated in the airway epithelium of cystic fibrosis ( CF ) patients , resulting in excess fluid removal from the airway lumen . ^^^ Herein we show that INO 4995 , a synthetic analog of the intracellular signaling molecule , D myo inositol 3 , 4 , 5 , 6 tetrakisphosphate , inhibits Na ( + ) and fluid absorption across CF airway epithelia , thus alleviating this critical pathology . ^^^ This conclusion was based on electrophysiological studies , fluid absorption , and ( 22 ) Na ( + ) flux measurements in CF airway epithelia , contrasted with normal epithelia , and on electrophysiological studies in Madin Darby canine kidney cells and 3T3 cells overexpressing ENaC . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Transepithelial Cl ( ) secretion in polarized renal A 6 cells is composed of two steps : ( 1 ) Cl ( ) entry step across the basolateral membrane mediated by Na ( + ) / K ( + ) / 2Cl ( ) cotransporter ( NKCC ) and ( 2 ) Cl ( ) releasing step across the apical membrane via cystic fibrosis transmembrane conductance regulator ( CFTR ) Cl ( ) channel . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Cystic fibrosis ( CF ) transmembrane conductance regulator ( CFTR ) Cl ( ) channel function is required for activating amiloride sensitive epithelial Na ( + ) channels ( ENaC ) in salt absorbing human sweat duct . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Consistent with their functional similarities in seawater , killifish gills and opercular epithelium expressed Na+ , K+ ATPase alpha 1a , Na+ , K+ , 2Cl cotransporter 1 ( NKCC 1 ) , cystic fibrosis transmembrane conductance regulator ( CFTR ) Cl channel and the signalling protein 14 3 3a at similar absolute levels . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
The cystic fibrosis transmembrane conductance regulator ( CFTR ) is a protein kinase A and ATP regulated Cl channel that also controls the activity of other membrane transport proteins , such as the epithelial Na+ channel ENaC . ^^^ Coexpression of rat ENaC with human CFTR or the human Cl channel CLC 0 caused inhibition of amiloride sensitive Na+ currents after cAMP dependent stimulation and in the presence of a 100 mM bath Cl concentration . ^^^ The results confirm a role of the carboxyl terminus of betaENaC for Cl ( ) dependent inhibition of the Na+ channel , which , however , may only be part of a complex regulation of ENaC by CFTR . . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
The residual response in Cftr null mice could be attributed to electrogenic ASBT activity , as it matched the TC coupled absorptive Na+ flux . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
RECENT FINDINGS : Recent studies have found that AMPK , which becomes activated during cellular metabolic stress , promotes the cellular uptake of fuel sources such as glucose and fatty acids to promote ATP generation and inhibits ion transport proteins such as the cystic fibrosis transmembrane conductance regulator Cl channel and the epithelial Na channel , thereby limiting the dissipation of transmembrane ion gradients . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
We demonstrated that the ion content ( Na ( + ) , Mg ( 2+ ) , P , S and Cl ( ) ) is significantly higher and the water content significantly lower in secretory granules from the CF cell line compared to the non CF cell line . ^^^ The absence or decreased expression of cystic fibrosis transmembrane conductance regulator ( CFTR ) induces increased Na ( + ) absorption and hyperabsorption of the airway surface liquid ( ASL ) resulting in a dehydrated and hyperviscous ASL . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Compared to brackish water sham fish , mRNA expression of CFTR and NKCC 1 decreased in the gills of sham fish transferred to freshwater , whereas Na ( + ) , K ( + ) ATPase alpha ( 1a ) mRNA expression and alpha protein abundance , as well as cell proliferation ( detected using BrdU ) increased . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Ezrin controls the macromolecular complexes formed between an adapter protein Na+ / H+ exchanger regulatory factor and the cystic fibrosis transmembrane conductance regulator . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
We have developed a polarized cell culture model of primary murine nasal epithelia in which the CF tissue exhibits not only a defect in cAMP mediated Cl secretion but also the Na+ hyperabsorption and upregulation of the Ca2+ activated Cl conductance observed in human airways . ^^^ Furthermore , we found that treatment of CF preparations with a Na+ channel blocker for 7 days prevented formation of mucus adherent to epithelial surfaces . ^^^ The cultured murine nasal epithelial preparation should be an excellent model tissue for gene transfer studies and pharmacological studies of Na+ channel blockers and mucolytic agents as well as for further characterization of CF ion transport defects . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Thus , CF epithelia cultures showed elevated Na+ transport , drastic hyperabsorption of surface liquid , and absence of cAMP induced Cl secretion as compared with non CF cultures . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Na+ / H+ exchanger regulatory factor isoform 1 overexpression modulates cystic fibrosis transmembrane conductance regulator ( CFTR ) expression and activity in human airway 16HBE14o cells and rescues DeltaF 508 CFTR functional expression in cystic fibrosis cells . ^^^ The Na ( + ) / H ( + ) exchanger regulatory factor isoform 1 ( NHERF 1 ) was the first identified CFTR binding protein . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Cystic fibrosis ( CF ) is an autosomal recessive disorder of Cl ( ) and Na ( + ) transport . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Finally , at the whole animal level , we tested the ability of curcumin and thapsigargin to ( 1 ) induce Cl secretion and reduce hyperabsorption of Na+ in the nasal epithelia of the deltaF 508 mouse in vivo , and ( 2 ) induce Cl secretion in intestine ( jejunum and distal colon ) and the gallbladder of the deltaF 508 CF mouse . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Airway epithelial cells from cystic fibrosis ( CF ) individuals can not secrete adequate Cl through cystic fibrosis transmembrane regulator , and their Na+ channel ( ENaC ) activity is increased so that excessive Na+ and water is absorbed from the lumen . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
The current data on the developmental expression and functions of ENaC Na ( + ) channels , the CFTR , ClC 2 Cl ( ndash ; ) channels , L type Ca ( 2+ ) channels , P 2 purinoceptors , and the Kir6 . 1 / SUR2 , ROMK ( Kir1 . 1 ) , and Kv K ( + ) channels are presented . . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Interactions of rat FXYD 4 ( corticosteroid hormone induced factor ( CHIF ) ) , FXYD 2 ( gamma ) , or FXYD 1 ( phospholemman ( PLM ) ) proteins with rat alpha 1 subunits of Na ( + ) , K ( + ) ATPase have been analyzed by co immunoprecipitation and covalent cross linking . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
METHODS : PDs across the fluid bathed ocular surface were measured in anesthetized wild type and CF mice in response to Na+ , Cl , and K+ ion substitution and transporter agonists , inhibitors , and substrates . ^^^ The Na+ dependent depolarization by amiloride was significantly greater in CF mice ( 19 + / 3 mV ) . ^^^ CONCLUSIONS : PD measurements coupled with model computations defined quantitatively the roles of Na+ and Cl transport processes in ocular surface ion and fluid secretion , and indicated that CFTR dependent changes in apparent epithelial Na+ channel ( ENaC ) activity could be accounted for by electrochemical coupling , without requiring ENaC CFTR interactions . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Recent experimental evidence suggests that at least five of the seven members of this family , FXYD 1 ( phospholemman ) , FXYD 2 ( gamma subunit of Na K ATPase ) , FXYD 3 ( Mat 8 ) , FXYD 4 ( CHIF ) , and FXYD 7 , are auxiliary subunits of Na K ATPase and regulate Na K ATPase activity in a tissue and isoform specific way . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Human airways and glands express the anion channel cystic fibrosis transmembrane conductance regulator , CFTR , and the epithelial Na ( + ) channel , ENaC . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Thus , P . aeruginosa reduces Na+ absorption , which could enhance local mucociliary clearance , a mechanism that seem to be attenuated in CF . . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
When seeded on permeable supports , PVD 9902 cells grew as electrically tight ( > 6 , 000 ohms 10 cm 2 ) , confluent monolayers that responded to forskolin with an increase in short circuit current ( 1 ( sc ) ; 8 + / 1 microA / cm2 ) that required Cl , HCO 3 ( ) , and Na+ , and was partially sensitive to bumetanide . mRNA was expressed for a number of anion transporters , including CFTR , electrogenic Na+ HCO 3 ( ) cotransporter 1b ( NBCe1b ) , downregulated in adenoma , pendrin , and Cl / formate exchanger . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Studies of expression of Na ( + ) and CI ( ) channels ( ENaC , CFTR ) indicated that these channels were subject to tissue specific regulation within the uterus , but more work is required to determine their role and the factors controlling their abundance and localization in early pregnancy . . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
ASL volume is maintained by the predominantly ciliated epithelium via coordinated regulation of ( a ) absorption , by the epithelial Na+ channel , and ( b ) secretion , by the Ca2+ activated Cl channel ( CaCC ) and CFTR . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
The corticosteroid hormone induced factor ( CHIF ) is a member of the one transmembrane segment protein family named FXYD , which also counts phospholemman and the Na , K pump gamma subunit . ^^^ Originally it was suggested that CHIF could induce the expression of the 1 ( Ks ) current when expressed in Xenopus laevis oocytes , but recently CHIF has attracted attention as a modulatory subunit of the Na , K pump . ^^^ Thereby , CHIF is the first accessory subunit shown to be capable of modulating both the Na , K pump and an ion channel . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Declining [ K ( + ) ] ( c ) cause parallel deactivation of CFTR by dephosphorylation , thereby limiting apical influx of Cl ( ) ( and its co ion Na ( + ) ) until [ K ( + ) ] ( c ) is stabilized . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
The apical fluid in CF cells had significantly higher concentrations of Na and Cl than that in control cultures . ^^^ In control cultures , the concentrations of Na and Cl in the apical fluid increased if glibenclamide , an inhibitor of the cystic fibrosis transmembrane conductance regulator ( CFTR ) was added to the apical medium . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
These results indicate that in Calu 3 cells : ( 1 ) under the basal ( unstimulated ) condition ambroxol increases Cl secretion by stimulating the entry step of Cl and decreases HCO 3 secretion by diminishing the activity of the CFTR channel and / or the Na+ / HCO3 ( ) dependent cotransporter , ( 2 ) under the adrenergic agonist stimulated condition , ambroxol decreases Cl secretion by acting on the Cl / HCO3 exchanger , and ( 3 ) ambroxol has a more powerful action than the adrenergic agonist on the Cl / HCO3 exchanger , leading fluid secretion to a moderately stimulated level from a hyper stimulated level . . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Transgenic mice with cystic fibrosis ( CF ) had significantly higher Na and C 1 concentrations in the airway surface liquid than normal mice . ^^^ In CF patients , CF heterozygotes , and rhinitis patients , the levels of Na and C 1 in the nasal fluid were significantly higher than in healthy controls . ^^^ Females with CF had significantly higher concentrations of Na , Cl and K in their nasal fluid compared to male patients . ^^^ In the CF cell cultures , the apical fluid had a higher Na and Cl concentration than in the controls . . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Bicarbonate secretion by rat bile duct brush cells indicated by immunohistochemical localization of CFTR , anion exchanger AE 2 , Na+ / HCO3 cotransporter , carbonic anhydrase 2 , Na+ / H+ exchangers NHE 1 and NHE 3 , H+ / K+ ATPase , and Na+ / K+ ATPase . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
In CF patients , CF heterozygotes , rhinitis , and PCD patients , [ Na ] and [ Cl ] were significantly higher compared when compared with those in controls . [ K ] was significantly higher in CF and PCD patients compared with that in controls . ^^^ Female CF patients had higher levels of Na , Cl , and K than male patients . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
First , we examined mechanisms underlying the cell volume regulation in a simple model consisting of Na+ / K+ pump , Na+ K+ 2Cl cotransporter 1 ( NKCC 1 ) , cystic fibrosis transmembrane conductance regulator , volume regulated Cl channel and background Na+ , K+ and Cl currents . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Expression of Na ( + ) / K ( + ) / 2Cl ( ) cotransporter and the cystic fibrosis transmembrane conductance regulator were also greater in seawater , suggesting that chronic COX 2 expression in the gills is regulated in parallel to the key ion transporters that mediate NaCl secretion . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
This analysis confirmed translation of the genes of interest and showed that NKCC 1 , Na+ , K+ ATPase and the M 3 cholinergic receptor are expressed on the basolateral membrane of duct cells , whereas KCC 1 , IK ( Ca ) 1 , CFTR , and ClC 3 are apically localized . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
CONCLUSION : In CF small airways , defective Cl ( ) secretion combined with unregulated ( persistent ) Na ( + ) absorption results in ASLdepletion . . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
At least six members of the family have been shown to associate with the Na , K ATPase in a cell and tissue specific manner , while four of them , namely the gamma subunit ( FXYD 2 ) , CHIF ( FXYD 4 ) , phospholemman ( FXYD 1 ) , and dysadherin ( FXYD 5 ) have been identified in kidney . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
FXYD 2 or gamma subunit and FXYD 4 or CHIF modulate the apparent affinity for Na ( + ) of Na , K ATPase in an opposite way , adapted to the physiological needs of Na ( + ) reabsorption in different segments of the renal tubule . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
The decrease in resistance was accompanied by an increase in mannitol flux across the epithelium and occurred in cystic fibrosis transmembrane conductance receptor ( CFTR ) epithelium pretreated with amiloride to block Na and Cl conductances , confirming that the decrease in resistance represented an increase in paracellular conductance . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
This review summarizes the effects of vasopressin on Na+ transport mediated by the amiloride sensitive epithelial sodium channel ( ENaC ) and the cystic fibrosis transmembrane conductance regulator ( CFTR ) Cl channel in immortalized or primary cultured cortical collecting duct cells , expressing either the wild type ENaC subunits , or mutations , or deletions of the PY domain of the beta or gamma ENaC subunits responsible for Liddle ' s syndrome , an inherited form of hypertension due to excessive salt absorption . . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Ion channels and transporters ( i . e . cystic fibrosis transmembrane regulator ( CFTR ) , inward rectifier potassium channel ( eKir ) , Na / K ATPase , Na / K / Cl2 co transporter ( NKCC ) , aquaporin 3 ( AQP 3 ) , and Na / H exchanger 1 ( NHE 1 ) ) are known to be expressed in gill epithelia of teleost fish . ^^^ Seawater acclimation induced the transcript and / or protein levels of Na / K ATPase , NKCC , CFTR , and eKir in CCs . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
While the effect of mutation of the cystic fibrosis transmembrane conductance regulator ( CFTR ) on airways remains controversial , some groups have demonstrated increases in Na ( + ) and Cl ( ) in CF airway surface liquid compared to normal airways . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Recent evidence suggests that 6 out of 7 FXYD proteins , FXYD 1 ( phospholemman ) , FXYD 2 ( gamma subunit of Na , K ATPase ) , FXYD 3 ( Mat 8 ) , FXYD 4 ( CHIF ) , FXYD 5 ( Ric ) and FXYD 7 associate with Na , K ATPase and modulate its transport properties e . g . its Na+ and / or its K+ affinity in a distinct way . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Accumulating evidence suggests that impaired mucus clearance in cystic fibrosis ( CF ) airways is a result of ASL depletion caused by excessive Na ( + ) absorption through the epithelial sodium channel ( ENaC ) . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Regulation of the CFTR Cl channel function involves a protein complex of activated protein kinase Cepsilon ( PKCepsilon ) bound to RACK 1 , a receptor for activated C kinase , and RACK 1 bound to the human Na ( + ) / H ( + ) exchanger regulatory factor ( NHERF 1 ) in human airway epithelial cells . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
METHODS / DESIGN : We adapted the standard NPD protocol for young children , designed a special catheter for them , used a slower perfusion rate , and shortened the protocol to include only measurement of basal PD , transepithelial sodium ( Na+ ) transport in response to the Na+ channel inhibitor amiloride , and CFTR mediated chloride ( Cl ) secretion in response to isoproterenol , a beta agonist in a Cl free solution . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
In vitro the ETKF motif of dra binds to the second PDZ domain of E3KARP , the affinity being comparable to that of the known ligand CFTR . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NHERFs have been implicated in the targeting , retention and regulation of membrane proteins including the beta ( 2 ) adrenergic receptor , cystic fibrosis transmembrane conductance regulator , and Trp 4 Ca ( 2+ ) channel , and NHERF 2 is now shown to also interact with PMCA2b . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
CFTR , NHE 3 , and ezrin were recognized by NHERF 2 but not NHERF 1 in glutathione S transferase pull down experiments . ^^^ We conclude that CFTR and NHE 3 reciprocally interact via a shared regulatory complex comprised of NHERF 2 , ezrin , and PKA . . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
One of the PDZ domains of NHERF 2 interacts specifically with the DSLL , DSFL , and DTRL motifs present at the carboxy termini of the 2 adrenergic receptor , the platelet derived growth factor receptor , and the cystic fibrosis transmembrane conductance regulator , respectively . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
Both NHERF proteins contain two PDZ domains ; recombinant protein protein binding assays and yeast two hybrid studies revealed that ROMK preferentially associates with the second PDZ domain of NHERF 1 and with the first PDZ domain of NHERF 2 , precisely opposite of what has been reported for CFTR . ^^^ Consistent with the scaffolding capacity of the NHERF proteins , coexpression of NHERF 2 with ROMK and CFTR dramatically increases the amount of ROMK protein that coimmunopurifies and functionally interacts with CFTR . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NHE 3 inhibits PKA dependent functional expression of CFTR by NHERF 2 PDZ interactions . ^^^ It has been shown that when CFTR and NHE 3 are co expressed on the apical membrane of the A 6 NHE3 cell monolayers , the two transporters interact via a shared regulatory complex composed of NHERF 2 , ezrin , and PKA . ^^^ When only CFTR is expressed on the apical membrane , deletion of any of the NHERF 2 binding domains inhibited both PKA dependent apical CFTR expression and its activation , while when NHE 3 was co expressed with CFTR PDZ 2 deletion was without effect on CFTR sorting and activity . ^^^
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
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NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA
Interacting proteins: Q15599 and P13569	Pubmed	SVM Score :0.0
NA