Pubmed abstracts for Protein-Protein Interaction search result :

Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.83920035
Here we report the cloning of the full length mouse K ( 5 ) LQT 1 complementary DNA and show that K ( 5 ) LQT 1 associates with IsK to form the channel underlying the 1 ( Ks ) cardiac current , which is a target of class 3 anti arrhythmic drugs and is involved in the LQT 1 syndrome . . 0.83920035^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.60311582
These results indicate that the IsK protein associates with both KvLQT 1 and ERG products to modulate IKr and IKs in cardiac myocytes . . 0.60311582^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.68227275
KCNE 1 associates with KCNQ 1 in vitro to generate a potassium current closely resembling the slowly activating delayed rectifier ( 1 ( Ks ) ) . 0.68227275^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.57818483
We observe that interaction between KCNQ 1 and KCNE 1 ( the beta subunit ) confers kinetic properties on IKs that make it suitable for participation in action potential repolarization and its adaptation to rate changes ; in particular , the channel develops an available reserve of closed states near the open state that can open rapidly on demand . 0.57818483^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.89683237
Association of minK with a channel protein known as KvLQT 1 produces a voltage gated outward K+ current ( 1 [ sK ] ) resembling the slow cardiac repolarization current ( 1 [ Ks ] ) . 0.89683237^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.63539626
There is controversy about the effects of the association between KvLQT 1 and minK on the single channel conductance . 0.63539626^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
The quaternary structure of the IminK channel complex has long remained a mystery , but recent studies suggest an interaction of the ISK protein with a traditional K+ channel subunit , identified in man as KVLQT 1 . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Co expression of KvLQT 1 with the IsK protein elicits slowly activating potassium currents resembling the cardiac Iks current . ^^^ We now show that IsK not only changes the kinetics of KvLQT 1 currents , but also its ion selectivity . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Injection into Xenopus oocytes of mRNA coding for the min K protein induces a similar current ( IsK ) and recent observations support the hypothesis that functional channels result from the association of the min K protein with an endogenous K+ channel similar to the recently cloned KvLQT 1 . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
It has been suggested that the cardiac slow delayed rectifier channel is formed by the association of two subunits : IsK ( also called minK ) and KvLQT 1 . ^^^ The function of tKvLQT 1 was tested by oocyte expression , alone or together with the full length KvLQT 1 or a human IsK clone ( hIsK ) . tKvLQT 1 alone did not generate functional channels . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Expression studies revealed that the association of KvLQT 1 with another subunit , IsK , reconstitutes a channel responsible for the IKs current involved in ventricular myocyte repolarization . ^^^ Six RW and two JLN mutated KvLQT 1 subunits were produced and co expressed with IsK in COS cells . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
IKs channels are composed of IsK and KvLQT 1 subunits and underly the slowly activating , voltage dependent IKs conductance in heart . ^^^ However , IKs channels induced by the coexpression of IsK and KvLQT 1 subunits have a 6 100 fold higher affinity for these blockers . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
IsK and KvLQT 1 : mutation in either of the two subunits of the slow component of the delayed rectifier potassium channel can cause Jervell and Lange Nielsen syndrome . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
KvLQT 1 potassium channel but not IsK is the molecular target for trans 6 cyano 4 ( N ethylsulfonyl N methylamino ) 3 hydroxy 2 , 2 dimethyl chromane . ^^^ Coexpression of KvLQT 1 in association with the channel regulator protein IsK produces a K+ current with characteristics reminiscent of the slow component of the delayed rectifier in cardiac myocytes . ^^^ We explored the pharmacological properties of trans 6 cyano 4 ( N ethylsulfonyl N methylamino ) 3 hydroxy 2 , 2 dime thyl chromane ( 293B ) , a chromanol compound , on the K+ current produced by direct intranuclear injection of KvLQT 1 and IsK cDNA plasmids in COS 7 cells . ^^^ Cells injected with KvLQT 1 cDNA alone exhibited a fast activating outward K+ current , whereas cells coinjected with KvLQT 1 plus IsK cDNAs exhibited a time dependent outward current with slower activation kinetics . ^^^ The chromanol 293B blocked the K+ current related to KvLQT 1 expression in both the absence or presence of IsK . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Vestibular dark cells ( VDC ) are known to electrogenically secrete K+ via slowly activating K+ ( IsK ) channels , consisting of IsK regulatory and KvLQT 1 channel subunits , and the associated short circuit current ( Isc ) is inhibited by agonists of the apical P2U ( P2Y2 ) receptor ( J . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
IsK , an apparent potassium channel subunit encoded by KCNE 1 on chromosome 21 , regulates both KVLQT 1 and HERG . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
KvLQT 1 gene product is associated with the regulator protein IsK to produce a component of the delayed rectifier K+ current in cardiac myocytes . ^^^ COS 7 cells injected intranuclearly with KvLQT 1 isoform 1 cDNA exhibited a fast activating K+ current , whereas those injected with a KvLQT 1 isoform 1 plus IsK cDNA showed a slow activating K+ current . ^^^ Our results indicate that KvLQT 1 isoform 2 exerts a pronounced negative dominance on isoform 1 channels and that the cardiac KvLQT 1 K+ channel complex is composed of at least three different proteins as follows : isoform 1 , isoform 2 , and IsK . . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
IsK ( minK ) protein , in concert with another channel protein KVLQT 1 , mediates a distinct , slowly activating , voltage gated potassium current across certain mammalian cell membranes . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Since all isoforms showed significant structural identity with KVLQT 1 ( 64 % identity in the transmembrane domains ) , which is known to associate with IsK , they were designated mKQT2 . 1 mKQT2 . 11 . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
It results from mutations in KVLQT 1 and ISK genes that encode the 2 subunits forming the K+ channel responsible for the cardiac and inner ear slowly activating component of the delayed rectifier K+ current ( IKs ) . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
However , unlike KCNQ 1 , KCNQ 2 and KCNQ 3 currents are not augmented by coexpression with the KCNQ 1 beta subunit , KCNE 1 ( minK , IsK ) . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
The KCNE 1 gene encodes a channel regulator IsK which in association with the KvLQT 1 K+ channel protein determines the slow component of the cardiac delayed rectifier current . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
MinK KvLQT 1 fusion proteins , evidence for multiple stoichiometries of the assembled IsK channel . ^^^ IsK , a slowly activating delayed rectifier K+ current through channels formed by the assembly of two channel proteins KvLQT 1 and MinK , modulates the repolarization of cardiac action potentials . ^^^ In contrast , expression of MK 24 and MKK 44 elicited current with activation kinetics and voltage dependence very similar to native IsK and identical to currents expressed by cells co transfected with independent MinK and KvLQT 1 cDNA . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
The IKs channel : coassembly of IsK ( minK ) and KvLQT 1 proteins . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
In the cardiac 1 ( KS ) channel complex comprising the alpha and beta subunits , KvLQT 1 and IsK , respectively , several mutations lead to a dominant negative loss of channel function . ^^^ Stilbenes and fenamates rescue the loss of 1 ( KS ) channel function induced by an LQT 5 mutation and other IsK mutants . ^^^ Here we show that binding of 1 ( KS ) channel activators , such as stilbenes and fenamates , to an extracellular domain flanking the human IsK transmembrane segment , restores normal 1 ( KS ) channel gating in otherwise inactive IsK C terminal mutants , including the naturally occurring LQT 5 mutant , D76N . ^^^ Owing to allosteric interactions , stilbene and fenamate compounds can rescue the dominant negative suppression of 1 ( KS ) produced by IsK mutations and thus , may have important therapeutic relevance for LQT syndrome . . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
KCNQ 1 ( KvLQT 1 ) interacts with the beta subunit KCNE 1 ( IsK , minK ) to form the slow , depolarization activated potassium current 1 ( Ks ) that is affected in some forms of cardiac arrhythmia . ^^^ KCNQ 1 ( KvLQT 1 ) interacts with the beta subunit KCNE 1 ( IsK , minK ) to form the slow , depolarization activated potassium current 1 ( Ks ) that is affected in some forms of cardiac arrhythmia . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
The KCNQ 1 gene encodes KvLQT 1 alpha subunits , which together with auxiliary IsK ( KCNE 1 , minK ) subunits form IK ( s ) K ( + ) channels . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Novel mutations in KvLQT 1 that affect Iks activation through interactions with Isk . ^^^ The R243H , R533W and R539W mutations induced a positive voltage shift of the channel activation but only when co expressed with IsK , pointing out the critical role of these positively charged residues in the modulation of the gating properties of KvLQT 1 by IsK . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Long Q T mutant ( KvLQT 1 ) K ( + ) channels associate with their regulatory subunit IsK to produce the slow component of the delayed rectifier potassium ( 1 ( Ks ) ) cardiac current . ^^^ We used RNase protection assays to determine the relative expression of KvLQT 1 isoforms 1 and 2 and IsK mRNAs in human ventricular layers . ^^^ Overall expression of KvLQT 1 and IsK genes was similar in the three layers . ^^^ COS 7 cells were intranuclearly injected with KvLQT 1 isoforms 1 or 2 plus IsK cDNAs , using two different isoform 2 to isoform 1 ratios . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Differential expression of KvLQT 1 and its regulator IsK in mouse epithelia . ^^^ KCNQ 1 encodes a pore forming K+ channel subunit termed KvLQT 1 which , in association with its regulatory beta subunit IsK ( also called minK ) , produces the slow component of the delayed rectifier cardiac K+ current . ^^^ We used in situ hybridization to localize KvLQT 1 and IsK mRNAs in various tissues from adult mice . ^^^ We showed that KvLQT 1 mRNA expression is widely distributed in epithelial tissues , in the absence ( small intestine , lung , liver , thymus ) or presence ( kidney , stomach , exocrine pancreas ) of its regulator IsK . ^^^ In the kidney and the stomach , however , the expression patterns of KvLQT 1 and IsK do not coincide . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Mice disrupted for the KvLQT 1 potassium channel regulator IsK gene accumulate mature T cells . ^^^ The IsK protein associates with KvLQT 1 potassium channels to generate the slow component of the outward rectifying K ( + ) current involved in human cardiac repolarization . ^^^ Mutations in either KCNE 1 ( encoding IsK ) or KCNQ 1 ( encoding KvLQT 1 ) genes have been associated with the long QT syndrome , a genetic disorder leading to prolonged cardiac repolarization and sudden death . ^^^ Both IsK and KvLQT 1 mRNA are expressed in murine thymus . ^^^ Our data suggest that , in addition to its role in myocardial repolarization , the IsK KvLQT 1 tandem also plays a crucial role in T cell homeostasis . . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
The K+ channel KCNQ 1 ( K ( 5 ) LQT 1 ) is a voltage gated K+ channel , coexpressed with regulatory subunits such as KCNE 1 ( IsK , mink ) or KCNE 3 , depending on the tissue examined . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
In heart 1 ( Ks ) is a heteromeric channel composed of KCNQ 1 ( KvLQT 1 ) and minK ( KCNE 1 , IsK ) . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Mutations of the KCNE 1 gene ( IsK , minK ) are related to hereditary forms of cardiac arrhythmias , so called long QT syndromes ( LQT ) . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
KCNE 1 ( IsK , minK ) co assembles with KCNQ 1 ( KvLQT 1 ) to form voltage dependent K ( + ) channels . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
We used a semi quantitative RT PCR technique to examine the relative expression of mRNAs for the voltage gated K ( + ) channel subunits , Kv1 . 2 , Kv1 . 4 , Kv1 . 5 , Kv2 . 1 , Kv4 . 2 , Kv4 . 3 , KvLQT 1 , HERG and IsK in samples of human atrial appendage . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
KCNE 1 ( IsK or MinK ) recapitulates 1 ( Ks ) when associated with KCNQ 1 , whereas it augments the amplitude of an 1 ( Kr ) like current when co expressed with HERG . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
It is known to be associated with mutations of the genes KCNQ 1 ( KVQTI ) and KCNE 1 ( Isk ) . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Functional studies of the KvLQT 1 V307L mutant ( alone or coexpressed with the wild type channel , in the presence of IsK ) revealed a pronounced shift of the half activation potential and an acceleration of the activation kinetics leading to a gain of function in 1 ( Ks ) . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Besides , a slight reduction in channel activity was observed when coexpressed with WT KvLQT 1 and IsK . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Mutations in the potassium channel gene KVLQT 1 ( LQTS 1 ) have been identified in JLNS and in autosomal dominant LQTS as well . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Autosomal recessive LQT , which is associated with deafness , has been found to occur with homozygous mutations in KVLQT 1 and KCNE 1 in JLNS families in which QTc prolongation was inherited as a dominant trait . ^^^ This deletion represents a new JLNS associated mutation in KVLQT 1 and has deleterious effects on the KVLQT 1 potassium channel , causing a frameshift and the truncation of the KVLQT 1 protein . ^^^ CONCLUSIONS : A novel homozygous KVLQT 1 mutation causes JLNS in an Amish family with deafness that is inherited as an autosomal recessive trait . . ^^^ Both autosomal dominant LQT ( Romano Ward syndrome ) and autosomal recessive LQT ( Jervell and Lange Nielsen syndrome , JLNS ) have been reported . ^^^ METHODS AND RESULTS : An Amish family with clinical evidence of JLNS was analyzed for mutations by use of single strand conformation polymorphism and DNA sequencing analyses for mutations in all known LQT genes . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
KCNQ 1 seems to be the major gene in JLNS . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Two families with the autosomal recessive Jervell and Lange Nielsen syndrome ( JLNS ) , and one family with the autosomal dominant Romano Ward syndrome ( RWS ) were evaluated for mutations in KCNQ 1 . ^^^ Two different novel frameshift mutations were discovered in one of the JLNS families ( 1188delC ) and in the RWS family ( 504delG ) . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
In a study of ten British and Norwegian families with JLNS , we have identified all of the mutations in the KCNQ 1 gene , including two that are novel . ^^^ Truncation of the protein proximal to the recently identified C terminal assembly domain is expected to preclude assembly of KCNQ 1 monomers into tetramers and explains the recessive inheritance of JLNS . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
METHODS : The potassium channel gene KCNQ 1 was sequenced in two unrelated Finnish patients with Jervell and Lange Nielsen syndrome ( JLNS ) , followed by genotyping of 114 LQTS probands and their available family members . ^^^ CONCLUSIONS : A single missense mutation of the KCNQ 1 gene accounts for 30 % of Finnish cases with LQTS , and it may be associated with both the RWS and JLNS phenotypes of the syndrome . ^^^ This mutation was present in homozygous form in two siblings with JLNS , and in heterozygous form in 34 of 114 probands with Romano Ward syndrome ( RWS ) and 282 family members . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Previously homozygous mutations in the potassium channel encoding genes , KvLQT 1 ( alpha subunit ) and KCNE 1 ( beta subunit ) , have been described in consanguineous families with JLNS . ^^^ We screened two nonconsanguineous families with JLNS for mutations in KvLQT 1 , using single strand conformation polymorphism analysis , denaturing high performance liquid chromatography , and DNA sequencing . ^^^ In this report we provide evidence that not only homozygous but also compound heterozygous mutations in KvLQT 1 may cause JLNS in nonconsanguineous families . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
In general , heterozygous mutations in KCNQ 1 cause Romano Ward syndrome ( LQT 1 only ) , while homozygous mutations cause JLNS ( LQT 1 and deafness ) . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
JLNS is associated with sensorineural deafness and has been shown to occur with homozygous mutations in KCNQ 1 or KCNE 1 in JLNS families in which QTc prolongation is inherited as a dominant trait . ^^^ CONCLUSION : Novel compound heterozygous nonsense mutations in C terminus of KCNQ 1 can cause JLNS . . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Patients with mutations in KCNQ 1 may show only the cardiac defect ( Romano Ward syndrome or RWS ) or may also have severe deafness ( Jervell and Lange Nielsen syndrome or JLNS ) . ^^^ Targeted disruption of mouse Kcnq 1 models JLNS in that mice are deaf and show abnormal ECGs . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
KCNQ 1 is associated with two different entities of LQTS , the autosomal dominant Romano Ward syndrome ( RWS ) , and the autosomal recessive Jervell and Lange Nielsen syndrome ( JLNS ) characterized by bilateral deafness in addition to cardiac arrhythmias . ^^^ KCNQ 1 is associated with two different entities of LQTS , the autosomal dominant Romano Ward syndrome ( RWS ) , and the autosomal recessive Jervell and Lange Nielsen syndrome ( JLNS ) characterized by bilateral deafness in addition to cardiac arrhythmias . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
RESULTS : We identified a novel mutation N1774S in the SCN5A gene of the BS family , a novel mutation G314S in a RWS family which had also been found in Europe , North America , and Japan , and a single nucleotide polymorphisms ( SNPs ) G643S in the KCNQ 1 of the JLNS family . ^^^ In this JLNS family , another heterozygous novel mutation in exon 2a was found in KCNQ 1 of the patients . ^^^ OBJECTIVE : To investigate the molecular pathology in families with long QT syndrome ( LQTS ) including Jervell Longe Nielsen syndrome ( JLNS ) and Romano ward syndrome ( RWS ) and Brugada syndrome ( BS ) in Chinese population . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Loss of function mutations in the human KCNQ 1 gene have been linked to Jervell and Lange Nielsen Syndrome ( JLNS ) , a disorder characterized by profound bilateral deafness and a cardiac phenotype . ^^^ To generate a mouse model for JLNS , we created a line of transgenic mice that have a targeted disruption in the Kcnq 1 gene . ^^^ Together , these data suggest that Kcnq 1 ( / ) mice are a potentially valuable animal model of JLNS . . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
INTRODUCTION : Inherited long QT syndrome ( LQTS ) recently has been associated with mutations in genes coding for potassium ( KVLQT 1 , KCNE 1 , and HERG ) or sodium ( SCN5A ) ion channels involved in regulating either sodium inward or potassium outward currents of heart cells , resulting in prolongation of the repolarization period . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Genomic structure of three long QT syndrome genes : KVLQT 1 , HERG , and KCNE 1 . ^^^ Mutations in KVLQT 1 , HERG , SCN5A , and KCNE 1 , genes encoding cardiac ion channels , cause LQT . ^^^ Familial and sporadic cases affected bymutations in KVLQT 1 , HERG , and KCNE 1 can nowbe genetically screened to identify individuals at risk of developing this disorder . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Defects have been identified in the KCNQ 1 , HERG , and KCNE 1 genes , whose proteins form the K+ channels for the slowly and rapidly inwardly rectifying K+ currents IKs and IKr . ^^^ Depending on their location and copy number , mutations of KCNQ 1 and KCNE 1 can cause either autosomal dominant Romano Ward syndrome or autosomal recessive Jervell and Lange Nielsen syndrome . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Four genes have been identified for the cardiac potassium ( KCNQ 1 , HERG and KCNE 1 ) and sodium ( SCN5A ) . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Since the resulting 142 amino acid peptide has a single putative transmembrane domain and a weak but suggestive homology with KCNE 1 ( minK ) , a protein associated with the KCNQ 1 potassium channel ( KVLQT 1 ) , we named this new gene KCNE 1 like ( KCNE1L ) . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
The voltage gated potassium channel KCNQ 1 associates with the small KCNE 1 subunit to form the cardiac IKs delayed rectifier potassium current and mutations in both genes can lead to the long QT syndrome . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
The deafness associated Jervell and Lange Nielsen syndrome ( JLNS ) mutation KCNE 1 ( D76N ) impairs KCNQ 4 function whereas the Romano Ward syndrome ( RWS ) mutant KCNE 1 ( S74L ) , which shows normal hearing in patients , does not impair KCNQ 4 channel function . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Recent physiological studies suggest that KCNE 1 encodes beta subunits ( hminK ) that co assemble with KvLQT 1 alpha subunits to form the slowly activating delayed rectifier K+ ( IKs ) channel . ^^^ Because KVLQT 1 mutations cause arrhythmia susceptibility in the long QT syndrome ( LQT ) , we hypothesized that mutations in KCNE 1 also cause this disorder . ^^^ Here , we define KCNE 1 missense mutations in affected members of two LQT families . ^^^ This is the first description of KCNE 1 as an LQT gene and confirms that hminK is an integral protein of the IKs channel . . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
More than 35 mutations in four cardiac ion channel genes KVLQT 1 ( voltage gated K channel gene causing one of the autosomal dominant forms of LQTS ) ( LQT 1 ) , HERG ( human ether a go go related gene . ) ( LQT 2 ) , SCN5A ( LQT 3 ) , and KCNE 1 ( minK , LQT 5 ) have been identified in LQTS . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
KVLQT 1 , HERG , SCN5A , KCNE 1 , and KCNE 2 . ^^^ Five genes have been implicated in Romano Ward syndrome , the autosomal dominant form of LQTS : KVLQT 1 , HERG , SCN5A , KCNE 1 , and KCNE 2 . ^^^ Mutations in KVLQT 1 and KCNE 1 also cause the Jervell and Lange Nielsen syndrome , a form of LQTS associated with deafness , a phenotypic abnormality inherited in an autosomal recessive fashion . ^^^ CONCLUSIONS : KVLQT 1 ( 42 % ) and HERG ( 45 % ) accounted for 87 % of identified mutations , and SCN5A ( 8 % ) , KCNE 1 ( 3 % ) , and KCNE 2 ( 2 % ) accounted for the other 13 % . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Fire genes , KCNQ 1 , HERG , SCN5A , KCNE 1 , and KCNE 2 , have been identified . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Whole cell , patch clamp experiments were conducted on COS 7 cells transfected with wild type and / or R259C KCNQ 1 with or without KCNE 1 . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
KvLQT 1 is a Shaker like voltage gated potassium channel that when complexed with minK ( KCNE 1 ) produces the slowly activating delayed rectifier 1 ( ks ) . ^^^ The emerging family of KCNE 1 related peptides includes KCNE 1 and KCNE 3 , both of which complex with KvLQT 1 to produce functionally distinct currents . ^^^ We exploited these functional differences and the general structural similarities of KCNE 1 and KCNE 3 to study which physical regions are critical for control of KvLQT 1 by making chimerical constructs of KCNE 1 and KCNE 3 . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Stimulation of M 1 receptors by 10 microM oxotremorine M ( Oxo M ) strongly reduced ( to 0 10 % ) currents produced by KCNQ 1 4 subunits expressed individually and also those produced by KCNQ 2 + KCNQ 3 and KCNQ 1 + KCNE 1 heteromers , which are thought to generate neuronal M currents ( IK , M ) and cardiac slow delayed rectifier currents ( IK , s ) , respectively . 3 . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
We report here 20 single nucleotide polymorphisms ( SNPs ) , including 10 novel ones , and their allelic frequencies detected in four genes that are known to be responsible for familial long QT syndrome in the Japanese population ; 7 polymorphisms are in the KCNQ 1 gene , 6 in the KCNH 2 gene , 5 in the SCN5A gene , and 2 in the KCNE 1 gene . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
All code for subunits of sodium or potassium channels : two a subunits of the potassium channels ( QVLQT 1 for LQT 1 , HERG for LQT 2 ) , the a subunit of the sodium channel INa ( SCN5A for LQT 3 ) , and two regulatory subunits of potassium channels ( KCNE 1 for LQT 5 regulating the KvLQT 1 channel and MiRP 1 regulating HERG ) . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
TEA ( + ) sensitive KCNQ 1 constructs reveal pore independent access to KCNE 1 in assembled 1 ( Ks ) channels . 1 ( Ks ) , a slowly activating delayed rectifier K ( + ) current through channels formed by the assembly of two subunits KCNQ 1 ( KvLQT 1 ) and KCNE 1 ( minK ) , contributes to the control of the cardiac action potential duration . ^^^ Previous investigations of external access to the KCNE 1 protein in assembled 1 ( Ks ) channels relied on occlusion of the pore by extracellular application of TEA ( + ) , despite the very low TEA ( + ) sensitivity ( estimated EC ( 50 ) > 100 mM ) of channels encoded by coassembly of wild type KCNQ 1 with the wild type ( WT ) or a series of cysteine mutated KCNE 1 constructs . ^^^ We have engineered a high affinity TEA ( + ) binding site into the h KCNQ 1 channel by either a single ( V319Y ) or double ( K318I , V319Y ) mutation , and retested it for pore delimited access to specific sites on coassembled KCNE 1 subunits . ^^^ Coexpression of either KCNQ 1 construct with WT KCNE 1 in Chinese hamster ovary cells does not alter the TEA ( + ) sensitivity of the homomeric channels ( IC ( 50 ) approximately 0 . 4 mM [ TEA ( + ) ] ( out ) ) , providing evidence that KCNE 1 coassembly does not markedly alter the structure of the outer pore of the KCNQ 1 channel . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
KCNE 1 reverses the response of the human K+ channel KCNQ 1 to cytosolic pH changes and alters its pharmacology and sensitivity to temperature . ^^^ In this study we report on the divergent responses to internal pH changes and further characterize some of the current properties of the human isoforms of KCNQ 1 and KCNE 1 expressed in Chinese hamster ovary ( CHO ) cells or Xenopus laevis oocytes . ^^^ These results show that coexpression of KCNE 1 reversed pH regulation of KCNQ 1 from inhibition to activation by acidic pHi . ^^^ In addition , KCNE 1 altered the pharmacological properties and sensitivity to temperature of KCNQ 1 . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
The putative KCNQ 1 subunits , KCNE 2 and KCNE 3 , were abundant in human stomach ; KCNE 1 , however , was absent . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
To clarify this situation we injected adenoviral vectors expressing wild type or LQT mutants of HERG and KCNE 1 into guinea pig myocardium . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Finally , genetic studies were performed prospectively in 16 consecutive patients , to look for HERG , KCNE 1 , KCNE 2 and KCNQ 1 mutations . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
KCNQ 1 , the first member of a new K+ channel family , associates with the small KCNE 1 subunit to form the slow cardiac delayed rectifier current , IKs . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
The voltage dependent K ( + ) channel responsible for the slowly activating delayed K ( + ) current 1 ( Ks ) is composed of pore forming KCNQ 1 and regulatory KCNE 1 subunits , which are mutated in familial forms of cardiac long QT syndrome . ^^^ Because KCNQ 1 and KCNE 1 genes also are expressed in epithelial tissues , such as the kidneys and the intestine , we have investigated the adaptation of KCNE 1 deficient mice to different K ( + ) and Na ( + ) intakes . ^^^ In addition , we found that KCNE 1 and KCNQ 1 mRNAs are expressed in the zona glomerulosa of adrenal glands where 1 ( Ks ) may directly participate in the control of aldosterone production by plasma K ( + ) . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
KCNQ 1 ( KvLTQ 1 ) is co assembled with the product of the KCNE 1 ( minimal K ( + ) channel protein ) gene in the heart to form a cardiac delayed rectifier like K ( + ) current . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Heterozygous mutations in genes encoding cardiac ionic channel subunits KCNQ 1 , HERG , SCN5A , KCNE 1 , and KCNE 2 are causally involved in the dominant form of long QT syndrome ( LQTS ) while homozygous mutations in KCNQ 1 and KCNE 1 cause LQTS with or without congenital deafness . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Several ion channels , including a slowly activating , voltage dependent , outwardly conducting K ( + ) channel composed of the KCNQ 1 ( KvLQT 1 ) and KCNE 1 ( IsK / minK ) subunits , are expressed at the apical surface of vestibular dark cells . ^^^ In the wild type mice , the KCNE 1 and KCNQ 1 proteins are expressed specifically at the apical membrane of dark cells , as early as gestational day ( GD ) 17 for KCNE 1 while KCNQ 1 mRNAs can be detected at GD 18 . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Mutations in any of the five genes KCNQ 1 , KCNH 2 , KCNE 1 , KCNE 2 , and SCN5A can be responsible for familial long QT syndrome ( LQTS ) , an arrhythmogenic disorder that entails a high risk of sudden death . beta Adrenergic blocking agents are the first therapeutic choice , and 80 % of patients treated with these agents show symptomatic relief ; however the remaining 20 % do not respond well . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Mutation analysis has established the presence of mutations in affected individuals in the genes KCNQ 1 and KCNE 1 : the potassium channel complex responsible for the cardiac 1 ( Ks ) current involved in repolarisation of the ventricular action potential . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
The slow ( 1 ( Ks ) ) and fast ( 1 ( Kr ) ) components of the delayed rectifier cardiac K ( + ) current are generated by pore forming alpha subunits KCNQ 1 and KCNH 2 , respectively , in association with regulatory beta subunit KCNE 1 , KCNE 2 and perphaps KCNE 3 . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Here it was demonstrated by immunohistochemistry that KCNE 1 and KCNQ 1 , which form together the slowly activated component of the delayed rectifying K+ current in the heart , also colocalize in the luminal membrane of proximal tubule in mouse kidney . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
XE 991 can also block KCNQ 1 K ( + ) channels expressed in oocytes , but sensitivity is reduced when the channels are coexpressed with minK ( KCNE 1 ) . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Mutations in the human minK gene KCNE 1 have been linked to autosomal dominant and autosomal recessive long QT ( LQT ) syndrome , a cardiac condition predisposing to ventricular arrhythmias . minK and KvLQT 1 , the LQT 1 gene product , form a native cardiac K+ channel that regulates the slowly delayed rectifier potassium current 1 ( Ks ) . ^^^ We used single strand conformation polymorphism and sequencing techniques to identify novel KCNE 1 mutations in patients with a congenital LQT syndrome of unknown genetic origin . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
One potassium channel , KCNQ 1 , and its regulator , KCNE 1 , are expressed in salivary glands and kidneys , and KCNE 1 null mutant mice are deficient in KCNQ 1 potassium currents . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
We found that GC could express multiple voltage dependent K ( + ) ( Kv ) channel subunits , including KCNQ 1 , KCNE 1 , Kv1 . 1 , Kv1 . 2 , Kv1 . 3 , Kv1 . 4 , Kv1 . 5 , Kv1 . 6 , Kvbeta1 . 3 , and Kvbeta 2 . ^^^ KCNE 1 and KCNQ 1 were coassociated in GC , and their expression coincided with the expression of 1 ( Ks ) . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Coexpression of WT KCNQ 1 and G643S KCNQ 1 with KCNE 1 resulted in approximately 30 % reduction in the slow delayed rectifier K+ current 1 ( Ks ) without much alteration in the kinetic properties except its deactivation process , suggesting that the G643S substitution had a weaker dominant negative effect on the heteromultimeric channel complexes . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Mutations in KCNQ 1 , KCNH 2 , KCNE 1 , KCNE 2 , and SCN5A genes encoding cardiac potassium and sodium ion channels cause LQT . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Since there are no cysteine residues in the extracellular domain of KCNE 1 , it has been proposed that TMS interacts with its partner protein KCNQ 1 . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
The specificity of KCNE 1 ( minK ) and KCNE 3 control of activation of the potassium channel KvLQT 1 maps to a triplet of amino acids within the KCNE transmembrane domain by chimera analysis . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
The importance of K+ cycling is underscored by the fact that mutations of KCNQ 1 , KCNE 1 , KCNQ 4 , GJB 2 , GJB 3 and GJB 6 lead to deafness in humans and that null mutations of KCNQ 1 , KCNE 1 , KCNJ 10 and SLC12A2 lead to deafness in mouse models . . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
This investigation was undertaken to determine which of the two structural units reconstituting the 1 ( Ks ) channel , KCNQ 1 ( KvLQT 1 ) and KCNE 1 ( minK / IsK ) , plays a key role in the cell swelling induced 1 ( Ks ) enhancement and to dissect a possible involvement of tyrosine phosphorylation therein . ^^^ KCNQ 1 was transiently expressed alone or together with KCNE 1 in a heterologous mammalian cell line . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Expression and coassociation of ERG 1 , KCNQ 1 , and KCNE 1 potassium channel proteins in horse heart . ^^^ In horse heart , we have used immunoblotting and immunostaining to demonstrate the expression of ERG 1 , KCNQ 1 , KCNE 1 , and KCNE 3 proteins and RT PCR to detect KCNE 2 message . ^^^ Both ERG 1 and KCNQ 1 coimmunoprecipitated with KCNE 1 . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Mutations in KCNQ 1 and KCNE 1 , the alpha and beta subunits of the 1 ( KS ) K+ channel , produce the cardiac long QT ( LQT ) syndrome . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Heterozygous mutations in KCNQ 1 , HERG , SCN5A , KCNE 1 and KCNE 2 genes are responsible for the dominant form without deafness whereas homozygous mutations in KCNQ 1 and KCNE 1 are responsible for the recessive form ( Jervell and Lange Nielsen syndrome ) associated with congenital deafness . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Deafness results also from mutations of KCNQ 1 or KCNE 1 , subunits of a K ( + ) channel that carries K ( + ) from strial marginal cells and vestibular dark cells into endolymph . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Recently , defect alleles in the genes for KCNQ 1 and KCNE 1 have been identified in patients with the J LN syndrome . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Except for KCNE 1 , Northern blots ( KCNQ 1 , MERG , Kv1 . 5 , connexins 40 and 43 , TREK 1 , and TASK 1 ) did not detect sex differences . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
We speculate that since KCNE 5 is expressed in cardiac tissue it may here along with the KCNE 1 beta subunit regulate KCNQ 1 channels . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Speed and sensitivity of mutation detection was improved by applying the denaturing high performance liquid chromatography ( DHPLC ) technique for analysis of the entire KCNQ 1 and KCNH 2 genes and the protein encoding part of the KCNE 1 and KCNE 2 genes . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
LQTS is caused by mutations in ion channel genes including the cardiac sodium channel gene SCN5A , and potassium channel subunit genes KCNQ 1 , KCNH 2 , KCNE 1 , and KCNE 2 . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
It consists of an assembly of two structurally distinct alpha and beta subunits called KCNQ 1 and KCNE 1 , respectively . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Coordinated down regulation of KCNQ 1 and KCNE 1 expression contributes to reduction of 1 ( Ks ) in canine hypertrophied hearts . ^^^ The present study was designed to probe the molecular basis for the decrease in 1 ( Ks ) by studying the characteristics of KCNE 1 and KCNQ 1 , the putative genes responsible for formation of the channel . ^^^ METHODS AND RESULTS : Using a combination of Northern blot , competitive multiplex PCR and immunoblot assays , we found that CAVB reduces KCNE 1 and KCNQ 1 RNA in the canine ventricles by 70 and 80 % , respectively . ^^^ Protein levels of KCNE 1 and KCNQ 1 were reduced by 60 and 50 % , respectively . ^^^ CONCLUSIONS : Our results indicate that the CAVB induced reduction in 1 ( Ks ) is due to a down regulation of KCNE 1 and KCNQ 1 transcription . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Mutations in either KCNQ 1 or KCNE 1 , the 1 ( Ks ) subunits , are associated with variants ( LQT 1 and LQT 5 ) of the congenital long QT syndrome . ^^^ We now show that cAMP mediated functional regulation of KCNQ1 / KCNE1 channels , a consequence of cAMP dependent protein kinase A phosphorylation of the KCNQ 1 N terminus , requires coexpression of KCNQ 1 with KCNE 1 , its auxiliary subunit . ^^^ Further , at least two KCNE 1 mutations linked to LQT 5 ( D76N and W87R ) cause functional disruption of cAMP mediated KCNQ1 / KCNE1 channel regulation despite the response of the substrate protein ( KCNQ 1 ) to protein kinase A phosphorylation . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Microarray analysis revealed that hypothyroidism induces significant reductions in KCNA 5 , KCNB 1 , KCND 2 , and KCNK 2 transcripts , whereas KCNQ 1 and KCNE 1 expression is increased . ^^^ In hyperthyroidism , in contrast , KCNA 5 and KCNB 1 expression is increased and KCNQ 1 and KCNE 1 expression is decreased . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
HERG encodes the alpha subunit of channels underlying 1 ( Kr ) , while 1 ( Ks ) is composed of subunits encoded by KCNQ 1 and KCNE 1 . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
KCNE 1 induced current was markedly upregulated by coexpression of KCNQ 1 and further increased by additional expression of ( S422D ) SGK 1 , SGK 2 , SGK 3 or ( T308D , S473D ) PKB . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
The sensitivity of the KCNQ 1 and KCNQ 4 channels to cell volume changes is independent of the presence of the auxiliary KCNE 1 3 subunits , although modulated by KCNE 1 in the case of KCNQ 1 . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Compared with wild type KCNE1 / KCNQ1 channels , coexpression of G52R KCNE 1 with KCNQ 1 in Xenopus oocytes did not amplify the KCNQ 1 current amplitudes and slightly changed the activation kinetics of the KCNQ 1 channels . ^^^ Coexpression of KCNQ 1 together with wild type KCNE 1 and G52R KCNE 1 reduced the wild type 1 ( ks ) current amplitude by 50 % , whereas other biophysical properties of the 1 ( ks ) were not altered . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Rb+ inward currents for channels formed by co assembly of KCNQ 1 with KCNE 1 , KCNE 3 and KCNE 5 , and for homomeric KCNQ 1 channels with point mutations in the pore helix S 5 or S 6 transmembrane domains . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
KCNQ 1 alpha subunits coassemble with KCNE 1 beta subunits to form channels that conduct the slow delayed rectifier K+ current ( IKs ) important for repolarization of the cardiac action potential . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Multiple mutations in several ion channel genes ( KCNQ 1 , KCNH 2 , SCN5A , KCNE 1 , KCNE 2 , and KCNJ 2 ) have been shown to cause autosomal dominant long QT syndrome ( LQTS ) , a familial cardiac disorder that causes syncope , seizures , and sudden death . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
SUBJECTS AND METHODS : Genomic DNA from 744 apparently healthy individuals 305 black , 187 white , 134 Asian , and 118 Hispanic was subject to a comprehensive mutational analysis of the 4 LQTS causing potassium channel genes : KCNQ 1 ( LQT 1 ) , KCNH 2 ( LQT 2 ) , KCNE 1 ( LQT 5 ) , and KCNE 2 ( LQT 6 ) . ^^^ RESULTS : Overall , 49 distinct amino acid altering variants ( 36 novel ) were identified : KCNQ 1 ( n = 16 ) , KCNH 2 ( n = 25 ) , KCNE 1 ( n = 5 ) , and KCNE 2 ( n = 3 ) . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Several of these mutations have been reported on the KCNQ 1 gene encoding for a potassium channel or its regulatory subunit ( KCNE 1 ) . ^^^ Potassium currents were recorded from CHO cells transfected with either wild type or mutant KCNQ 1 in the presence or in the absence of its regulatory subunit ( KCNE 1 ) , using the whole cell configuration of the patch clamp technique . ^^^ Wild type KCNQ 1 current amplitudes are increased particularly by KCNE 1 co expression but no current is observed with the KCNQ 1 ( L251P ) mutant either in the presence or in the absence of KCNE 1 . ^^^ Coexpressing KCNE 1 with equal amount of cDNAs encoding wild type and mutant KCNQ 1 results in an 11 fold reduction in the amplitude of potassium currents . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
METHODS AND RESULTS : CHO cells were transfected with KCNQ 1 cDNA ( encoding the pore forming subunit ) with or without the ancillary subunit KCNE 1 . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
To date , 6 loci have been identified with the genes responsible for the forms LQT 1 , LQT 2 , LQT 5 and LQT 6 , coding for the potassium channels ( KCNQ 1 , HERG , KCNE 1 and KCNE 2 , respectively ) which , in the heterozygote state , are responsible for the main forms of LQTS without deafness and , in the homozygote state ( KCNQ 1 and KCNE 1 ) for the recessive forms of LQTS with or without deafness . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Genetic variations of KCNQ 1 , KCNH 2 , SCN5A , KCNE 1 , and KCNE 2 in drug induced long QT syndrome patients . ^^^ Five cLQTS genes ( KCNH 2 , KCNQ 1 , SCN5A , KCNE 1 , KCNE 2 ) were thoroughly screened for genetic variations in 32 drug induced aLQTS patients with confirmed TdP and 32 healthy individuals . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
METHODS AND RESULTS : Genetic analyses of KVLQT 1 , HERG , KCNE 1 , KCNE 2 , and SCN5A detected compound mutations in 20 of 252 LQTS probands ( 7 . 9 % ) . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Accordingly , defective KCNE 1 and / or KCNQ 1 lead to long QT syndrome , a disorder causing fainting and sudden cardiac death . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
METHODS : We performed analysis of KCNQ 1 ( KVLQT 1 ) , KCNH 2 ( HERG ) , SCN5A , KCNE 1 , and KCNE 2 defects in a subgroup of 12 adult subjects with unexplained sudden death , derived from a 13 year , 270 patient autopsy series of SCD . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Further biochemical studies ruled out three candidate proteins , namely KCNE 1 , yotiao , and KCNQ 1 itself , as effectors of this coiled coil mediated trafficking . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Whole cell patch clamp techniques were employed to examine the effects of bepridil , a Ca2+ channel blocker with Vaughan Williams class 3 action , on a slow component of cardiac delayed rectifier K+ current ( IKs ) , which was reconstituted in HEK 293 cells by transfecting KCNQ 1 and KCNE 1 . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
LQT 5 by invalidating the Kcne 1 K+ channel beta subunit and the Andersen syndrome by invalidation of the KCNJ 2 gene encoding for a cardiac inward rectifier K+ channel . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
BACKGROUND : Mutations in five cardiac voltage gated ion channel genes , including KCNQ 1 , HERG , SCN5A , KCNE 1 and KCNE 2 , constitute the principal cause of inherited long QT syndrome ( LQTS ) . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
KCNE 1 binds to the KCNQ 1 pore to regulate potassium channel activity . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
The cardiac slow delayed rectifier potassium channel ( IKs ) , comprised of ( KCNQ 1 ) and beta ( KCNE 1 ) subunits , is regulated by sympathetic nervous stimulation , with activation of beta adrenergic receptors PKA phosphorylating IKs channels . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
So far , six genes ( KCNQ 1 , HERG , SCN5A , ANK 2 , KCNE 1 , KCNE 2 ) have been demonstrated to be involved in the development of LQTS . ^^^ Furthermore , two small genes KCNE 1 and KCNE 2 ( at the LQT 5 and LQT 6 loci ) , and the SGK 1 gene ( encoding a kinase regulating KCNE 1 and SCN5A channels ) were sequenced . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Endolymphatic K+ concentrations were normal and membrane proteins necessary for K+ secretion were present , including the K+ channel KCNQ 1 and KCNE 1 , Na+ / 2Cl / K+ cotransporter SLC12A2 and the gap junction GJB 2 . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
We evaluated 28 unrelated Chinese kindreds with AF and sequenced eight genes of potassium channels ( KCNQ 1 , HERG , KCNE 1 , KCNE 2 , KCNE 3 , KCNE 4 , KCNE 5 , and KCNJ 2 ) . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
We show that KCNQ 1 and KCNE 1 are associated in testis and that their expression is closely regulated during development . ^^^ KCNQ 1 and KCNE 1 were also studied in various germ cell pathologies . ^^^ In these samples , KCNQ 1 and KCNE 1 were not expressed . ^^^ Our results demonstrate that the expression of KCNQ 1 and KCNE 1 is associated with early stages of spermatogenesis and with the presence of undifferentiated healthy or neoplastic germ cells . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Comprehensive mutational analysis of the 5 LQTS causing channel genes , KCNQ 1 ( LQT 1 ) , KCNH 2 ( LQT 2 ) , SCN5A ( LQT 3 ) , KCNE 1 ( LQT 5 ) , and KCNE 2 ( LQT 6 ) , along with KCNJ 2 ( Andersen Tawil syndrome ) and targeted analysis of 18 CPVT 1 associated exons in RyR 2 , was performed with the use of denaturing high performance liquid chromatography and direct DNA sequencing . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Genetic studies have identified mutations in six ion channel genes , KCNQ 1 , KCNH 2 , SCN5A , KCNE 1 and KCNE 2 and the accessory protein Ankyrin B gene , to be responsible for this disorder . ^^^ Co expression of mutant ( KCNQ 1 P343S ) and wild type ( KCNQ 1 ) cRNA in Xenopus oocytes produced potassium currents reduced by approximately 92 % , while IKs reconstitution experiments with a combination of KCNQ 1 mutant , wild type and KCNE 1 subunits yielded currents reduced by approximately 60 % . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
A kinase anchoring proteins ( AKAPs ) are thought to be passive members of protein complexes that coordinate the association of cAMP dependent protein kinase A ( PKA ) with cellular substrates to facilitate targeted PKA protein phosphorylation . 1 ( Ks ) , the slow heart potassium current , is carried by the 1 ( Ks ) potassium channel , a substrate for PKA phosphorylation in response to sympathetic nerve stimulation , is a macromolecular complex that includes the KCNQ 1 alpha subunit , the KCNE 1 regulatory subunit , and the AKAP Yotiao . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
However , these results are in stark contrast with earlier KCNE 1 deletion studies , which demonstrated that a COOH terminal region , highly conserved between KCNE 1 and KCNE 3 , was responsible for KCNE 1 modulation of KCNQ 1 ( Tapper , A . ^^^ To ascertain whether KCNE 3 peptides behave similarly to KCNE 1 , we examined a panel of NH ( 2 ) and COOH terminal KCNE 3 truncation mutants to directly determine the regions required for assembly with and modulation of KCNQ 1 channels . ^^^ These results demonstrate that the KCNE 3 transmembrane domain is sufficient for assembly with and modulation of KCNQ 1 channels and suggests a bipartite model for KCNQ 1 modulation by KCNE 1 and KCNE 3 subunits . ^^^ In this model , the KCNE 3 transmembrane domain is active in modulation and overrides the COOH terminus ' contribution , whereas the KCNE 1 transmembrane domain is passive and reveals COOH terminal modulation of KCNQ 1 channels . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
We screened a white population for single nucleotide polymorphisms ( SNPs ) in five long QT syndrome genes , namely , KCNQ 1 ( LQT 1 ) , HERG ( LQT 2 ) , SCN5A ( LQT 3 ) , KCNE 1 ( LQT 5 ) , and KCNE 2 ( LQT 6 ) . ^^^ Ten SNPs were in KCNE 1 , six in HERG , eight in KCNQ 1 , four in KCNE 2 , and seven in SCN5A . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
METHODS : A cardiac channel gene screen for LQTS causing mutations in KCNQ 1 ( LQT 1 ) , KCNH 2 ( LQT 2 ) , SCN5A ( LQT 3 ) , KCNE 1 ( LQT 5 ) , and KCNE 2 ( LQT 6 ) was performed for 541 consecutive , unrelated patients ( 358 females , average age at diagnosis 24 + / 16 years , average QTc 482 + / 57 ms ) referred to Mayo Clinic ' s Sudden Death Genomics Laboratory for LQTS genetic testing between August 1997 and July 2004 . ^^^ RESULTS : Overall , 211 putative pathogenic mutations in KCNQ 1 ( 88 ) , KCNH 2 ( 89 ) , SCN5A ( 32 ) , KCNE 1 ( 1 ) , and KCNE 2 ( 1 ) were found in 272 unrelated patients ( 50 % ) . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
We show here that mice with deletion of the KCNQ 1 protein partner KCNE 1 have spontaneous episodes of atrial fibrillation despite normal atrial size and structure . ^^^ Whereas loss of KCNE 1 function prolongs ventricular action potentials in humans , KCNE 1 / mice displayed unexpectedly shortened atrial action potentials , and multiple potential mechanisms were identified : ( 1 ) K+ currents ( total and those sensitive to the KCNQ 1 blocker chromanol 293B ) were significantly increased in atrial cells from KCNE 1 / mice compared with controls , and ( 2 ) when CHO cells expressing KCNQ 1 and KCNE 1 were pulsed very rapidly ( at rates comparable to the normal mouse heart and to human atrial fibrillation ) , the sigmoidicity of IKs activation prevented current accumulation , whereas cells expressing KCNQ 1 alone displayed marked current accumulation at these very rapid rates . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
KCNQ 1 ( Kv 7 . 1 ) alpha subunits and KCNE 1 beta subunits co assemble to form channels that conduct the slow delayed rectifier K+ current ( IKs ) in the heart . ^^^ V310I KCNQ 1 reduced IKs amplitude when co expressed with wild type KCNQ 1 and KCNE 1 subunits . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Genetic mutations in the alpha ( KCNQ 1 ) and beta ( KCNE 1 ) subunits of 1 ( Ks ) underlie Long QT Syndrome type 1 and 5 ( LQT 1 and LQT 5 ) , respectively , and predispose carriers to the development of polymorphic ventricular arrhythmias and sudden cardiac death . beta adrenergic stimulation increases 1 ( Ks ) and results in rate dependent AP shortening , a control system that can be disrupted by some mutations linked to LQT 1 and LQT 5 . ^^^ We reconstituted 1 ( Ks ) in CHO cells ( ie , KCNQ 1 coexpressed with KCNE 1 and the adaptator protein Yotiao ) and quantitatively examined the effects of beta adrenergic stimulation on channel kinetics . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
RT PCR detected the expression of members of the KCNQ family from KCNQ 1 to KCNQ 5 and of the accessory proteins KCNE 1 to KCNE 3 in the rat hepatocytes , but not KCNQ 2 and KCNE 2 in human liver . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Using a two step design we analyzed 174 SNPs from the KCNQ 1 , KCNH 2 , KCNE 1 , and KCNE 2 genes in 689 individuals from the population based KORA study and 14 SNPs with results suggestive of association in a confirmatory sample of 3277 individuals from the same survey . ^^^ We detected association to a gene variant in intron 1 of the KCNQ 1 gene ( rs 757092 , +1 . 7 ms / allele , P=0 . 0002 ) and observed weaker association to a variant upstream of the KCNE 1 gene ( rs 727957 , +1 . 2 ms / allele , P=0 . 0051 ) . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Loss of function in the slow component of the delayed rectifier potassium current ( 1 ( Ks ) ) channels ( KCNQ 1 , KCNE 1 ) , the rapid component of the potassium current ( 1 ( Kr ) ) channels ( KCNH 2 , KCNE 2 ) and the inward rectifier potassium current ( 1 ( Kl ) , Kir2 . 1 ) channel ( KCNJ 2 ) is linked to the LQTSs ( type 1 , 2 , 5 , 6 , and 7 ( Andersen syndrome ) ) and the JLNSs ( type 1 and 2 ) . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
OBJECTIVE : The voltage gated K+ channel KCNQ 1 associates with the small KCNE 1 beta subunit to underlie the IKs repolarizing current in the heart . ^^^ Absolute cDNA quantification revealed a relatively homogeneous distribution of each transcript , except for KCNE 4 , inside left atria and endo and epicardia of left ventricular wall with the following abundance : KCNQ 1 > > KCNE 4 > or = KCNE 1 > KCNE 3 > KCNE 2 > KCNE 5 . ^^^ CONCLUSIONS : Our data show that KCNQ 1 forms a channel complex with 5 KCNE subunits in a specific manner but only interactions with KCNE 1 , KCNE 2 , and KCNE 3 may have physiological relevance in the human heart . . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
To characterize the physiological consequences of the V141M mutation , Xenopus laevis oocytes were injected with cRNA encoding wild type ( wt ) KCNQ 1 or mutant V141M KCNQ 1 subunits , with or without KCNE 1 . ^^^ In the absence of KCNE 1 , wtKCNQ 1 and V141M KCNQ 1 currents had similar biophysical properties . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Association of KCNQ 1 , KCNE 1 , KCNH 2 and SCN5A polymorphisms with QTc interval length in a healthy population . ^^^ This suggests that genetic determinants located in KCNQ 1 , KCNE 1 , KCNH 2 and SCN5A influence QTc length in healthy individuals and may represent risk factors for arrhythmias or cardiac sudden death in patients with cardiovascular diseases . . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Using RT / PCR cellular mRNA of KCNQ 1 but not of KCNE 1 channels was detected in INS 1 cells . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
With this method , we identified the mutation ( s ) in all four patients with congenital LQTS ( KCNQ 1 A341V , KCNH 2 N633D , KCNH 2 2768Cdel and KCNE 1 K70 N Y81C double mutations ) . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Further , AZ sensitive K ( slow ) current was extant in beta cells from KCNQ 1 or KCNE 1 null mice lacking cardiac slow delayed rectifier currents . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
METHODS : Between August 1997 and July 2004 , 541 consecutive , unrelated patients ( 358 females , average age at diagnosis 24 years , average QTc 482 ms ) were referred to Mayo Clinic ' s Sudden Death Genomics Laboratory for comprehensive mutational analysis of the five cardiac channel genes implicated in LQTS : KCNQ 1 ( LQT 1 ) , KCNH 2 ( LQT 2 ) , SCN5A ( LQT 3 ) , KCNE 1 ( LQT 5 ) , and KCNE 2 ( LQT 6 ) . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
DNA samples were screened for 10 missense mutations in 5 genes associated with the congenital long QT ( LQT ) syndrome ( KCNQ 1 , KCNH 2 , SCN5A , KCNE 1 , KCNE 2 ) . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
In the heart , KCNQ 1 is coassembled with KCNE 1 to produce a cardiac delayed rectifier K+ current . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Blood samples submitted for molecular diagnostic studies on 7 infants were subject to DNA extraction and mutation analysis of 18 selected exons in 5 LQTS genes ( KCNQ 1 , HERG , SCN5A , KCNE 1 , and KCNE 2 ) . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
The entire coding regions of KCNQ 1 , KCNH 2 , SCN5A , KCNE 1 , and KCNE 2 were screened by denaturing high performance liquid chromatography and DNA sequencing . ^^^ RESULTS : We identified 235 different mutations , 138 of which were novel , in 310 ( 72 % ) of 430 probands ( 49 % KCNQ 1 , 39 % KCNH 2 , 10 % SCN5A , 1 . 7 % KCNE 1 , and 0 . 7 % KCNE 2 ) . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
BACKGROUND : Data on the Jervell and Lange Nielsen syndrome ( J LN ) , the long QT syndrome ( LQTS ) variant associated with deafness and caused by homozygous or compound heterozygous mutations on the KCNQ 1 or on the KCNE 1 genes encoding the 1 ( Ks ) current , are still based largely on case reports . ^^^ Most mutations ( 90 . 5 % ) are on the KCNQ 1 gene ; mutations on the KCNE 1 gene are associated with a more benign course . beta Blockers have only partial efficacy ; 51 % of the patients had events despite therapy and 27 % had CA / SD . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Effects on the two major currents responsible for repolarization of the cardiac action potential ( mediated by the human ether  gogo related gene ( HERG ) product ) , and by the potassium channel Q 1 ( KCNQ 1 ) co expressed with the potassium channel accessory subunit E 1 ( KCNE 1 ) were examined , as well as effects on the sodium inward current ( mediated by the sodium channel 5A ( SCN5A ) and generating the rapid upstroke of the action potential ) . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Genetic polymorphisms in KCNQ 1 , HERG , KCNE 1 and KCNE 2 genes in the Chinese , Malay and Indian populations of Singapore . ^^^ AIMS : To determine the genetic variability of long QT syndrome ( LQTS ) associated genes ( KCNQ 1 , HERG , KCNE 1 and KCNE 2 ) among three distinct ethnic groups in the Singapore population . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Increased aldosterone secretion has been found in a mouse lacking the KCNE 1 gene which codes for a regulatory protein of the KCNQ 1 gene product , forming the channel for the outward rectifying delayed K+ current . ^^^ Normal and adenomatous adrenal samples and NCI H 295 cell line were used to : a ) evaluate KCNE 1 and KCNQ 1 gene expression , b ) sequence the full length cDNAs of KCNE 1 and both KCNQ 1 isoforms . ^^^ These differently spliced KCNE 1 and KCNQ 1 mRNAs were expressed in adrenal tissue . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Eleven patients gave their consent to mutational analysis of KCNQ 1 , KCNH 2 , SCN5A , KCNE 1 , KCNE 2 and KCNJ 2 genes ( associated with congenital long QT syndrome ) . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
METHODS : The published primers currently used by many research laboratories to conduct a comprehensive analysis of the 60 translated exons in the KCNQ 1 ( LQT 1 ) , KCNH 2 ( LQT 2 ) , SCN5A ( LQT 3 ) , KCNE 1 ( LQT 5 ) , and KCNE 2 ( LQT 6 ) genes were analyzed for the presence of common intronic single nucleotide polymorphisms ( SNPs ) . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
We have obtained significant differences between susceptible and resistant individuals for the allele , genotype , and haplotype frequencies for three SNPs of the KCNE 1 gene , and for the allele frequencies for one SNP of KCNQ 1 and one SNP of KCNQ 4 . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
In marked contrast , pretreatment with tyrosine kinase inhibitor tyrphostin A 25 ( 20 microM ) strongly attenuated both the hyposmotic stimulation of 1 ( Ks ) in myocytes and the hyposmotic stimulation of current in BHK cells co expressing Ks channel subunits KCNQ 1 and KCNE 1 . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Modulation of functional properties of KCNQ 1 channel by association of KCNE 1 and KCNE 2 . ^^^ Assembly of KCNQ 1 K ( + ) channel alpha subunits and KCNE 1 underlies cardiac 1 ( Ks ) , while KCNQ 1 interacts with all other members of KCNE forming complexes with different properties . ^^^ Here we investigated synergic actions of KCNE 1 and KCNE 2 on functional properties of KCNQ 1 heterologously expressed in COS 7 cells . ^^^ Patch clamp recordings from cells expressing KCNQ 1 and KCNE 1 exhibited the slowly activating current , while co expression of KCNQ 1 with KCNE 2 produced a practically time independent current . ^^^ When KCNQ 1 was co expressed with both of KCNE 1 and KCNE 2 , the membrane current exhibited a voltage and time dependent current whose characteristics differed substantially from those of the KCNQ1 / KCNE1 current . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
The slow IKS K+ channel plays a major role in repolarizing the cardiac action potential and consists of the assembly of KCNQ 1 and KCNE 1 subunits . ^^^ Mutations in either KCNQ 1 or KCNE 1 genes produce the long QT syndrome , a life threatening ventricular arrhythmia . ^^^ KCNE 1 forms a ternary complex with wild type KCNQ 1 and Ca ( 2+ ) CaM that prevents inactivation , facilitates channel assembly , and mediates a Ca ( 2+ ) sensitive increase of IKS current , with a considerable Ca ( 2+ ) dependent left shift of the voltage dependence of activation . ^^^ KCNE 1 association to KCNQ 1 long QT mutants significantly improves mutant channel expression and prevents macroscopic inactivation . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
METHODS : Using denaturing high performance liquid chromatography and DNA sequencing , mutational analysis of 23 RyR 2 exons previously implicated in CPVT 1 , comprehensive analysis of all translated exons in CASQ 2 ( CPVT 2 ) , KCNQ 1 ( LQT 1 ) , KCNH 2 ( LQT 2 ) , SCN5A ( LQT 3 ) , KCNE 1 ( LQT 5 ) , KCNE 2 ( LQT 6 ) , and KCNJ 2 ( Andersen Tawil syndrome [ ATS 1 ] , also annotated LQT 7 ) , and analysis of 10 ANK 2 exons implicated in LQT 4 were performed on genomic DNA from 11 unrelated patients ( 8 females ) referred to Mayo Clinic ' s Sudden Death Genomics Laboratory explicitly for CPVT genetic testing . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
It is becoming clear that mutations in the KVLQT 1 , human `` ether a go go ' ' related gene , cardiac voltage dependent sodium channel gene , minK and MiRP 1 genes , respectively , are responsible for the LQT 1 , LQT 2 , LQT 3 , LQT 5 and LQT 6 variants of the Romano Ward syndrome , characterized by autosomal dominant transmission and no deafness . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Persons with Jevell Lange Neilsen syndrome inherit pathological allele from both parents ( KvLQT 1 or LQT 5 minK ) . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
BACKGROUND : Congenital long QT syndrome ( LQTS ) is caused by mutations of genes encoding the slow component of the delayed rectifier current ( LQT 1 , LQT 5 ) , the rapid component of the delayed rectifier current ( LQT 2 , LQT 6 ) , or the Na ( + ) current ( LQT 3 ) , resulting in ST T wave abnormalities on the ECG . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Human cardiac KCNQ 1 and KCNE 1 genes were cotransfected into HEK 293 cells , and a cell clone stably expressing both genes was selected . ^^^ The typical 1 ( Ks ) was slowly activated upon depolarization voltages in HEK 293 cells stably expressing human cardiac KCNQ 1 and KCNE 1 genes , and the current was inhibited by 1 ( Ks ) blockers HMR 1556 and chromanol 293B , with 50 % inhibitory concentrations ( IC ( 50 ) s ) of 83 . 8 nM : and 9 . 2 muM : , respectively . 1 ( Ks ) showed a significant temperature dependent increase in its magnitude upon elevating bath temperature to 36 degrees C from room temperature ( 21 degrees C ) . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
We report the molecular pattern of 44 unrelated patients with LQTS using denaturing high performance liquid chromatography analysis of the KCNQ 1 , KCNH 2 , SCN5A , KCNE 1 and KCNE 2 genes . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Four LQT genes have been identified : KVLQT 1 ( LQT 1 ) on chromosome 11p15 . 5 , HERG ( LQT 2 ) on chromosome 7q35 36 , SCN5A ( LQT 3 ) on chromosome 3p21 24 , and MinK ( LQT 5 ) on chromosome 21q22 . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
We found significant linkage of QTc with the loci for LQT 1 on chromosome 11 and LQT 4 on chromosome 4 but not to LQT 2 , LQT 3 , or LQT 5 . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
We used electrophysiological and immunocytochemical methods to compare the cellular phenotypes of wild type minK and four LQT 5 mutants co expressed with KvLQT 1 in Xenopus oocytes and HERG in HEK 293 cells . ^^^ We conclude that minK is a co factor in the expression of both IKs and IKr and propose that clinical manifestations of LQT 5 may be complicated by differing effects of minK mutations on KvLQT 1 and HERG . . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Three of these , LQT 1 , LQT 2 , and LQT 5 , encode potassium channel subunits . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
LQT was found to be caused by mutations in four genes LTQ 1 , LQT 2 , LQT 3 and LQT 5 , and linkage was reported for an additional locus , LQT 4 , on chromosome 4q25 27 . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Genetic studies have identified four forms of congenital long QT syndrome ( LQTS ) caused by mutations in ion channel genes located on chromosomes 3 ( LQT 3 ) , 7 ( LQT 2 ) , 11 ( LQT 1 ) , and 21 ( LQT 5 ) . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
The channel underlying the slow component of the voltage dependent delayed outward rectifier K+ current , 1 ( Ks ) , in heart is composed of the minK and KvLQT 1 proteins . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Voltage dependent inactivation of the human K+ channel KvLQT 1 is eliminated by association with minimal K+ channel ( minK ) subunits . 1 . ^^^ Delayed rectifier K+ ( IKs ) channels formed by heteromultimeric coassembly of KvLQT 1 and minimal K+ channel ( minK ) subunits did not inactivate . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Mutations of genes encoding KvLQT 1 and minK are responsible for the hereditary long QT syndrome ( loci LQT 1 and LQT 5 , respectively ) . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
METHODS : We enrolled 27 symptomatic patients with LQTS undergoing ICD therapy ( QTc 540 + / 64 ms ( 1 / 2 ) ; 85 % female , 63 % cardiac arrest ; 33 % syncope despite beta blockers ; 4 % with severe phenotype ) and 81 genotyped patients with LQTS undergoing conventional drug therapy ( 28 LQT 1 , 39 LQT 2 , 1 LQT 3 , 13 LQT 5 ) . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Coexpression of minK with KvLQT 1 results in a conductance with pharmacological and biophysical properties more similar to 1 ( Ks ) than other known delayed rectifier K+ currents in the heart . . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Molecular mechanism and functional significance of the MinK control of the KvLQT 1 channel activity . ^^^ The IKs channel is formed via the assembly of two transmembrane proteins , KvLQT 1 and MinK . ^^^ Here , we show a new mode of association between ion channel forming subunits in that the cytoplasmic C terminal end of MinK interacts directly with the pore region of KvLQT 1 . ^^^ However , because MinK also reveals a large number of previously silent KvLQT 1 channels ( 10 60 ) , the overall effect is a large increase ( 10 4 ) in the macroscopic K+ current . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
The induced channels appear to result from the interaction of the minK protein with other channel forming subunits such as the KvLQT 1 channel . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
It has been discovered recently that the KvLQT 1 and minK proteins functionally interact to generate a current with biophysical properties similar to 1 ( Ks ) , the slowly activating delayed rectifier cardiac potassium current . ^^^ METHODS AND RESULTS : We expressed LQTS associated KvLQT 1 mutants in Xenopus oocytes either individually or in combination with wild type KvLQT 1 or in combination with both wild type KvLQT 1 and minK . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
KvLQT 1 and minK subunits coassemble to form IKs channels . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Many genes and their mutations causing familial cardiovascular diseases have been discovered , including familial hypertrophic cardiomyopathy which is caused by mutated cardiac beta myosin heavy chain , light chains , troponin T , troponin 1 , or alpha tropomyosin , and long QT syndrome by KvLQT 1 , HERG , minK or cardiac voltage dependent Na channel mutation . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
KVLQT 1 and minK encode subunits that coassemble to form channels that mediate 1 ( Ks ) . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Recent advances in molecular genetics have allowed to identify mutations in four genes , KvLQT 1 ( 11p15 . 5 ) , HERG ( 7q35 ) , SCN5A ( 3p21 ) and minK ( 21q22 ) , which cause LQTS . ^^^ The KvLQT 1 and minK genes code the slowly activating , delayed rectifier ( Iks ) potassium channel , the HERG gene code the rapidly activating , delayed rectifier ( Ikr ) potassium channel of the heart , while the SCN5A gene codes a cardiac sodium channel . ^^^ Mutations in KvLQT 1 , minK and HERG genes affects repolarising , rectifier potassium currents , while SCN5A mutations cause delayed inactivation and reopening of the cardiac sodium channel , which initiates the depolarisation of cardiac cells . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
These findings indicate that the association of minK with KvLQT 1 interferes with the binding of R L 3 or prevents its action once bound to KvLQT 1 subunits . . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Nature . 384 : 80 83 ) Using the Xenopus oocyte expression system , we analyzed in detail the gating characteristics of homomeric KvLQT 1 channels and of heteromeric KvLQT1 / minK channels using two electrode voltage clamp recordings . ^^^ In coexpression experiments of KvLQT 1 with minK , inactivation seems to be largely absent , although biphasic tails are also observed that could be related to similar phenomena . . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Four LQT genes have been identified for autosomal dominant LQT : K+ channel genes KVLQT 1 on chromosome 11p15 . 5 , HERG on 7q35 36 and minK on 21q22 , and the cardiac Na+ channel gene SCN5A on chromosome 3p21 24 . ^^^ Two genes , KVLQT 1 and minK , have been identified for Jervell and Lange Nielsen syndrome . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Assembled from minK and KvLQT 1 subunits , IKs channels are notable for a heteromeric ion conduction pathway in which both subunit types contribute to pore formation . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Using fluctuation analysis and single channel recordings , we have studied the currents formed by human KvLQT 1 subunits alone and in conjunction with human or rat minK subunits . ^^^ The broad spectrum causes the apparent single channel current value to depend on the bandwidth of the recording , and is mirrored in very `` flickery ' ' single channel events of the channels from coexpressed KvLQT 1 and human minK subunits . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Long QT syndrome associated mutations in the S 4 S5 linker of KvLQT 1 potassium channels modify gating and interaction with minK subunits . ^^^ Co expression of minK and KvLQT 1 channel subunits induces a slow delayed rectifier K ( + ) current , 1 ( Ks ) , characterized by slow activation and a markedly increased magnitude compared with current induced by KvLQT 1 subunits alone . ^^^ Coexpression of minK with R243C or W248R KvLQT 1 subunits suppressed current , suggesting that coassembly of mutant subunits with minK prevented normal channel gating . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
In the case of the arrhythmogenic disorders , the long QT syndromes and Brugada syndrome , mutations have been described in a number of ion channel proteins , including cardiac potassium ( KVLQT 1 , HERG and minK ) and sodium ( SCN5A ) channels . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
The KvLQT 1 and minK subunits that coassemble to form 1 ( sK ) channels , contain potential N glycosylation sites . ^^^ To examine the role of glycosylation in channel function , a Chinese hamster ovary cell line deficient in glycosylation ( Lec 1 ) and its parental cell line ( Pro 5 ) were transiently transfected with human KvLQT 1 ( hKvLQT 1 ) cDNA , alone and in combination with the rat ( rminK ) or human minK ( hminK ) cDNA . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Total RNA was prepared from the atrial appendages from 0 to 8 hours after the onset of pacing , and mRNA levels of Kv1 . 2 , Kv 1 . 4 , Kv1 . 5 , Kv2 . 1 , Kv4 . 2 , Kv4 . 3 , erg , KvLQT 1 , and minK were determined by RNase protection assay . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
The ion channel responsible for the IKs current is made of two proteins , the KvLQT 1 protein and the MinK protein . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Channels formed by coassembly of the KCNQ 1 ( KvLQT 1 ) subunit and the minK subunit underlie slowly activating cardiac delayed rectifier ( 1 ( Ks ) ) in the heart , whereas two other members of the KCNQ channel family , KCNQ 2 and KCNQ 3 , coassemble to underlie the M current in the nervous system . ^^^ In this study , we examined the role of the minK subunit in determining the response of KCNQ 1 channels to blockade by the cognitive enhancer XE 991 . ^^^ Coexpression with minK markedly decreased the sensitivity of KCNQ 1 to blockade by XE 991 . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
The mRNA amounts of KVLQT 1 ( IKs ) , minK ( beta subunit of IKs ) , HERG ( IKr ) , and KV1 . 5 ( IKur ) were measured by reverse transcription polymerase chain reaction and normalized to the mRNA amount of GAPDH . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
KvLQT 1 alpha subunits coassemble with minK beta subunits to form channels that conduct the slow delayed rectifier K+ current ( 1 ( Ks ) ) in the heart . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
MinK subdomains that mediate modulation of and association with KvLQT 1 . ^^^ MinK , a 129 amino acid protein containing one transmembrane spanning domain modulates KvLQT 1 , greatly slowing activation , increasing current amplitude , and removing inactivation . ^^^ Coexpression of KvLQT 1 with a MinK COOH terminus deletion mutant ( MinK DeltaCterm ) in Xenopus oocytes resulted in a rapidly activated potassium current closely resembling currents recorded from oocytes expressing KvLQT 1 alone , indicating that this region is necessary for modulation . ^^^ To determine whether MinK DeltaCterm was associated with KvLQT 1 , a functional tag ( G55C ) that confers susceptibility to partial block by external cadmium was engineered into the transmembrane domain of MinK DeltaCterm . ^^^ Currents derived from coexpression of KvLQT 1 with MinK DeltaCterm were cadmium sensitive , suggesting that MinK DeltaCterm does associate with KvLQT 1 , but does not modulate gating . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
MinK endows the 1 ( Ks ) potassium channel pore with sensitivity to internal tetraethylammonium . 1 ( Ks ) channels are heteromeric complexes of pore forming KvLQT 1 subunits and pore associated MinK subunits . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
These include two potassium channel alpha subunits ( KVLQT 1 and HERG ) , two potassium channel beta subunits ( minK and MiRP 1 ) , and one sodium channel gene ( SCN5A ) . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Slowly activating 1 : ( Ks ) ( KCNQ1 / MinK ) channels were expressed in Xenopous : oocytes and their sensitivity to chromanols was compared to homomeric KCNQ 1 channels . ^^^ To elucidate the contribution of the ss subunit MinK on chromanol block , a formerly described chromanol HMR 1556 and its enantiomer S 5557 were tested for enantio specificity in blocking 1 : ( Ks ) and KCNQ 1 as shown for the single enantiomers of chromanol 293B . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
The majority ( 92 % ) of current was 1 ( Kr ) . mRNA levels of rabbit ERG , KVLQT 1 , and minK in left ventricular myocardium did not differ between control and long term amiodarone . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
These include two potassium channel alpha subunits ( KVLQT 1 , HERG ) , two potassium channel beta subunits ( minK , MiRP 1 ) , and one sodium channel gene ( SCN5A ) . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Native cardiac 1 ( Ks ) channels comprising KCNQ 1 and accessory MinK subunits do not inactivate because of the functional interaction of KCNQ 1 with MinK . ^^^ Here we show that when expressing mutant 1 ( Ks ) channels formed from KCNQ 1 ( L273F ) and MinK , MinK association no longer eliminates KCNQ 1 inactivation . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Heterologous coexpression of KvLQT 1 and the accessory protein minK yields an IKs like current . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
The concentrations required for inhibition of ICFTR are three to fivefold higher than those reported for inhibition of KvLQT 1 + minK expressed in Xenopus oocytes . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
The slowly activating cardiac potassium current ( 1 ( Ks ) ) is generated by a heteromultimeric potassium channel complex consisting of pore forming ( KvLQT 1 ) and accessory ( minK ) subunits belonging to the KCNQ and KCNE gene families , respectively . ^^^ We have identified a single cysteine residue in the KvLQT 1 S6 segment ( Cys 331 ) that contributes to Cd2+ coordination in conjunction with cysteine residues engineered into the minK transmembrane domain . ^^^ On the basis of homology modeling that compares the KvLQT 1 S6 segment with the structure of the bacterial potassium channel KcsA , we predict that the sulfhydryl side chain of Cys 331 projects away from the central axis of the KvLQT 1 pore and suggest that minK resides outside of the permeation pathway . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
MinK is the beta subunit of the slow activating component of the delayed rectifier potassium current ( 1 ( Ks ) ) channel , and associates with the alpha subunit , KvLQT 1 . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
To investigate whether MinK , MiRP 1 , and MiRP 2 operate similarly with their known native alpha subunit partners ( KCNQ 1 , HERG , and Kv3 . 4 , respectively ) two conserved residues associated with human disease and influential in channel function were evaluated . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
When this gene was expressed along with a full length KCNQ 1 , it suppressed potassium currents , whether a regulatory subunit minK was co expressed or not . . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
OBJECTIVE : Co expression of the KvLQT 1 and minK potassium channel subunits is required to recapitulate 1 ( Ks ) , the slow component of the cardiac delayed rectifier current , and mutations in either gene cause the congenital Long QT syndrome . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Inhibitory effects of volatile anesthetics on currents produced on heterologous expression of KvLQT 1 and minK in Xenopus oocytes . ^^^ We investigated the effects of the volatile anesthetics isoflurane and sevoflurane on cloned IKs coexpressed by KvLQT 1 and minK . ^^^ Currents were induced following injection into oocytes of KvLQT 1 mRNA ( 10 ng ) with or without minK mRNA ( 1 ng ) , which were transcribed in vitro from cDNAs of normal rats hearts . ^^^ A two electrode voltage clamp recording technique was used to investigate the effects of isoflurane ( 0 1 . 5 minimum alveolar concentration , MAC ) and sevoflurane ( 0 1 . 5 MAC ) on IKs ( KvLQT 1 with minK ) and KvLQT 1 alone currents . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Kv1 . 4 , Kv1 . 5 , Kv2 . 1 , Kv4 . 2 , erg , LQT 1 , and minK were assayed by RNase protection assay . ^^^ LQT 1 was decreased by T 3 in both chambers ( P < 0 . 01 ) and not affected by PTU . minK was not detectable in the control state and was up regulated only in the atrium : a peak on the 4th day followed by a decline to the undetectable level on the 10 15th days . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Co expression of F193L KCNQ 1 with the K ( + ) channel minK suppressed peak ( by 23 . 3 % ) and tail ( by 38 . 2 % ) currents compared with those obtained by the combination of wild type ( WT ) KCNQ 1 and minK . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Pore and state dependent cadmium block of 1 ( Ks ) channels formed with MinK 55C and wild type KCNQ 1 subunits . ^^^ Human MinK and KCNQ 1 subunits assemble to form 1 ( Ks ) channels . ^^^ Two recent reports argue that MinK 55C is distant from the pore : one finds TEA does not affect Cd ( 2+ ) block if channels are formed with a KCNQ 1 mutant ( K318I , V319Y ) that increases TEA affinity ; the second proposes that Cd ( 2+ ) binds between MinK 55C and a cysteine in KCNQ 1 that is posited to lie toward the channel periphery . ^^^ First , Cd ( 2+ ) block of MinK 55C channels formed with wild type KCNQ 1 is shown to depend not only on voltage and trans ions but state ( showing decreased on rate with increased open time and blocker trapping on channel closure ) . ^^^ Conversely , MinK 55C channels with K318I , V319Y KCNQ 1 are found to demonstrate Cd ( 2+ ) block that is independent of voltage , trans ions and state ( and to have a lower unitary conductance ) : thus , the KCNQ 1 mutations alter the process under study , yielding Cd ( 2+ ) inhibition that is pore independent and , perforce , TEA insensitive . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Charybdotoxin binding in the 1 ( Ks ) pore demonstrates two MinK subunits in each channel complex . 1 ( Ks ) voltage gated K ( + ) channels contain four pore forming KCNQ 1 subunits and MinK accessory subunits in a number that has been controversial . ^^^ First , CTX on rate , off rate , and equilibrium affinity are found to be the same for channels of monomers and those with a fixed 2 : 4 MinK : KCNQ 1 valence . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Selective blockers ( chromanol 293B , HMR 1556 , L 735 , 821 ) of the KvLQT 1 plus minK channel , which carriy the slow delayed rectifier potassium current ( 1 ( Ks ) ) , were also considered to treat arrhythmias , including atrial fibrillation ( AF ) . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
KvLQT 1 is associated with a function altering beta subunit , minK to form IKs . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
METHODS : Expression and distribution of the inward rectifier subunits Kir2 . 1 and Kir2 . 3 , the rapid delayed rectifier alpha subunit ERG , the slow delayed rectifier alpha subunit KvLQT 1 , the beta subunit minK , the L type Ca ( 2+ ) subunit Ca ( 5 ) 1 . 2 , and the Na ( + ) , Ca ( 2+ ) exchanger were quantified by Western blot on isolated cardiomyocytes and localized by immunohistochemistry in tissue sections obtained from canine hearts . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
The mRNA levels of Kv4 . 2 , Kv1 . 4 , KChIP 2 , and Kir2 . 1 were significantly downregulated , whereas the Kv4 . 3 , Erg , KvLQT 1 , and minK were unaltered in the failing ventricles compared with the control left ventricles . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
Apico basal differences were found in the expression of only those channel proteins which are involved in mediation of the transient outward K ( + ) current and the slow delayed rectifier K ( + ) current : expression of Kv1 . 4 , KChIP 2 , KvLQT 1 and MinK was significantly higher in apical than in basal myocardium in both canine and human hearts . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
OBJECTIVES : We tested the hypothesis that the developmental changes occurring in 1 ( Kr ) and 1 ( Ks ) can be explained by changes in the expression of ERG encoding 1 ( Kr ) , and KCNQ 1 , the beta subunit minK , and the recently reported subunit FHL 2 encoding 1 ( Ks ) . ^^^ METHODS : We used Western blotting and real time polymerase chain reaction to compare the expression of ERG , KCNQ 1 , minK , and FHL 2 in 1 week old pups and adult dogs . ^^^ Whereas expression of KCNQ 1 and FHL 2 is unchanged between the two age groups , minK is minimally expressed at 1 week and increases in adults , consistent with developmental increase in 1 ( Ks ) . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
In the canine heart , the expression of Kv4 . 3 , Kv1 . 4 , KChIP 2 and KvLQT 1 was significantly higher , and that of Nav1 . 5 and MinK much lower , in EPI than in MID . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
This study reveals a novel amino acid determinant of the minK KvLQT 1 interaction , and if the role of minK38G in AF is confirmed , would suggest mechanistic heterogeneity in genetic determinants of AF . . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
The diagnostic analyses are performed by sequencing the exons of five genes , KCNQ 1 , HERG , SCN5A , minK and MiRP 1 . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
KCNQ 1 alpha subunits form functionally distinct potassium channels by coassembling with KCNE ancillary subunits MinK and MiRP 2 . ^^^ MinK KCNQ 1 channels generate the slowly activating , voltage dependent cardiac IKs current . ^^^ This hypothesis was tested using concomitant mutagenesis in KCNQ 1 and in the membrane localized ' activation triplet ' regions of MinK and MiRP 2 to identify pairs of residues that interact to control KCNQ 1 activation . ^^^ The results place KCNE subunits close to the KCNQ 1 pore , indicating interaction of MiRP 2 72 with KCNQ 1 338 ; and MinK 59 , 58 with KCNQ 1 339 , 340 . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
KvLQT 1 and minK mRNA and protein levels were downregulated in both bradypaced and tachypaced rabbits , whereas ERG was significantly downregulated in bradypaced rabbits only . ^^^
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
NA
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
NA
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
NA
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
NA
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
NA
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
NA
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
NA
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
NA
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
NA
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
NA
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
NA
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
NA
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
NA
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
NA
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
NA
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
NA
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
NA
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
NA
Interacting proteins: P15382 and P51787	Pubmed	SVM Score :0.0
NA