| Interacting proteins: P78562 and Q9GZV9 |
Pubmed |
SVM Score :0.0 |
| PURPOSE OF REVIEW : Three hypophosphatemic diseases , 10 linked dominant hypophosphatemic rickets / osteomalacia ( XLH ) , autosomal dominant hypophosphatemic rickets / osteomalacia ( ADHR ) and tumor induced rickets / osteomalacia ( TIO ) , show very similar clinical features including hypophosphatemia due to renal phosphate wasting . ^^^ RECENT FINDINGS : The PHEX gene and fibroblast growth factor ( FGF ) 23 were identified as responsible genes for XLH and ADHR , respectively . ^^^ |
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| Interacting proteins: P78562 and Q9GZV9 |
Pubmed |
SVM Score :0.0 |
| In addition , the elevated serum FGF 23 levels were demonstrated in most patients with XLH . ^^^ It is likely that increased serum levels of FGF 23 contributes to the development of hypophosphatemia not only in TIO but also in XLH . . ^^^ |
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| Interacting proteins: P78562 and Q9GZV9 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: P78562 and Q9GZV9 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: P78562 and Q9GZV9 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: P78562 and Q9GZV9 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: P78562 and Q9GZV9 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: P78562 and Q9GZV9 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: P78562 and Q9GZV9 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: P78562 and Q9GZV9 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: P78562 and Q9GZV9 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: P78562 and Q9GZV9 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: P78562 and Q9GZV9 |
Pubmed |
SVM Score :0.0 |
| NA |
|
| Interacting proteins: P78562 and Q9GZV9 |
Pubmed |
SVM Score :0.0 |
| The sequences of hypF , hypC and hypD complete the hyp gene cluster required for hydrogenase activity in Bradyrhizobium japonicum . ^^^ |
|
| Interacting proteins: P78562 and Q9GZV9 |
Pubmed |
SVM Score :0.0 |
| The products of the Rhizobium leguminosarum hyp gene cluster are necessary for synthesis of a functional uptake [ NiFe ] hydrogenase system in symbiosis with pea plants , and at least for HypB and HypF , a role in hydrogenase specific nickel metabolism has been postulated ( L . ^^^ |
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| Interacting proteins: P78562 and Q9GZV9 |
Pubmed |
SVM Score :0.0 |
| Six genes , arranged as a single operon , were identified , and designated hypA , B , F , C , D and E based on the sequence similarities of all of them , except hypF , to genes from the hydrogenase pleiotropic operon ( hyp ) from Escherichia coli . ^^^ |
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| Interacting proteins: P78562 and Q9GZV9 |
Pubmed |
SVM Score :0.0 |
| Despite a high identity between the two copies of hyp gene products , substantial structural differences were identified between the two copies of hypF genes . ^^^ |
|
| Interacting proteins: P78562 and Q9GZV9 |
Pubmed |
SVM Score :0.0 |
| The biosynthesis of [ NiFe ] hydrogenases is a complex process that requires the function of the Hyp proteins HypA , HypB , HypC , HypD , HypE , HypF , and HypX for assembly of the H ( 2 ) activating [ NiFe ] site . ^^^ We conclude that the entire Hyp apparatus ( HypA , HypB , HypC , HypD , HypE , and HypF ) is involved in an efficient incorporation of the [ NiFe ] center into the RH . . ^^^ |
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| Interacting proteins: P78562 and Q9GZV9 |
Pubmed |
SVM Score :0.0 |
| To establish the mechanisms whereby Phex regulates FGF 23 , we assessed Phex dependent hydrolysis of recombinant FGF 23 in vitro and measured fgf 23 message levels in the Hyp mouse homologue of XLH . ^^^ In addition , Hyp mice displayed a bone restricted increase in fgf 23 transcripts in association with inactivating Phex mutations . ^^^ Increased expression of fgf 23 was also observed in Hyp derived osteoblasts in culture . ^^^ |
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| Interacting proteins: P78562 and Q9GZV9 |
Pubmed |
SVM Score :0.0 |
| Our lab previously characterized mutants in the accessory hyp genes , hypA , hypB , hypD and hypF that were all severely affected for hydrogenase activity , and in some cases ( hypA and hypB mutants ) also affected for urease activity . ^^^ |
|
| Interacting proteins: P78562 and Q9GZV9 |
Pubmed |
SVM Score :0.0 |
| The primary defects in 10 linked hypophosphatemic rickets ( HYP ) and autosomal dominant hypophosphatemic rickets ( ADHR ) are now identified as inactivating mutations in a Zn metalloendopeptidase ( PHEX ) and activating mutations in fibroblast growth factor 23 ( FGF 23 ) , respectively . ^^^ This has stimulated the proposal of a global model that takes into account the remarkable similarities between the inherited diseases ( HYP and ADHR ) and the tumor acquired disease OHO . ^^^ The following review will present a global hypothesis that attempts to explain the experimental and clinical observations in HYP , ADHR , and OHO , plus diverse mouse models that include the MEPE null mutant , HYP PHEX transgenic mouse , and MEPE PHEX double null mutant . . ^^^ In HYP , loss of PHEX function is proposed to result in an increase in uncleaved full length FGF 23 and / or inappropriate processing of MEPE . ^^^ |
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| Interacting proteins: P78562 and Q9GZV9 |
Pubmed |
SVM Score :0.0 |
| To further expand our understanding regarding the role of Fgf 23 in phosphate homeostasis and skeletal mineralization , we crossed Fgf 23 / animals with Hyp mice , the murine equivalent of XLH . ^^^ Interestingly , Hyp males lacking both Fgf 23 alleles were indistinguishable from Fgf 23 / / mice , both in terms of serum phosphate levels and skeletal changes , suggesting that Fgf 23 is upstream of the phosphate regulating gene with homologies to endopeptidases on the 10 chromosome ( Phex ) and that the increased plasma Fgf 23 levels in Hyp mice ( and in XLH patients ) may be at least partially responsible for the phosphate imbalance in this disorder . . ^^^ |
|
| Interacting proteins: P78562 and Q9GZV9 |
Pubmed |
SVM Score :0.0 |
| The authors found that Hyp mice had increased expression of the MEPE and another phosphaturic factor , Fgf 23 . ^^^ Increased Fgf 23 levels in Hyp mice were not affected by superimposed Mepe deficiency . ^^^ |
|
| Interacting proteins: P78562 and Q9GZV9 |
Pubmed |
SVM Score :0.0 |
| Consistent with this hypothesis , FGF 23 levels were shown to be elevated in most patients with XLH and in Hyp mice . ^^^ |
|
| Interacting proteins: P78562 and Q9GZV9 |
Pubmed |
SVM Score :0.0 |
| Several hereditary disorders of isolated phosphate wasting have been described , including 10 linked hypophosphataemic rickets ( XLH ) , hypophosphataemic bone disease ( HBD ) , hereditary hypophosphataemic rickets with hypercalciuria ( HHRH ) and autosomal dominant hypophosphataemic rickets ( ADHR ) . ^^^ |
|
| Interacting proteins: P78562 and Q9GZV9 |
Pubmed |
SVM Score :0.0 |
| Oncogenic osteomalacia ( OOM ) , 10 linked hypophosphatemia ( XLH ) , and autosomal dominant hypophosphatemic rickets ( ADHR ) are phenotypically similar disorders characterized by hypophosphatemia , decreased renal phosphate reabsorption , normal or low serum calcitriol concentrations , normal serum concentrations of calcium and parathyroid hormone , and defective skeletal mineralization . ^^^ XLH results from mutations in the PHEX gene , encoding a membrane bound endopeptidase , whereas ADHR is associated with mutations of the gene encoding FGF 23 . ^^^ |
|
| Interacting proteins: P78562 and Q9GZV9 |
Pubmed |
SVM Score :0.0 |
| To determine whether the serum FGF 23 concentration is regulated by dietary intake of Pi , we fed wild type ( WT ) , Npt2a gene ablated ( Npt2a ( / ) ) , and Hyp mice diets containing varying Pi contents ( 0 . 02 1 . 65 % ) . ^^^ The serum concentrations of FGF 23 in Hyp mice were 5 to 25 fold higher than values in WT mice , and the values varied with dietary Pi intake . ^^^ Fgf 23 mRNA abundance in calvaria was significantly higher in Hyp mice than in WT mice on the 1 % Pi diet ; in both groups of mice , fgf 23 mRNA abundance in calvarial bone was suppressed by 85 % on the low ( 0 . 02 % ) Pi diet . ^^^ |
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| Interacting proteins: P78562 and Q9GZV9 |
Pubmed |
SVM Score :0.0 |
| Pathogenic role of Fgf 23 in Hyp mice . ^^^ To determine the causal role of FGF 23 in XLH , we generated a combined Fgf 23 deficient enhanced green fluorescent protein ( eGFP ) reporter and Phex deficient Hyp mouse model ( Fgf 23 ( + / ) / Hyp ) . eGFP expression was expressed in osteocytes embedded in bone that exhibited marked upregulation of eGFP in response to Phex deficiency and in CD 31 positive cells in bone marrow venules that expressed low eGFP levels independently of Phex . ^^^ In bone marrow stromal cells ( BMSCs ) derived from Fgf 23 ( / ) / Hyp mice , eGFP expression was also selectively increased in osteocyte like cells within mineralization nodules and detected in low levels in CD 31 positive cells . ^^^ Regardless , the deletion of Fgf 23 from Hyp mice reversed the hypophosphatemia , abnormal 1 , 25 ( OH ) ( 2 ) D ( 3 ) levels , rickets , and osteomalacia associated with Phex deficiency . ^^^ These results suggest that Fgf 23 acts downstream of Phex to cause both the renal and bone phenotypes in Hyp mice . . ^^^ |
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| Interacting proteins: P78562 and Q9GZV9 |
Pubmed |
SVM Score :0.0 |
| Mutation at a locus ( HPDR ) on the 10 chromosome ( McKusick 30780 [ HPDR 1 ] ; 30781 [ HPDR 2 ] ) causes impaired renal phosphate transport , hypophosphatemia , and an associated impairment in the process of mineralization in bone and teeth ( 10 linked hypophosphatemia [ XLH ] ) . ^^^ |
|
| Interacting proteins: P78562 and Q9GZV9 |
Pubmed |
SVM Score :0.0 |
| Recent molecular insight has put fibroblast growth factor ( FGF 23 ) into the centre of pathophysiological considerations because of ( 1 ) overproduction ( tumour associated osteomalacia ) or ( 2 ) hypothetically , accumulation resulting from mutations causing resistance to processing or degradation ( autosomal dominant hypophosphataemia ) or ( 3 ) loss of function of a protease ( PHEX ) interfering with FGF 23 breakdown ( XLH ) . ^^^ |
|
| Interacting proteins: P78562 and Q9GZV9 |
Pubmed |
SVM Score :0.0 |
| Genetic studies of autosomal dominant hypophosphatemic rickets ( ADHR ) and 10 linked hypophosphatemia ( XLH ) identified the phosphaturic hormone FGF 23 and the membrane metalloprotease PHEX , and investigations of tumor induced osteomalacia ( TIO ) discovered the extracellular matrix protein MEPE . ^^^ Similarities between ADHR , XLH , and TIO suggest a model to explain the common pathogenesis of renal phosphate wasting and defective mineralization in these disorders . ^^^ FGF 23 elevations in ADHR are due to mutations of FGF 23 that block its degradation , in XLH from indirect actions of inactivating mutations of PHEX to modify the expression and / or degradation of FGF 23 and MEPE , and in TIO because of increased production of FGF 23 and MEPE . ^^^ FGF 23 elevations in ADHR are due to mutations of FGF 23 that block its degradation , in XLH from indirect actions of inactivating mutations of PHEX to modify the expression and / or degradation of FGF 23 and MEPE , and in TIO because of increased production of FGF 23 and MEPE . ^^^ Finally , additional studies are required to establish the molecular mechanisms of FGF 23 and MEPE actions on kidney and bone , as well as to confirm the role of these and other potential `` phosphatonins , ' ' such as frizzled related protein 4 , in the pathogenesis of the renal and skeletal phenotypes in XLH and TIO . ^^^ |
|
| Interacting proteins: P78562 and Q9GZV9 |
Pubmed |
SVM Score :0.0 |
| We investigated if the circulating levels of the phosphaturic factor FGF 23 are elevated in subjects with XLH . ^^^ Although we failed to find a statistically significant increase , FGF 23 levels were significantly correlated with the degree of hypophosphatemia in XLH . ^^^ METHODS : Fasting serum FGF 23 levels and serum biochemical parameters were measured using a human FGF 23 ( C terminal ) ELISA assay in 11 subjects with XLH and 42 age matched controls , 5 subjects with hypophosphatemia of unknown cause , and 14 hyperphosphatemic subjects with end stage renal disease ( ESRD ) . ^^^ RESULTS AND CONCLUSIONS : FGF 23 ( RU / ml ) concentrations were not different ( p = 0 . 11 ) between control and hypophosphatemic XLH subjects , but were significantly increased in hyperphosphatemic subjects with ESRD ( p < 0 . 001 ) . ^^^ There was a strong inverse correlation between FGF 23 and serum phosphorus ( r = 0 . 60 ) and calcium and phosphorus ( Ca 10 P ) product ( r = 0 . 65 ) in XLH , and a strong positive relationship between FGF 23 and Pi ( r = 0 . 50 ) and Ca 10 P product ( r = 0 . 62 ) in ESRD . ^^^ |
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| Interacting proteins: P78562 and Q9GZV9 |
Pubmed |
SVM Score :0.0 |
| A new class of phosphate regulating factors , collectively known as the phosphatonins , have been shown to be associated with the hypophosphatemic diseases , tumor induced osteomalacia ( TIO ) , 10 linked hypophosphatemic rickets ( XLH ) , and autosomal dominant hypophosphatemic rickets ( ADHR ) . ^^^ |
|
| Interacting proteins: P78562 and Q9GZV9 |
Pubmed |
SVM Score :0.0 |
| Rickets / osteomalacia is characterized by impaired mineralization of bone matrix . 10 linked hypophosphatemic rickets / osteomalacia ( XLH ) , autosomal dominant hypophosphatemic rickets / osteomalacia ( ADHR ) and tumor induced rickets / osteomalacia ( TIO ) share common clinical features including impaired proximal tubular phosphate reabsorption . ^^^ Moreover , FGF 23 levels were elevated in most patients with XLH suggesting FGF 23 is also involved in the pathogenesis of XLH . ^^^ |
|
| Interacting proteins: P78562 and Q9GZV9 |
Pubmed |
SVM Score :0.0 |
| The data indicate that FGF 23 induces phenotypic changes in mice resembling those of patients with ADHR , OOM , and XLH and that FGF 23 is an important determinant of Pi homeostasis and bone mineralization . . ^^^ The gene product , FGF 23 , is produced by tumors from patients with oncogenic osteomalacia ( OOM ) , circulates at increased levels in most patients with 10 linked hypophosphatemia ( XLH ) and is phosphaturic when injected into rats or mice , suggesting involvement in the regulation of phosphate ( Pi ) homeostasis . ^^^ |
|
| Interacting proteins: P78562 and Q9GZV9 |
Pubmed |
SVM Score :0.0 |
| Three metabolic bone diseases display similar characteristics such as hypophosphatemia due to chronically elevated renal phosphate clearance , inappropriately low 1 , 25 ( OH ) 2 vitamin D serum levels , and variable bone disease ( rickets and osteomalacia ) . 10 linked dominant hypophosphatemic rickets ( XLH ) , also called vitamin D resistant rickets and autosomal dominant hypophosphatemic rickets ( ADHR ) represent two inherited diseases , whereas oncogenic hypophosphatemia ( OHO ) , also known as tumor induced osteomalacia ( TIO ) , is an acquired paraneoplastic syndrome that , in certain aspects , has much in common with XLH and ADHR . ^^^ |
|
| Interacting proteins: P78562 and Q9GZV9 |
Pubmed |
SVM Score :0.0 |
| The pathogenesis of 10 linked hypophosphatemic rickets ( XLH ) , autosomal dominant hypophosphatemic rickets ( ADHR ) , and oncogenic osteomalacia ( OOM ) was discussed . . ^^^ |
|
| Interacting proteins: P78562 and Q9GZV9 |
Pubmed |
SVM Score :0.0 |
| Recently , it has been suggested that some putative P regulating factors might play an important role in the pathogenesis of hypophosphatemic disorders , including TIO , ADHR , and XLH . ^^^ |
|
| Interacting proteins: P78562 and Q9GZV9 |
Pubmed |
SVM Score :0.0 |
| The ADHR mutations in FGF 23 produce a protein species less susceptible to proteolytic processing . 10 linked hypophosphatemic rickets ( XLH ) , tumor induced osteomalacia ( TIO ) , and fibrous dysplasia of bone ( FD ) are disorders involving phosphate homeostasis that share phenotypes with ADHR , indicating that FGF 23 may be a common denominator for the pathophysiology of these syndromes . ^^^ |
|
| Interacting proteins: P78562 and Q9GZV9 |
Pubmed |
SVM Score :0.0 |
| In addition , FGF 23 was shown to be involved in the development of several diseases including autosomal dominant hypophosphatemic rickets / osteomalacia ( ADHR ) , 10 linked hypophosphatemic rickets / osteomalacia ( XLH ) and tumor induced rickets / osteomalacia ( TIO ) . ^^^ |
|
| Interacting proteins: P78562 and Q9GZV9 |
Pubmed |
SVM Score :0.0 |
| However , presence of some phosphaturic factors is assumed from investigations about TIO , XLH , ADHR . ^^^ |
|
| Interacting proteins: P78562 and Q9GZV9 |
Pubmed |
SVM Score :0.0 |
| This phenotype parallels that of patients with tumor induced osteomalacia ( TIO ) , 10 linked hypophosphatemic rickets ( XLH ) , and fibrous dysplasia ( FD ) , in whom elevated serum FGF 23 levels are often observed . ^^^ |
|
| Interacting proteins: P78562 and Q9GZV9 |
Pubmed |
SVM Score :0.0 |
| FGF 23 was recently shown to be involved in the development of several hypophosphatemic diseases , including 10 linked hypophosphatemic rickets / osteomalacia ( XLH ) and tumor induced rickets / osteomalacia ( TIO ) . ^^^ However , discrepant results concerning circulatory levels of FGF 23 in patients with TIO and XLH have been reported using these two assays . ^^^ We simultaneously measured FGF 23 levels in 13 patients with adult onset hypophosphatemic osteomalacia and 29 patients with XLH by these two assays . ^^^ The full length assay indicated that FGF 23 was above the upper limit of the reference range in all patients with osteomalacia and in 24 of 29 patients with XLH . ^^^ However , the C terminal assay in dicated that FGF 23 was within the reference range in 3 of 13 patients with osteomalacia and 16 of 29 patients with XLH . ^^^ |
|
| Interacting proteins: P78562 and Q9GZV9 |
Pubmed |
SVM Score :0.0 |
| Hereditary hypophosphatemic rickets groups together 10 linked hypophosphatemic rickets ( XLH ) , autosomal dominant hypophosphatemic rickets ( ADHR ) and hereditary hypophosphatemic rickets with hypercalciuria ( HHRH , autosomal recessive ) . ^^^ Mutations causing hereditary hypophosphatemic rickets involve PHEX located on Xp11 . 22 for XLH and FGF 23 located on 12p13 for ADHR . ^^^ |
|
| Interacting proteins: P78562 and Q9GZV9 |
Pubmed |
SVM Score :0.0 |
| The purpose of this review paper is to provide a summary of recent developments in the childhood disorders of renal phosphate wasting , with particular emphasis on two of the hereditary conditions , 10 linked hypophosphatemia ( XLH ) and autosomal dominant hypophosphatemic rickets ( ADHR ) , as they are distinguished from the paraneoplastic syndrome , oncogenic hypophosphatemic osteomalacia ( OHO ) . ^^^ |
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| Interacting proteins: P78562 and Q9GZV9 |
Pubmed |
SVM Score :0.0 |
| A case of neuroendocrine oncogenic osteomalacia associated with a PHEX and fibroblast growth factor 23 expressing sinusidal malignant schwannoma . ^^^ The cell line did express PHEX ( phosphate regulating gene with homologies to endopeptidases located on the 10 chromosome ) and FGF 23 ( fibroblast growth factor 23 ) transcripts on northern hybridization ; however , none of the known mutations from the related mendelian disorders of 10 linked hypophosphatemic rickets or autosomal dominant hypophosphatemic rickets could be detected . ^^^ HMS 97 cells might be useful for further studies that aim to determine the genetic mechanism that leads to the observed PHEX and FGF 23 expression , both of which might have a direct role in the pathogenesis of oncogenic osteomalacia . ^^^ |
|
| Interacting proteins: P78562 and Q9GZV9 |
Pubmed |
SVM Score :0.0 |
| In addition , we failed to detect the Phex substrate FGF 23 in osteoblasts . ^^^ |
|
| Interacting proteins: P78562 and Q9GZV9 |
Pubmed |
SVM Score :0.0 |
| Of great interest is the recent identification of novel Pi regulating genes , PHEX and FGF 23 , that play a role in the pathophysiology of inherited ( 10 linked hypophosphatemia and autosomal dominant hypophosphatemic rickets ) and acquired ( oncogenic hypophosphatemic rickets ) disorders characterized by renal Pi wasting and associated skeletal abnormalities . ^^^ Studies are currently underway to elucidate the molecular basis for impaired renal Pi reabsorption in these disorders and to determine the precise physiological role of PHEX and FGF 23 in the regulation of Pi homeostasis . . ^^^ |
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| Interacting proteins: P78562 and Q9GZV9 |
Pubmed |
SVM Score :0.0 |
| Human recombinant endopeptidase PHEX has a strict S 1 ' specificity for acidic residues and cleaves peptides derived from fibroblast growth factor 23 and matrix extracellular phosphoglycoprotein . ^^^ In addition , using quenched fluorescence peptides derived from fibroblast growth factor 23 and matrix extracellular phosphoglycoprotein sequences flanked by Abz and N ( 2 , 4 dinitrophenyl ) ethylenediamine , we showed that these physiologically relevant proteins are potential PHEX substrates . ^^^ |
|
| Interacting proteins: P78562 and Q9GZV9 |
Pubmed |
SVM Score :0.0 |
| Clinical and laboratory findings in this disorder are similar to those in oncogenic osteomalacia , in which tumors abundantly express FGF 23 messenger RNA , and to those in 10 linked hypophosphatemia , which is caused by inactivating mutations in a phosphate regulating endopeptidase called PHEX . ^^^ |
|
| Interacting proteins: P78562 and Q9GZV9 |
Pubmed |
SVM Score :0.0 |
| Recent advances in the understanding of these diseases include discovery of mutations in the genes encoding human phosphate regulating gene with homologies to endopeptidases on the 10 chromosome ( PHEX ) and fibroblast growth factor 23 ( FGF 23 ) and the finding of overproduction of FGF 23 and other proteins including matrix extracellular phosphoglycoprotein ( MEPE ) and frizzled related protein 4 ( FRP 4 ) in tumor induced osteomalacia . ^^^ |
|
| Interacting proteins: P78562 and Q9GZV9 |
Pubmed |
SVM Score :0.0 |
| Sequence analyses of the PHEX and FGF 23 genes were normal . ^^^ |
|
| Interacting proteins: P78562 and Q9GZV9 |
Pubmed |
SVM Score :0.0 |
| Many unanswered questions remain , including the relationship between PHEX ( phosphate regulating gene with homologies to endopeptidases on the 10 chromosome ) mutations and elevated fibroblast growth factor 23 . ^^^ |
|
| Interacting proteins: P78562 and Q9GZV9 |
Pubmed |
SVM Score :0.0 |
| The identification of FGF 23 as a factor involved in several disorders of phosphate regulation and of PHEX as the gene mutated in 10 linked Hypophosphatemic Rickets indicates that both these genes may be involved in phosphate homeostasis , although their physiological roles are unclear . ^^^ FGF 23 expression in the bone cells increased with increased mineralization over a 20 day treatment period under mineralizing conditions with beta glycerophosphate , while PHEX expression decreased . ^^^ |
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| Interacting proteins: P78562 and Q9GZV9 |
Pubmed |
SVM Score :0.0 |
| The type 2 c transporter is also regulated by PTH , FGF 23 and PHEX . ^^^ Studying the mechanisms of the regulation of type 2 c transporters by PHEX and FGF 23 has increased understanding of the control of proximal tubular Pi handling and bone mineralization . . ^^^ |
|
| Interacting proteins: P78562 and Q9GZV9 |
Pubmed |
SVM Score :0.0 |
| The FTC phenotype is regarded as the metabolic mirror image of hypophosphatemic conditions , where causal mutations are known in genes FGF 23 or PHEX . ^^^ |
|
| Interacting proteins: P78562 and Q9GZV9 |
Pubmed |
SVM Score :0.0 |
| Recent studies of inherited and acquired hypophosphatemia which exhibit similar biochemical and clinical features , have led to the identification of novel genes , phosphate regulating gene with homologies to endopeptidases on the 10 chromosome ( PHEX ) and fibroblast growth factor 23 ( FGF 23 ) , that play a role in the regulation of Pi homeostasis . ^^^ |
|
| Interacting proteins: P78562 and Q9GZV9 |
Pubmed |
SVM Score :0.0 |
| Molecular studies in patients with renal Pi wasting resulted in the identification of novel regulators of Pi homeostasis : fibroblast growth factor 23 ( FGF 23 ) and a phosphate regulating gene with homologies to endopeptidases on the 10 chromosome ( PHEX ) . ^^^ In mouse models , increased FGF 23 production or loss of Phex function causes hypophosphatemia and decreased renal Pi reabsorption , secondary to decreased BBM Npt2a protein abundance . ^^^ |
|
| Interacting proteins: P78562 and Q9GZV9 |
Pubmed |
SVM Score :0.0 |
| A review of medical records and mutational analyses of the PHEX and FGF 23 genes were performed on 17 unrelated Korean children with HR . ^^^ |
|
| Interacting proteins: P78562 and Q9GZV9 |
Pubmed |
SVM Score :0.0 |
| Genetic studies of these disorders have identified mutations in PHEX and FGF 23 as the causes of 10 linked dominant disorder and autosomal dominant forms , respectively . ^^^ METHODS AND RESULTS : Direct nucleotide sequencing of FGF 23 and PHEX revealed that the elder daughter was heterozygous for an R567X mutation in PHEX , rather than FGF 23 , suggesting that the genetic transmission occurred as an 10 linked dominant trait . ^^^ |
|
| Interacting proteins: P78562 and Q9GZV9 |
Pubmed |
SVM Score :0.0 |
| Recent data identifying a novel mechanism of phosphaturia in 10 linked hypophosphatemic rickets , autosomal dominant hypophosphatemic rickets , and oncogenic osteomalacia demonstrate that elevated levels of novel circulating phosphaturic factors such as fibroblast growth factor 23 ( FGF 23 ) and PHEX are responsible for phosphate wasting . ^^^ |
|
| Interacting proteins: P78562 and Q9GZV9 |
Pubmed |
SVM Score :0.0 |
| Defective PHEX function leaves phosphaturic peptides such as FGF 23 uncleaved , enabling these peptides , known as phosphatonins , to fully exert their phosphaturic potential in the proximal tubule of the kidney . ^^^ |
|
| Interacting proteins: Q9GZV9 and P78562 |
Pubmed |
SVM Score :0.0 |
| A case of neuroendocrine oncogenic osteomalacia associated with a PHEX and fibroblast growth factor 23 expressing sinusidal malignant schwannoma . ^^^ The cell line did express PHEX ( phosphate regulating gene with homologies to endopeptidases located on the 10 chromosome ) and FGF 23 ( fibroblast growth factor 23 ) transcripts on northern hybridization ; however , none of the known mutations from the related mendelian disorders of 10 linked hypophosphatemic rickets or autosomal dominant hypophosphatemic rickets could be detected . ^^^ |
|
| Interacting proteins: Q9GZV9 and P78562 |
Pubmed |
SVM Score :0.0 |
| PHEX is thought to inactivate a phosphaturic factor , which may be fibroblast growth factor 23 ( FGF ) 23 . ^^^ |
|
| Interacting proteins: Q9GZV9 and P78562 |
Pubmed |
SVM Score :0.0 |
| Molecular studies in patients with renal Pi wasting resulted in the identification of novel regulators of Pi homeostasis : fibroblast growth factor 23 ( FGF 23 ) and a phosphate regulating gene with homologies to endopeptidases on the 10 chromosome ( PHEX ) . ^^^ |
|
| Interacting proteins: Q9GZV9 and P78562 |
Pubmed |
SVM Score :0.0 |
| Recent data identifying a novel mechanism of phosphaturia in 10 linked hypophosphatemic rickets , autosomal dominant hypophosphatemic rickets , and oncogenic osteomalacia demonstrate that elevated levels of novel circulating phosphaturic factors such as fibroblast growth factor 23 ( FGF 23 ) and PHEX are responsible for phosphate wasting . ^^^ |
|
| Interacting proteins: Q9GZV9 and P78562 |
Pubmed |
SVM Score :0.0 |
| In addition , we failed to detect the Phex substrate FGF 23 in osteoblasts . ^^^ |
|
| Interacting proteins: Q9GZV9 and P78562 |
Pubmed |
SVM Score :0.0 |
| There is not yet enough evidence that FGF 23 is phosphatonin , and the relation between PHEX and FGF 23 is unclear . ^^^ |
|
| Interacting proteins: Q9GZV9 and P78562 |
Pubmed |
SVM Score :0.0 |
| Human recombinant endopeptidase PHEX has a strict S 1 ' specificity for acidic residues and cleaves peptides derived from fibroblast growth factor 23 and matrix extracellular phosphoglycoprotein . ^^^ In addition , using quenched fluorescence peptides derived from fibroblast growth factor 23 and matrix extracellular phosphoglycoprotein sequences flanked by Abz and N ( 2 , 4 dinitrophenyl ) ethylenediamine , we showed that these physiologically relevant proteins are potential PHEX substrates . ^^^ |
|
| Interacting proteins: Q9GZV9 and P78562 |
Pubmed |
SVM Score :0.0 |
| Recent advances in the understanding of these diseases include discovery of mutations in the genes encoding human phosphate regulating gene with homologies to endopeptidases on the 10 chromosome ( PHEX ) and fibroblast growth factor 23 ( FGF 23 ) and the finding of overproduction of FGF 23 and other proteins including matrix extracellular phosphoglycoprotein ( MEPE ) and frizzled related protein 4 ( FRP 4 ) in tumor induced osteomalacia . ^^^ |
|
| Interacting proteins: Q9GZV9 and P78562 |
Pubmed |
SVM Score :0.0 |
| Many unanswered questions remain , including the relationship between PHEX ( phosphate regulating gene with homologies to endopeptidases on the 10 chromosome ) mutations and elevated fibroblast growth factor 23 . ^^^ |
|
| Interacting proteins: Q9GZV9 and P78562 |
Pubmed |
SVM Score :0.0 |
| The primary defects in 10 linked hypophosphatemic rickets ( HYP ) and autosomal dominant hypophosphatemic rickets ( ADHR ) are now identified as inactivating mutations in a Zn metalloendopeptidase ( PHEX ) and activating mutations in fibroblast growth factor 23 ( FGF 23 ) , respectively . ^^^ |
|
| Interacting proteins: Q9GZV9 and P78562 |
Pubmed |
SVM Score :0.0 |
| Homozygous ablation of fibroblast growth factor 23 results in hyperphosphatemia and impaired skeletogenesis , and reverses hypophosphatemia in Phex deficient mice . ^^^ |
|
| Interacting proteins: Q9GZV9 and P78562 |
Pubmed |
SVM Score :0.0 |
| Recent studies of inherited and acquired hypophosphatemia which exhibit similar biochemical and clinical features , have led to the identification of novel genes , phosphate regulating gene with homologies to endopeptidases on the 10 chromosome ( PHEX ) and fibroblast growth factor 23 ( FGF 23 ) , that play a role in the regulation of Pi homeostasis . ^^^ |
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| Interacting proteins: Q9GZV9 and P78562 |
Pubmed |
SVM Score :0.0 |
| Of great interest is the recent identification of novel Pi regulating genes , PHEX and FGF 23 , that play a role in the pathophysiology of inherited ( 10 linked hypophosphatemia and autosomal dominant hypophosphatemic rickets ) and acquired ( oncogenic hypophosphatemic rickets ) disorders characterized by renal Pi wasting and associated skeletal abnormalities . ^^^ Studies are currently underway to elucidate the molecular basis for impaired renal Pi reabsorption in these disorders and to determine the precise physiological role of PHEX and FGF 23 in the regulation of Pi homeostasis . . ^^^ |
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| Interacting proteins: Q9GZV9 and P78562 |
Pubmed |
SVM Score :0.0 |
| In addition , the elevated serum FGF 23 levels were demonstrated in most patients with XLH . ^^^ It is likely that increased serum levels of FGF 23 contributes to the development of hypophosphatemia not only in TIO but also in XLH . . ^^^ |
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| Interacting proteins: Q9GZV9 and P78562 |
Pubmed |
SVM Score :0.0 |
| Genetic studies of autosomal dominant hypophosphatemic rickets ( ADHR ) and 10 linked hypophosphatemia ( XLH ) identified the phosphaturic hormone FGF 23 and the membrane metalloprotease PHEX , and investigations of tumor induced osteomalacia ( TIO ) discovered the extracellular matrix protein MEPE . ^^^ FGF 23 elevations in ADHR are due to mutations of FGF 23 that block its degradation , in XLH from indirect actions of inactivating mutations of PHEX to modify the expression and / or degradation of FGF 23 and MEPE , and in TIO because of increased production of FGF 23 and MEPE . ^^^ Finally , additional studies are required to establish the molecular mechanisms of FGF 23 and MEPE actions on kidney and bone , as well as to confirm the role of these and other potential `` phosphatonins , ' ' such as frizzled related protein 4 , in the pathogenesis of the renal and skeletal phenotypes in XLH and TIO . ^^^ FGF 23 , PHEX , and MEPE regulation of phosphate homeostasis and skeletal mineralization . ^^^ There is evidence for a hormone / enzyme / extracellular matrix protein cascade involving fibroblastic growth factor 23 ( FGF 23 ) , a phosphate regulating gene with homologies to endopeptidases on the 10 chromosome ( PHEX ) , and a matrix extracellular phosphoglycoprotein ( MEPE ) that regulates systemic phosphate homeostasis and mineralization . ^^^ |
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| Interacting proteins: Q9GZV9 and P78562 |
Pubmed |
SVM Score :0.0 |
| We investigated if the circulating levels of the phosphaturic factor FGF 23 are elevated in subjects with XLH . ^^^ Although we failed to find a statistically significant increase , FGF 23 levels were significantly correlated with the degree of hypophosphatemia in XLH . ^^^ METHODS : Fasting serum FGF 23 levels and serum biochemical parameters were measured using a human FGF 23 ( C terminal ) ELISA assay in 11 subjects with XLH and 42 age matched controls , 5 subjects with hypophosphatemia of unknown cause , and 14 hyperphosphatemic subjects with end stage renal disease ( ESRD ) . ^^^ RESULTS AND CONCLUSIONS : FGF 23 ( RU / ml ) concentrations were not different ( p = 0 . 11 ) between control and hypophosphatemic XLH subjects , but were significantly increased in hyperphosphatemic subjects with ESRD ( p < 0 . 001 ) . ^^^ There was a strong inverse correlation between FGF 23 and serum phosphorus ( r = 0 . 60 ) and calcium and phosphorus ( Ca 10 P ) product ( r = 0 . 65 ) in XLH , and a strong positive relationship between FGF 23 and Pi ( r = 0 . 50 ) and Ca 10 P product ( r = 0 . 62 ) in ESRD . ^^^ |
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| Interacting proteins: Q9GZV9 and P78562 |
Pubmed |
SVM Score :0.0 |
| Elevated serum FGF 23 levels correlate with the degree of hypophosphatemia in XLH , suggesting that loss of Phex function in this disorder results in either diminished degradation and / or increased biosynthesis of FGF 23 . ^^^ To establish the mechanisms whereby Phex regulates FGF 23 , we assessed Phex dependent hydrolysis of recombinant FGF 23 in vitro and measured fgf 23 message levels in the Hyp mouse homologue of XLH . ^^^ In addition , Hyp mice displayed a bone restricted increase in fgf 23 transcripts in association with inactivating Phex mutations . ^^^ Increased expression of fgf 23 was also observed in Hyp derived osteoblasts in culture . ^^^ Phex dependent hydrolysis of full length FGF 23 or its N and C terminal fragments could not be demonstrated in the presence or absence of decanoyl Arg Val Lys Arg chloromethyl ketone in COS 7 cells expressing Phex and FGF 23 . ^^^ |
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| Interacting proteins: Q9GZV9 and P78562 |
Pubmed |
SVM Score :0.0 |
| Clinical and laboratory findings in this disorder are similar to those in oncogenic osteomalacia , in which tumors abundantly express FGF 23 messenger RNA , and to those in 10 linked hypophosphatemia , which is caused by inactivating mutations in a phosphate regulating endopeptidase called PHEX . ^^^ |
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| Interacting proteins: Q9GZV9 and P78562 |
Pubmed |
SVM Score :0.0 |
| Recent molecular insight has put fibroblast growth factor ( FGF 23 ) into the centre of pathophysiological considerations because of ( 1 ) overproduction ( tumour associated osteomalacia ) or ( 2 ) hypothetically , accumulation resulting from mutations causing resistance to processing or degradation ( autosomal dominant hypophosphataemia ) or ( 3 ) loss of function of a protease ( PHEX ) interfering with FGF 23 breakdown ( XLH ) . ^^^ |
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| Interacting proteins: Q9GZV9 and P78562 |
Pubmed |
SVM Score :0.0 |
| Moreover , FGF 23 levels were elevated in most patients with XLH suggesting FGF 23 is also involved in the pathogenesis of XLH . ^^^ |
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| Interacting proteins: Q9GZV9 and P78562 |
Pubmed |
SVM Score :0.0 |
| Sequence analyses of the PHEX and FGF 23 genes were normal . ^^^ |
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| Interacting proteins: Q9GZV9 and P78562 |
Pubmed |
SVM Score :0.0 |
| The gene product , FGF 23 , is produced by tumors from patients with oncogenic osteomalacia ( OOM ) , circulates at increased levels in most patients with 10 linked hypophosphatemia ( XLH ) and is phosphaturic when injected into rats or mice , suggesting involvement in the regulation of phosphate ( Pi ) homeostasis . ^^^ The data indicate that FGF 23 induces phenotypic changes in mice resembling those of patients with ADHR , OOM , and XLH and that FGF 23 is an important determinant of Pi homeostasis and bone mineralization . . ^^^ |
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| Interacting proteins: Q9GZV9 and P78562 |
Pubmed |
SVM Score :0.0 |
| The identification of FGF 23 as a factor involved in several disorders of phosphate regulation and of PHEX as the gene mutated in 10 linked Hypophosphatemic Rickets indicates that both these genes may be involved in phosphate homeostasis , although their physiological roles are unclear . ^^^ FGF 23 expression in the bone cells increased with increased mineralization over a 20 day treatment period under mineralizing conditions with beta glycerophosphate , while PHEX expression decreased . ^^^ |
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| Interacting proteins: Q9GZV9 and P78562 |
Pubmed |
SVM Score :0.0 |
| The type 2 c transporter is also regulated by PTH , FGF 23 and PHEX . ^^^ Studying the mechanisms of the regulation of type 2 c transporters by PHEX and FGF 23 has increased understanding of the control of proximal tubular Pi handling and bone mineralization . . ^^^ |
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| Interacting proteins: Q9GZV9 and P78562 |
Pubmed |
SVM Score :0.0 |
| The FTC phenotype is regarded as the metabolic mirror image of hypophosphatemic conditions , where causal mutations are known in genes FGF 23 or PHEX . ^^^ |
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| Interacting proteins: Q9GZV9 and P78562 |
Pubmed |
SVM Score :0.0 |
| The authors found that Hyp mice had increased expression of the MEPE and another phosphaturic factor , Fgf 23 . ^^^ Increased Fgf 23 levels in Hyp mice were not affected by superimposed Mepe deficiency . ^^^ |
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| Interacting proteins: Q9GZV9 and P78562 |
Pubmed |
SVM Score :0.0 |
| The ADHR mutations in FGF 23 produce a protein species less susceptible to proteolytic processing . 10 linked hypophosphatemic rickets ( XLH ) , tumor induced osteomalacia ( TIO ) , and fibrous dysplasia of bone ( FD ) are disorders involving phosphate homeostasis that share phenotypes with ADHR , indicating that FGF 23 may be a common denominator for the pathophysiology of these syndromes . ^^^ |
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| Interacting proteins: Q9GZV9 and P78562 |
Pubmed |
SVM Score :0.0 |
| In addition , FGF 23 was shown to be involved in the development of several diseases including autosomal dominant hypophosphatemic rickets / osteomalacia ( ADHR ) , 10 linked hypophosphatemic rickets / osteomalacia ( XLH ) and tumor induced rickets / osteomalacia ( TIO ) . ^^^ |
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| Interacting proteins: Q9GZV9 and P78562 |
Pubmed |
SVM Score :0.0 |
| A review of medical records and mutational analyses of the PHEX and FGF 23 genes were performed on 17 unrelated Korean children with HR . ^^^ |
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| Interacting proteins: Q9GZV9 and P78562 |
Pubmed |
SVM Score :0.0 |
| This phenotype parallels that of patients with tumor induced osteomalacia ( TIO ) , 10 linked hypophosphatemic rickets ( XLH ) , and fibrous dysplasia ( FD ) , in whom elevated serum FGF 23 levels are often observed . ^^^ |
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| Interacting proteins: Q9GZV9 and P78562 |
Pubmed |
SVM Score :0.0 |
| To determine whether the serum FGF 23 concentration is regulated by dietary intake of Pi , we fed wild type ( WT ) , Npt2a gene ablated ( Npt2a ( / ) ) , and Hyp mice diets containing varying Pi contents ( 0 . 02 1 . 65 % ) . ^^^ The serum concentrations of FGF 23 in Hyp mice were 5 to 25 fold higher than values in WT mice , and the values varied with dietary Pi intake . ^^^ Fgf 23 mRNA abundance in calvaria was significantly higher in Hyp mice than in WT mice on the 1 % Pi diet ; in both groups of mice , fgf 23 mRNA abundance in calvarial bone was suppressed by 85 % on the low ( 0 . 02 % ) Pi diet . ^^^ The present data demonstrate that dietary Pi regulates the serum FGF 23 concentration in mice , and such regulation is independent of phex function . ^^^ |
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| Interacting proteins: Q9GZV9 and P78562 |
Pubmed |
SVM Score :0.0 |
| FGF 23 was recently shown to be involved in the development of several hypophosphatemic diseases , including 10 linked hypophosphatemic rickets / osteomalacia ( XLH ) and tumor induced rickets / osteomalacia ( TIO ) . ^^^ However , discrepant results concerning circulatory levels of FGF 23 in patients with TIO and XLH have been reported using these two assays . ^^^ We simultaneously measured FGF 23 levels in 13 patients with adult onset hypophosphatemic osteomalacia and 29 patients with XLH by these two assays . ^^^ The full length assay indicated that FGF 23 was above the upper limit of the reference range in all patients with osteomalacia and in 24 of 29 patients with XLH . ^^^ However , the C terminal assay in dicated that FGF 23 was within the reference range in 3 of 13 patients with osteomalacia and 16 of 29 patients with XLH . ^^^ |
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| Interacting proteins: Q9GZV9 and P78562 |
Pubmed |
SVM Score :0.0 |
| Genetic studies of these disorders have identified mutations in PHEX and FGF 23 as the causes of 10 linked dominant disorder and autosomal dominant forms , respectively . ^^^ METHODS AND RESULTS : Direct nucleotide sequencing of FGF 23 and PHEX revealed that the elder daughter was heterozygous for an R567X mutation in PHEX , rather than FGF 23 , suggesting that the genetic transmission occurred as an 10 linked dominant trait . ^^^ |
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| Interacting proteins: Q9GZV9 and P78562 |
Pubmed |
SVM Score :0.0 |
| Mutations causing hereditary hypophosphatemic rickets involve PHEX located on Xp11 . 22 for XLH and FGF 23 located on 12p13 for ADHR . ^^^ |
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| Interacting proteins: Q9GZV9 and P78562 |
Pubmed |
SVM Score :0.0 |
| Several hereditary disorders of isolated phosphate wasting have been described , including 10 linked hypophosphataemic rickets ( XLH ) , hypophosphataemic bone disease ( HBD ) , hereditary hypophosphataemic rickets with hypercalciuria ( HHRH ) and autosomal dominant hypophosphataemic rickets ( ADHR ) . ^^^ |
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| Interacting proteins: Q9GZV9 and P78562 |
Pubmed |
SVM Score :0.0 |
| Oncogenic osteomalacia ( OOM ) , 10 linked hypophosphatemia ( XLH ) , and autosomal dominant hypophosphatemic rickets ( ADHR ) are phenotypically similar disorders characterized by hypophosphatemia , decreased renal phosphate reabsorption , normal or low serum calcitriol concentrations , normal serum concentrations of calcium and parathyroid hormone , and defective skeletal mineralization . ^^^ XLH results from mutations in the PHEX gene , encoding a membrane bound endopeptidase , whereas ADHR is associated with mutations of the gene encoding FGF 23 . ^^^ XLH results from mutations in the PHEX gene , encoding a membrane bound endopeptidase , whereas ADHR is associated with mutations of the gene encoding FGF 23 . ^^^ |
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| Interacting proteins: Q9GZV9 and P78562 |
Pubmed |
SVM Score :0.0 |
| A new class of phosphate regulating factors , collectively known as the phosphatonins , have been shown to be associated with the hypophosphatemic diseases , tumor induced osteomalacia ( TIO ) , 10 linked hypophosphatemic rickets ( XLH ) , and autosomal dominant hypophosphatemic rickets ( ADHR ) . ^^^ |
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| Interacting proteins: Q9GZV9 and P78562 |
Pubmed |
SVM Score :0.0 |
| Inactivating mutations of the PHEX ( phosphate regulating gene with homologies to endopeptidases on the 10 chromosome ) endopeptidase , the disease causing gene in 10 linked hypophosphatemia ( XLH ) , results in increased circulating levels of fibroblastic growth factor 23 ( FGF 23 ) , a bone derived phosphaturic factor . ^^^ To determine the causal role of FGF 23 in XLH , we generated a combined Fgf 23 deficient enhanced green fluorescent protein ( eGFP ) reporter and Phex deficient Hyp mouse model ( Fgf 23 ( + / ) / Hyp ) . eGFP expression was expressed in osteocytes embedded in bone that exhibited marked upregulation of eGFP in response to Phex deficiency and in CD 31 positive cells in bone marrow venules that expressed low eGFP levels independently of Phex . ^^^ Pathogenic role of Fgf 23 in Hyp mice . ^^^ In bone marrow stromal cells ( BMSCs ) derived from Fgf 23 ( / ) / Hyp mice , eGFP expression was also selectively increased in osteocyte like cells within mineralization nodules and detected in low levels in CD 31 positive cells . ^^^ Regardless , the deletion of Fgf 23 from Hyp mice reversed the hypophosphatemia , abnormal 1 , 25 ( OH ) ( 2 ) D ( 3 ) levels , rickets , and osteomalacia associated with Phex deficiency . ^^^ |
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| Interacting proteins: Q9GZV9 and P78562 |
Pubmed |
SVM Score :0.0 |
| Defective PHEX function leaves phosphaturic peptides such as FGF 23 uncleaved , enabling these peptides , known as phosphatonins , to fully exert their phosphaturic potential in the proximal tubule of the kidney . ^^^ |
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| Interacting proteins: Q9GZV9 and P78562 |
Pubmed |
SVM Score :0.0 |
| The purpose of this review paper is to provide a summary of recent developments in the childhood disorders of renal phosphate wasting , with particular emphasis on two of the hereditary conditions , 10 linked hypophosphatemia ( XLH ) and autosomal dominant hypophosphatemic rickets ( ADHR ) , as they are distinguished from the paraneoplastic syndrome , oncogenic hypophosphatemic osteomalacia ( OHO ) . ^^^ |
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| Interacting proteins: Q9GZV9 and P78562 |
Pubmed |
SVM Score :0.0 |
| Mutation at a locus ( HPDR ) on the 10 chromosome ( McKusick 30780 [ HPDR 1 ] ; 30781 [ HPDR 2 ] ) causes impaired renal phosphate transport , hypophosphatemia , and an associated impairment in the process of mineralization in bone and teeth ( 10 linked hypophosphatemia [ XLH ] ) . ^^^ |
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| Interacting proteins: Q9GZV9 and P78562 |
Pubmed |
SVM Score :0.0 |
| The products of the Rhizobium leguminosarum hyp gene cluster are necessary for synthesis of a functional uptake [ NiFe ] hydrogenase system in symbiosis with pea plants , and at least for HypB and HypF , a role in hydrogenase specific nickel metabolism has been postulated ( L . ^^^ |
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| Interacting proteins: Q9GZV9 and P78562 |
Pubmed |
SVM Score :0.0 |
| Six genes , arranged as a single operon , were identified , and designated hypA , B , F , C , D and E based on the sequence similarities of all of them , except hypF , to genes from the hydrogenase pleiotropic operon ( hyp ) from Escherichia coli . ^^^ |
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| Interacting proteins: Q9GZV9 and P78562 |
Pubmed |
SVM Score :0.0 |
| Three metabolic bone diseases display similar characteristics such as hypophosphatemia due to chronically elevated renal phosphate clearance , inappropriately low 1 , 25 ( OH ) 2 vitamin D serum levels , and variable bone disease ( rickets and osteomalacia ) . 10 linked dominant hypophosphatemic rickets ( XLH ) , also called vitamin D resistant rickets and autosomal dominant hypophosphatemic rickets ( ADHR ) represent two inherited diseases , whereas oncogenic hypophosphatemia ( OHO ) , also known as tumor induced osteomalacia ( TIO ) , is an acquired paraneoplastic syndrome that , in certain aspects , has much in common with XLH and ADHR . ^^^ |
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| Interacting proteins: Q9GZV9 and P78562 |
Pubmed |
SVM Score :0.0 |
| The pathogenesis of 10 linked hypophosphatemic rickets ( XLH ) , autosomal dominant hypophosphatemic rickets ( ADHR ) , and oncogenic osteomalacia ( OOM ) was discussed . . ^^^ |
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| Interacting proteins: Q9GZV9 and P78562 |
Pubmed |
SVM Score :0.0 |
| Recently , it has been suggested that some putative P regulating factors might play an important role in the pathogenesis of hypophosphatemic disorders , including TIO , ADHR , and XLH . ^^^ |
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| Interacting proteins: Q9GZV9 and P78562 |
Pubmed |
SVM Score :0.0 |
| However , presence of some phosphaturic factors is assumed from investigations about TIO , XLH , ADHR . ^^^ |
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| Interacting proteins: Q9GZV9 and P78562 |
Pubmed |
SVM Score :0.0 |
| The sequences of hypF , hypC and hypD complete the hyp gene cluster required for hydrogenase activity in Bradyrhizobium japonicum . ^^^ |
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| Interacting proteins: Q9GZV9 and P78562 |
Pubmed |
SVM Score :0.0 |
| Despite a high identity between the two copies of hyp gene products , substantial structural differences were identified between the two copies of hypF genes . ^^^ |
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| Interacting proteins: Q9GZV9 and P78562 |
Pubmed |
SVM Score :0.0 |
| The biosynthesis of [ NiFe ] hydrogenases is a complex process that requires the function of the Hyp proteins HypA , HypB , HypC , HypD , HypE , HypF , and HypX for assembly of the H ( 2 ) activating [ NiFe ] site . ^^^ We conclude that the entire Hyp apparatus ( HypA , HypB , HypC , HypD , HypE , and HypF ) is involved in an efficient incorporation of the [ NiFe ] center into the RH . . ^^^ |
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| Interacting proteins: Q9GZV9 and P78562 |
Pubmed |
SVM Score :0.0 |
| Our lab previously characterized mutants in the accessory hyp genes , hypA , hypB , hypD and hypF that were all severely affected for hydrogenase activity , and in some cases ( hypA and hypB mutants ) also affected for urease activity . ^^^ |
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| Interacting proteins: Q9GZV9 and P78562 |
Pubmed |
SVM Score :0.0 |
| NA |
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| Interacting proteins: Q9GZV9 and P78562 |
Pubmed |
SVM Score :0.0 |
| NA |
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| Interacting proteins: Q9GZV9 and P78562 |
Pubmed |
SVM Score :0.0 |
| NA |
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| Interacting proteins: Q9GZV9 and P78562 |
Pubmed |
SVM Score :0.0 |
| NA |
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| Interacting proteins: Q9GZV9 and P78562 |
Pubmed |
SVM Score :0.0 |
| NA |
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| Interacting proteins: Q9GZV9 and P78562 |
Pubmed |
SVM Score :0.0 |
| NA |
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| Interacting proteins: Q9GZV9 and P78562 |
Pubmed |
SVM Score :0.0 |
| NA |
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| Interacting proteins: Q9GZV9 and P78562 |
Pubmed |
SVM Score :0.0 |
| NA |
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| Interacting proteins: Q9GZV9 and P78562 |
Pubmed |
SVM Score :0.0 |
| NA |
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| Interacting proteins: Q9GZV9 and P78562 |
Pubmed |
SVM Score :0.0 |
| NA |
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| Interacting proteins: Q9GZV9 and P78562 |
Pubmed |
SVM Score :0.0 |
| NA |
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