Pex/PEX tissue distribution and evidence for a deletion in the 3' region of the Pex gene in X-linked hypophosphatemic mice
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Cloning of human PEX cDNA. Expression, subcellular localization, and endopeptidase activityHuman recombinant endopeptidase PHEX has a strict S1' specificity for acidic residues and cleaves peptides derived from fibroblast growth factor-23 and matrix extracellular phosphoglycoproteinFGF23 and Phosphate Wasting DisordersFibroblast Growth Factor 23: A New Dimension to Diseases of Calcium-Phosphorus MetabolismExtracellular matrix mineralization in periodontal tissues: Noncollagenous matrix proteins, enzymes, and relationship to hypophosphatasia and X-linked hypophosphatemiaExcessive Osteocytic Fgf23 Secretion Contributes to Pyrophosphate Accumulation and Mineralization Defect in Hyp MiceDysregulated gene expression in the primary osteoblasts and osteocytes isolated from hypophosphatemic Hyp miceProximal tubular phosphate reabsorption: molecular mechanismsFGF23, PHEX, and MEPE regulation of phosphate homeostasis and skeletal mineralizationProteolytic processing of osteopontin by PHEX and accumulation of osteopontin fragments in Hyp mouse bone, the murine model of X-linked hypophosphatemiaCharacterization of PHEX endopeptidase catalytic activity: identification of parathyroid-hormone-related peptide107-139 as a substrate and osteocalcin, PPi and phosphate as inhibitorsRole of matrix extracellular phosphoglycoprotein in the pathogenesis of X-linked hypophosphatemiaPTHrP(1-34)-mediated repression of the PHEX gene in osteoblastic cells involves the transcriptional repressor E4BP4Differential regulation of PHEX expression in bone and parathyroid gland by chronic renal insufficiency and 1,25-dihydroxyvitamin D3The mouse Na(+)-sulfate cotransporter gene Nas1. Cloning, tissue distribution, gene structure, chromosomal assignment, and transcriptional regulation by vitamin DTargeted inactivation of Npt2 in mice leads to severe renal phosphate wasting, hypercalciuria, and skeletal abnormalitiesAmelogenesis imperfecta and other biomineralization defects in Fam20a and Fam20c null miceIsolated C-terminal tail of FGF23 alleviates hypophosphatemia by inhibiting FGF23-FGFR-Klotho complex formation.Nuclear isoforms of fibroblast growth factor 2 are novel inducers of hypophosphatemia via modulation of FGF23 and KLOTHOIncreased bone volume and correction of HYP mouse hypophosphatemia in the Klotho/HYP mouse.Circulating fibroblast growth factor 23 in patients with end-stage renal disease treated by peritoneal dialysis is intact and biologically activeEffects of Npt2 gene ablation and low-phosphate diet on renal Na(+)/phosphate cotransport and cotransporter gene expression.Expression and distribution of SIBLING proteins in the predentin/dentin and mandible of hyp mice.The molecular background to hypophosphataemic rickets.Genetic diagnosis of X-linked dominant Hypophosphatemic Rickets in a cohort study: tubular reabsorption of phosphate and 1,25(OH)2D serum levels are associated with PHEX mutation type.Phosphatonin washout in Hyp mice proximal tubules: evidence for posttranscriptional regulation.Hypophosphatemic rickets: revealing novel control points for phosphate homeostasis.Mutational analysis of PHEX, FGF23 and DMP1 in a cohort of patients with hypophosphatemic rickets.Recent advances in renal phosphate handling.Knockout of nuclear high molecular weight FGF2 isoforms in mice modulates bone and phosphate homeostasis.Regulation of phosphate transport by fibroblast growth factor 23 (FGF23): implications for disorders of phosphate metabolism.A clinician's guide to X-linked hypophosphatemiaKbus/Idr, a mutant mouse strain with skeletal abnormalities and hypophosphatemia: identification as an allele of 'Hyp'.Fibroblast growth factor 23 regulates renal 1,25-dihydroxyvitamin D and phosphate metabolism via the MAP kinase signaling pathway in Hyp miceMolecular characterisation of the Hyp deletion and an improved assay for its detection.Deficiency of Thrombospondin-4 in Mice Does Not Affect Skeletal Growth or Bone Mass Acquisition, but Causes a Transient Reduction of Articular Cartilage ThicknessThe wrickkened pathways of FGF23, MEPE and PHEX.Correction of proximal tubule phosphate transport defect in Hyp mice in vivo and in vitro with indomethacin.Aberrant Phex function in osteoblasts and osteocytes alone underlies murine X-linked hypophosphatemia.Hexa-D-arginine treatment increases 7B2•PC2 activity in hyp-mouse osteoblasts and rescues the HYP phenotype.
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Pex/PEX tissue distribution and evidence for a deletion in the 3' region of the Pex gene in X-linked hypophosphatemic mice
description
article científic
@ca
article scientifique
@fr
articolo scientifico
@it
artigo científico
@pt
bilimsel makale
@tr
scientific article published on March 1997
@en
vedecký článok
@sk
vetenskaplig artikel
@sv
videnskabelig artikel
@da
vědecký článek
@cs
name
Pex/PEX tissue distribution an ...... X-linked hypophosphatemic mice
@en
Pex/PEX tissue distribution an ...... -linked hypophosphatemic mice.
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type
label
Pex/PEX tissue distribution an ...... X-linked hypophosphatemic mice
@en
Pex/PEX tissue distribution an ...... -linked hypophosphatemic mice.
@nl
prefLabel
Pex/PEX tissue distribution an ...... X-linked hypophosphatemic mice
@en
Pex/PEX tissue distribution an ...... -linked hypophosphatemic mice.
@nl
P2093
P2860
P356
P1476
Pex/PEX tissue distribution an ...... X-linked hypophosphatemic mice
@en
P2093
Gauthier C
Goodyer CG
Krishnamurthy G
Soumounou Y
Tenenhouse HS
P2860
P304
P356
10.1172/JCI119276
P407
P50
P577
1997-03-01T00:00:00Z