Recombinant Mouse Metalloendopeptidase homolog PEX (Phex)

1. the phex mutation may create the lower set point for extracellular phosphate concentrations in a murine model of X-linked hypophosphatemia PMID: 23700148
2. demonstrates a previously unknown impact of retinol on the cell-autonomous mineralization defect of Phex-deficient osteoblasts PMID: 23266491
3. results identify full-length OPN as novel, physiologically relevant substrates for PHEX, suggesting accumulation of mineralization-inhibiting OPN fragments may contribute to the mineralization defect seen in the osteomalacic bone characteristic of XLH/HYP PMID: 22991293
4. Mutation in Phex gene predisposes BALB/c-Phex(Hyp-Duk)/Y mice to otitis media. PMID: 23028440
5. These data suggest that Phex mutations alter the responsiveness of bone cells to extracellular phosphate concentrations and may create a lower set point for "normal" phosphate levels. PMID: 22006791
6. PHEX and DMP1 control a common pathway regulating bone mineralization and FGF23 production, the latter involving activation of the FGFR signaling in osteocytes. PMID: 21507898
7. ASARM peptides likely play a bone PHEX-dependent role in renal phosphate regulation and FGF23 expression. PMID: 21177780
8. Cooperative role of NF-{kappa}B and poly(ADP-ribose) polymerase 1 (PARP-1) in the TNF-induced inhibition of PHEX expression in osteoblasts. PMID: 20817730
9. OPN ASARM inhibits mineralization by binding to hydroxyapatite in a phosphorylation-dependent manner and can be cleaved by PHEX. PMID: 19775205
10. Preproenkephalin deletion in Hyp mice (Phex-deficient) partially rescues their bone mineralization defect. PMID: 20204609
11. Phex protein is expressed in osteoblasts and osteocytes during the embryonic and postnatal periods PMID: 11811562
12. Glucocorticoid regulation of the murine PHEX gene. PMID: 12110521
13. MEPE may be involved in the pathogenesis defective mineralization due to Phex deficiency in X-linked hypophosphatemia (XLH) and the Hyp-mouse. PMID: 12220505
14. mutagen-induced point mutation in phex causes exon skipping, x-linked hypophosphatemia, and rickets PMID: 12414538
15. findings suggest that hypophosphatemia bone and teeth abnormalities partially correct after phosphate-regulating gene with homologies to endopeptidases on the X chromosome (PHEX) gene transfer but additional factors of PHEX expression are likely critical PMID: 12456809
16. rickets and osteomalacia are not the only causes of decreased vertebral growth in Hyp mice and Phex protein may influence bone growth and mineralization by distinct pathways PMID: 14751570
17. Phex may control mineralization and removal of hypertrophic chondrocytes and cartilage matrix in growth plate by regulating the synthesis and deposition of certain bone matrix proteins and proteases such as matrix metalloproteinase 9 PMID: 15050894
18. downregulation of Phex expression by PTH in vitro is associated with inhibition of matrix mineralization, consistent with a role for Phex in bone mineralization PMID: 16084134
19. TNF-alpha-mediated reduction in Phex protein is at least in part responsible for inhibition of osteoblast mineralization, and the described mechanism may contribute to the abnormal bone metabolism associated with IBD. PMID: 16890604
20. loss of function is related to defect of type II sodium-dependent phosphate co-transporter in [developing] teeth PMID: 17251516
21. A new mutation was mapped to X-chromosome between 65.4 cM and 66.6 cM, where Phex gene resides. Sequence analysis revealed a unique T-to-C transition mutation resulting in Phe-to-Ser substitution at amino acid 80 of PHEX protein. PMID: 17710565
22. PHEX functions in bone to coordinate bone mineralization and systemic phosphate homeostasis by directly regulating the mineralization process and producing FGF23. PMID: 17848631
23. findings suggest that loss of Phex function is related to overexpression of FGF23 in teeth, which is an intrinsic defect of Hyp mouse teeth. PMID: 17942069
24. Phex Hyp-Duk/Y mice develop endolhympattic hydrops without evidence of endolymphatic duct obstruction PMID: 18289812
25. PHEX rescued the inhibition of osteoblast culture mineralization by pASARM, and mass spectrometry of cleaved peptides obtained after pASARM-PHEX incubations identified pASARM as a substrate for PHEX PMID: 18597632
26. The PHEX transgene corrects mineralization defects in 9-month-old hypophosphatemic mice. PMID: 19082853
27. Our in vivo studies provide genetic evidence for a pathological role of increased FGF23 activities in regulating abnormal phosphate homeostasis in Hyp (PHEX-deficient) mice. PMID: 19584304

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KEGG: mmu:18675

STRING: 10090.ENSMUSP00000078863

UniGene: Mm.2529