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Although increased serum fibroblast growth factor 23 (FGF-23) underlies the HYP phenotype, the mechanism(s) by which PHEX mutations inhibit FGF-23 degradation and\/or enhance production remains unknown. Here we show that treatment of wild-type mice with the proprotein convertase (PC) inhibitor, decanoyl-Arg-Val-Lys-Arg-chloromethyl ketone (Dec), increases serum FGF-23 and produces the HYP phenotype. Because PC2 is uniquely colocalized with PHEX in osteoblasts\/bone, we examined if PC2 regulates PHEX-dependent FGF-23 cleavage and production. Transfection of murine osteoblasts with PC2 and its chaperone protein 7B2 cleaved FGF-23, whereas Signe1 (7B2) RNA interference (RNAi) transfection, which limited 7B2 protein production, decreased FGF-23 degradation and increased Fgf-23 mRNA and protein. The mechanism by which decreased 7B2\u2022PC2 activity influences Fgf-23 mRNA was linked to reduced conversion of the precursor to bone morphogenetic protein 1 (proBMP1) to active BMP1, which resulted in limited cleavage of dentin matrix acidic phosphoprotein 1 (DMP1), and consequent increased Fgf-23 mRNA. The significance of decreased 7B2\u2022PC2 activity in XLH was confirmed by studies of hyp-mouse bone, which revealed significantly decreased Sgne1 (7B2) mRNA and 7B2 protein, and limited cleavage of proPC2 to active PC2. The expected downstream effects of these changes included decreased FGF-23 cleavage and increased FGF-23 synthesis, secondary to decreased BMP1-mediated degradation of DMP1. Subsequent Hexa-D-Arginine treatment of hyp-mice enhanced bone 7B2\u2022PC2 activity, normalized FGF-23 degradation and production, and rescued the HYP phenotype. These data suggest that decreased PHEX-dependent 7B2\u2022PC2 activity is central to the pathogenesis of XLH. \u00a9 2013 American Society for Bone and Mineral Research<\/jats:p>","DOI":"10.1002\/jbmr.1738","type":"journal-article","created":{"date-parts":[[2012,8,9]],"date-time":"2012-08-09T19:21:16Z","timestamp":1344540076000},"page":"56-72","source":"Crossref","is-referenced-by-count":57,"title":["Hexa-D-arginine treatment increases 7B2\u2022PC2 activity in <i>hyp<\/i>-mouse osteoblasts and rescues the <i>HYP<\/i> phenotype"],"prefix":"10.1093","volume":"28","author":[{"given":"Baozhi","family":"Yuan","sequence":"first","affiliation":[{"name":"Department of Medicine, University of Wisconsin-Madison and Geriatric Research and Education Center, William S. Middleton Memorial Veterans Hospital, Madison, WI, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jian Q","family":"Feng","sequence":"additional","affiliation":[{"name":"Department of Biomedical Sciences, Baylor College of Dentistry, Texas A&M Health Science Center, Dallas, TX, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stephen","family":"Bowman","sequence":"additional","affiliation":[{"name":"Department of Medicine, University of Wisconsin-Madison and Geriatric Research and Education Center, William S. Middleton Memorial Veterans Hospital, Madison, WI, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ying","family":"Liu","sequence":"additional","affiliation":[{"name":"Department of Biomedical Sciences, Baylor College of Dentistry, Texas A&M Health Science Center, Dallas, TX, USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Robert D","family":"Blank","sequence":"additional","affiliation":[{"name":"Department of Medicine, University of Wisconsin-Madison and Geriatric Research and Education Center, William S. 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