FGF-23 in fibrous dysplasia of bone and its relationship to renal phosphate wasting

Mara Riminucci, Michael T. Collins, Neal S Fedarko, Natasha Cherman, Alessandro Corsi, Kenneth E. White, Steven Waguespack, Anurag Gupta, Tamara Hannon, Michael J. Econs, Paolo Bianco, Pamela Gehron Robey

Research output: Contribution to journalArticle

Abstract

FGF-23, a novel member of the FGF family, is the product of the gene mutated in autosomal dominant hypophosphatemic rickets (ADHR). FGF-23 has been proposed as a circulating factor causing renal phosphate wasting not only in ADHR (as a result of inadequate degradation), but also in tumor-induced osteomalacia (as a result of excess synthesis by tumor cells). Renal phosphate wasting occurs in approximately 50% of patients with McCune-Albright syndrome (MAS) and fibrous dysplasia of bone (FD), which result from postzygotic mutations of the GNAS1 gene. We found that FGF-23 is produced by normal and FD osteoprogenitors and bone-forming cells in vivo and in vitro. In situ hybridization analysis of FGF-23 mRNA expression identified "fibrous" cells, osteogenic cells, and cells associated with microvascular walls as specific cellular sources of FGF-23 in FD. Serum levels of FGF-23 were increased in FD/MAS patients compared with normal age-matched controls and significantly higher in FD/MAS patients with renal phosphate wasting compared with those without, and correlated with disease burden bone turnover markers commonly used to assess disease activity. Production of FGF-23 by FD tissue may play an important role in the renal phosphate-wasting syndrome associated with FD/MAS.

Original languageEnglish (US)
Pages (from-to)683-692
Number of pages10
JournalJournal of Clinical Investigation
Volume112
Issue number5
DOIs
StatePublished - Jan 1 2003

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Fibrous Dysplasia of Bone
Polyostotic Fibrous Dysplasia
Phosphates
Kidney
Wasting Syndrome
Bone and Bones
Bone Remodeling
Genes
In Situ Hybridization
Messenger RNA
Mutation

ASJC Scopus subject areas

  • Medicine(all)

Cite this

Riminucci, M., Collins, M. T., Fedarko, N. S., Cherman, N., Corsi, A., White, K. E., ... Robey, P. G. (2003). FGF-23 in fibrous dysplasia of bone and its relationship to renal phosphate wasting. Journal of Clinical Investigation, 112(5), 683-692. https://doi.org/10.1172/JCI18399

FGF-23 in fibrous dysplasia of bone and its relationship to renal phosphate wasting. / Riminucci, Mara; Collins, Michael T.; Fedarko, Neal S; Cherman, Natasha; Corsi, Alessandro; White, Kenneth E.; Waguespack, Steven; Gupta, Anurag; Hannon, Tamara; Econs, Michael J.; Bianco, Paolo; Robey, Pamela Gehron.

In: Journal of Clinical Investigation, Vol. 112, No. 5, 01.01.2003, p. 683-692.

Research output: Contribution to journalArticle

Riminucci, M, Collins, MT, Fedarko, NS, Cherman, N, Corsi, A, White, KE, Waguespack, S, Gupta, A, Hannon, T, Econs, MJ, Bianco, P & Robey, PG 2003, 'FGF-23 in fibrous dysplasia of bone and its relationship to renal phosphate wasting', Journal of Clinical Investigation, vol. 112, no. 5, pp. 683-692. https://doi.org/10.1172/JCI18399
Riminucci, Mara ; Collins, Michael T. ; Fedarko, Neal S ; Cherman, Natasha ; Corsi, Alessandro ; White, Kenneth E. ; Waguespack, Steven ; Gupta, Anurag ; Hannon, Tamara ; Econs, Michael J. ; Bianco, Paolo ; Robey, Pamela Gehron. / FGF-23 in fibrous dysplasia of bone and its relationship to renal phosphate wasting. In: Journal of Clinical Investigation. 2003 ; Vol. 112, No. 5. pp. 683-692.
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