Document Detail


Fibrous dysplasia and fibroblast growth factor-23 regulation.
MedLine Citation:
PMID:  23532406     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Fibrous dysplasia (FD) is a skeletal disorder caused by activating mutations in Gsα that result in elevations in cAMP. A feature of FD is elevated blood levels of the bone cell-derived phosphaturic hormone, fibroblast growth factor-23 (FGF23). FGF23 regulates serum phosphorus and active vitamin D levels by action on proximal renal tubule cells. An essential step in the production of biologically active FGF23 is glycosylation by the UDP-N-acetyl-α-D-galactosamine:polypeptide N-acetylgalactosaminyl transferase (ppGalNAc-T3). In the absence of glycosylation, FGF23 is processed into inactive N- and C-terminal proteins by a subtilisin proprotein convertase, probably furin. Normally, most if not all circulating FGF23 is intact. In FD, C-terminal levels are elevated, suggesting altered FGF23 processing. Altered processing in FD is the result of a cAMP-dependent, coordinated decrease in ppGalNAc-T3 and an increase in furin enzyme activity. These findings, and emerging data from other diseases, suggest regulation of FGF23 processing may be a physiologically important process.
Authors:
Alison M Boyce; Nisan Bhattacharyya; Michael T Collins
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Publication Detail:
Type:  Journal Article; Research Support, N.I.H., Intramural; Research Support, Non-U.S. Gov't; Review    
Journal Detail:
Title:  Current osteoporosis reports     Volume:  11     ISSN:  1544-2241     ISO Abbreviation:  Curr Osteoporos Rep     Publication Date:  2013 Jun 
Date Detail:
Created Date:  2013-05-02     Completed Date:  2014-01-07     Revised Date:  2014-06-03    
Medline Journal Info:
Nlm Unique ID:  101176492     Medline TA:  Curr Osteoporos Rep     Country:  United States    
Other Details:
Languages:  eng     Pagination:  65-71     Citation Subset:  IM    
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MeSH Terms
Descriptor/Qualifier:
DNA / genetics*
DNA Mutational Analysis
Fibroblast Growth Factors / biosynthesis,  genetics*
Fibrous Dysplasia of Bone / genetics*,  metabolism
Gene Expression Regulation*
Humans
Mutation*
Grant Support
ID/Acronym/Agency:
Z99 DE999999/DE/NIDCR NIH HHS
Chemical
Reg. No./Substance:
0/fibroblast growth factor 23; 62031-54-3/Fibroblast Growth Factors; 9007-49-2/DNA
Comments/Corrections

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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