Document Detail

Fibrous dysplasia and fibroblast growth factor-23 regulation.
MedLine Citation:
PMID:  23532406     Owner:  NLM     Status:  MEDLINE    
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.
Alison M Boyce; Nisan Bhattacharyya; Michael T Collins
Related Documents :
23975336 - Characterization and modulation of glucose uptake in a human blood-brain barrier model.
21321996 - Adiponectin increases mmp-3 expression in human chondrocytes through adipor1 signaling ...
23272106 - The essential phosphoinositide kinase mss-4 is required for polar hyphal morphogenesis,...
18455986 - Allosteric regulation of histidine kinases by their cognate response regulator determin...
23276636 - Modulation of mapk and akt signaling pathways in proximal segment of injured sciatic ne...
9482736 - A new rac target posh is an sh3-containing scaffold protein involved in the jnk and nf-...
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    
Export Citation:
APA/MLA Format     Download EndNote     Download BibTex
MeSH Terms
DNA / genetics*
DNA Mutational Analysis
Fibroblast Growth Factors / biosynthesis,  genetics*
Fibrous Dysplasia of Bone / genetics*,  metabolism
Gene Expression Regulation*
Grant Support
Reg. No./Substance:
0/fibroblast growth factor 23; 62031-54-3/Fibroblast Growth Factors; 9007-49-2/DNA

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

Previous Document:  Biophysical Regulation of Stem Cell Differentiation.
Next Document:  Transgene delivery via intracellular electroporetic nanoinjection.