Document Detail

A mouse model for human osteogenesis imperfecta type VI.
MedLine Citation:
PMID:  23413146     Owner:  NLM     Status:  MEDLINE    
Osteogenesis imperfecta type VI (OI type VI) has recently be linked to a mutation in the SERPINF1 gene, which encodes pigment epithelium-derived factor (PEDF), a ubiquitously expressed protein originally described for its neurotrophic and antiangiogenic properties. In this study, we characterized the skeletal phenotype of a mouse with targeted disruption of Pedf. In normal mouse bone, Pedf was localized to osteoblasts and osteocytes. Micro-computed tomography (µCT) and quantitative bone histomorphometry in femurs of mature Pedf null mutants revealed reduced trabecular bone volume and the accumulation of unmineralized bone matrix. Fourier transform infrared microscopy (FTIR) indicated an increased mineral:matrix ratio in mutant bones, which were more brittle than controls. In vitro, osteoblasts from Pedf null mice exhibited enhanced mineral deposition as assessed by Alizarin Red staining and an increased mineral:matrix determined by FTIR analysis of calcified nodules. The findings in this mouse model mimic the principal structural and biochemical features of bone observed in humans with OI type VI and consequently provide a useful model with which to further investigate the role of PEDF in this bone disorder.
Rosalind Bogan; Ryan C Riddle; Zhu Li; Sarvesh Kumar; Anjali Nandal; Marie-Claude Faugere; Adele Boskey; Susan E Crawford; Thomas L Clemens
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Publication Detail:
Type:  Journal Article; Research Support, N.I.H., Extramural; Research Support, U.S. Gov't, Non-P.H.S.    
Journal Detail:
Title:  Journal of bone and mineral research : the official journal of the American Society for Bone and Mineral Research     Volume:  28     ISSN:  1523-4681     ISO Abbreviation:  J. Bone Miner. Res.     Publication Date:  2013 Jul 
Date Detail:
Created Date:  2013-06-19     Completed Date:  2013-11-04     Revised Date:  2014-07-02    
Medline Journal Info:
Nlm Unique ID:  8610640     Medline TA:  J Bone Miner Res     Country:  United States    
Other Details:
Languages:  eng     Pagination:  1531-6     Citation Subset:  IM    
Copyright Information:
Copyright © 2013 American Society for Bone and Mineral Research.
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MeSH Terms
Bone Density / genetics*
Disease Models, Animal*
Eye Proteins / genetics*,  metabolism
Femur* / metabolism,  radiography
Mice, Mutant Strains
Nerve Growth Factors / genetics*,  metabolism
Osteocytes* / metabolism,  pathology
Osteogenesis Imperfecta* / genetics,  metabolism,  radiography
Serpins / genetics*,  metabolism
Grant Support
Reg. No./Substance:
0/Eye Proteins; 0/Nerve Growth Factors; 0/Serpins; 0/pigment epithelium-derived factor
Comment In:
J Bone Miner Res. 2013 Jul;28(7):1519-22   [PMID:  23696068 ]

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