Severe osteogenesis imperfecta in cyclophilin B-deficient mice.

Osteogenesis Imperfecta (OI) is a human syndrome characterized by exquisitely fragile bones due to osteoporosis. The majority of autosomal dominant OI cases result from point or splice site mutations in the type I collagen genes, which are thought to lead to aberrant osteoid within developing bones....

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Bibliographic Details
Main Authors: Jae Won Choi, Shari L Sutor, Lonn Lindquist, Glenda L Evans, Benjamin J Madden, H Robert Bergen, Theresa E Hefferan, Michael J Yaszemski, Richard J Bram
Format: Article
Language:English
Published: Public Library of Science (PLoS) 2009-12-01
Series:PLoS Genetics
Online Access:http://europepmc.org/articles/PMC2777385?pdf=render