Ontology highlight
ABSTRACT:
SUBMITTER: Carriero A
PROVIDER: S-EPMC4477967 | biostudies-literature | 2014 Jun
REPOSITORIES: biostudies-literature
Carriero A A Zimmermann E A EA Paluszny A A Tang S Y SY Bale H H Busse B B Alliston T T Kazakia G G Ritchie R O RO Shefelbine S J SJ
Journal of bone and mineral research : the official journal of the American Society for Bone and Mineral Research 20140601 6
The multiscale hierarchical structure of bone is naturally optimized to resist fractures. In osteogenesis imperfecta, or brittle bone disease, genetic mutations affect the quality and/or quantity of collagen, dramatically increasing bone fracture risk. Here we reveal how the collagen defect results in bone fragility in a mouse model of osteogenesis imperfecta (oim), which has homotrimeric α1(I) collagen. At the molecular level, we attribute the loss in toughness to a decrease in the stabilizing ...[more]