Enhanced Wnt signaling improves bone mass and strength, but not brittleness, in the Col1a1(+/mov13) mouse model of type I Osteogenesis Imperfecta.
作者： Christina M. Jacobsen , Marissa A. Schwartz , Heather J. Roberts , Kyung-Eun Lim , Lyudmila Spevak
DOI: 10.1016/J.BONE.2016.06.005
关键词:
摘要: Osteogenesis Imperfecta (OI) comprises a group of genetic skeletal fragility disorders. The mildest form OI, type I, is frequently caused by haploinsufficiency mutations in COL1A1, the gene encoding α1(I) chain 1 collagen. Children with OI I have 95-fold higher fracture rate compared to unaffected children. Therapies for pediatric population are limited anti-catabolic agents. In adults osteoporosis, anabolic therapies that enhance Wnt signaling bone improve mass, and ongoing clinical trials determining if these also reduce risk. We performed proof-of-principle experiment mice determine whether enhancing could benefit children I. crossed mouse model (Col1a1(+/Mov13)) high mass (HBM) (Lrp5(+/p.A214V)) has increased strength from enhanced signaling. Offspring inherited HBM alleles had than allele alone. However, OI+HBM still bones lower ductility wild-type mice. conclude does not make normal, but properties Therefore, agents likely 1.
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