Effect of sclerostin inactivation in a mouse model of severe dominant osteogenesis imperfecta.

ABSTRACT: Osteogenesis imperfecta (OI) is a rare bone disease that is associated with fractures and low bone mass. Sclerostin inhibition is being evaluated as a potential approach to increase bone mass in OI. We had previously found that in Col1a1^Jrt/+ mice, a model of severe OI, treatment with an anti-sclerostin antibody had a minor effect on the skeletal phenotype. In the present study, we assessed the effect of genetic sclerostin inactivation in the Col1a1^Jrt/+ mouse. We crossed Col1a1^Jrt/+ mice with Sost knockout mice to generate Sost-deficient Col1a1^Jrt/+ mice and assessed differences between Col1a1^Jrt/+ mice with homozygous Sost deficiency and Col1a1^Jrt/+ mice with heterozygous Sost deficiency. We found that Col1a1^Jrt/+

SUBMITTER: Marulanda J 

PROVIDER: S-EPMC10043013 | biostudies-literature | 2023 Mar

REPOSITORIES: biostudies-literature