Murine Animal Models in Osteogenesis Imperfecta: The Quest for Improving the Quality of Life.

ABSTRACT: Osteogenesis imperfecta is a rare genetic disorder characterized by bone fragility, due to alterations in the type I collagen molecule. It is a very heterogeneous disease, both genetically and phenotypically, with a high variability of clinical phenotypes, ranging from mild to severe forms, the most extreme cases being perinatal lethal. There is no curative treatment for OI, and so great efforts are being made in order to develop effective therapies. In these attempts, the in vivo preclinical studies are of paramount importance; therefore, serious analysis is required to choose the right murine OI model able to emulate as closely as possible the disease of the target OI population. In this review, we summarize the features of OI murine models that have been used for preclinical studies unt

SUBMITTER: Alcorta-Sevillano N 

PROVIDER: S-EPMC9820162 | biostudies-literature | 2022 Dec

REPOSITORIES: biostudies-literature