Exploration of the skeletal phenotype of the Col1a1 ^+/Mov13 mouse model for haploinsufficient osteogenesis imperfecta type 1.

ABSTRACT:

Introduction

Osteogenesis Imperfecta is a rare genetic connective tissue disorder, characterized by skeletal dysplasia and fragile bones. Currently only two mouse models have been reported for haploinsufficient (HI) mild Osteogenesis Imperfecta (OI); the Col1a1 ^+/Mov13 (Mov13) and the Col1a1 ^+/-365 mouse model. The Mov13 mice were created by random insertion of the Mouse Moloney leukemia virus in the first intron of the Col1a1 gene, preventing the initiation of transcription. Since the development of the Mov13 mice almost four decades ago and its basic phenotypic characterization in the 90s, there have not been many further studies. We aimed to extensively characterize the Mov13 mouse model in order to critically evaluate its possible use

SUBMITTER: Claeys L 

PROVIDER: S-EPMC10031054 | biostudies-literature | 2023

REPOSITORIES: biostudies-literature