Natural disease history of the D2-mdx mouse model for Duchenne muscular dystrophy.

ABSTRACT: The C57BL/10ScSn-Dmd^mdx/J (BL10-mdx) mouse has been the most commonly used model for Duchenne muscular dystrophy (DMD) for decades. Their muscle dysfunction and pathology is, however, less severe than in patients with DMD, which complicates preclinical studies. Recent discoveries indicate that disease severity is exacerbated when muscular dystrophy mouse models are generated on a DBA2/J genetic background. Knowledge on the natural history of animal models is pivotal for high-quality preclinical testing. However, for BL10-mdx mice on a DBA2/J background (D2-mdx), limited data are available. We addressed this gap in the natural history knowledge. First, we compared histopathological aspects in skeletal muscles of young D2-mdx, BL10-mdx, an

SUBMITTER: van Putten M 

PROVIDER: S-EPMC6593893 | biostudies-literature | 2019 Jul

REPOSITORIES: biostudies-literature