Why the study?
Common DMD mouse models have less severe pathology than human patients, and limited natural history data were available for the more severely affected D2-mdx mouse model.
Population
Young D2-mdx, BL10-mdx, and wild-type mice, plus older D2-mdx mice
Comparison
D2-mdx vs BL10-mdx vs wild-type mice, and male vs female and 10- vs 34-wk-old D2-mdx mice
Design
Preclinical natural disease history animal study
Follow-up
34 weeks
Authors
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Supports D2-mdx as improved DMD model for cardiac pathology; leaves translation to patients open.
The D2-mdx mouse model exhibits more severe skeletal muscle pathology than the traditional BL10-mdx model and progressive heart pathology, providing a more representative model for preclinical Duchenne muscular dystrophy studies.
Putten et al. (2019) studied this question.
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