Three mouse models of muscular dystrophy: the natural history of strength and fatigue in dystrophin-, dystrophin/utrophin-, and laminin α2-deficient mice
AM Connolly, RM Keeling, S Mehta, A Pestronk… - Neuromuscular …, 2001 - Elsevier
To optimize and evaluate treatments for muscular dystrophy, it is important to know the
natural history of the disease in the absence of therapeutic intervention. Here we
characterized disease progression of three mutant mouse strains of muscular dystrophy:
mdx mice, which lack dystrophin; mdx: utrn−/− mice, which also lack utrophin; and dy/dy
mice, which are deficient in laminin α2. Normal mice show a marked increase in forelimb
strength over the first 10 weeks of life and little fatigue (< 5%) over five consecutive strength …
natural history of the disease in the absence of therapeutic intervention. Here we
characterized disease progression of three mutant mouse strains of muscular dystrophy:
mdx mice, which lack dystrophin; mdx: utrn−/− mice, which also lack utrophin; and dy/dy
mice, which are deficient in laminin α2. Normal mice show a marked increase in forelimb
strength over the first 10 weeks of life and little fatigue (< 5%) over five consecutive strength …