Progressive limb and girdle muscle atrophy leading to loss of ambulation is a hallmark of
dysferlinopathies, which include limb-girdle muscular dystrophy type 2B and Miyoshi
myopathy. However, animal models fail to fully reproduce the disease severity observed in
humans, with dysferlin-null (Dysf−/−) mice exhibiting minor muscle damage and weakness
without dramatic ambulatory dysfunction. As we have previously reported significant Dysf
expression in blood vessels, we investigated the role of vascular function in development of …