Abstract
Background: Dysferlin is a sarcolemmal protein that is defective in Miyoshi myopathy (MM) and limb girdle muscular dystrophy type 2B (LGMD2B). Affixin (β-parvin) is an integrin-linked kinase- binding protein that is involved in the linkage between integrin and actin cytoskeleton. We previously reported that affixin is a dysferlin binding protein that colocalizes with dysferlin at the sarcolemma of normal human skeletal muscle. The immunoreactivity of affixin was reduced in sarcolemma of MM and LGMD2B muscles.
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