Quantitative freeze-fracture studies of membrane changes in chicken muscular dystrophy.

Abstract

Muscular dystrophy induces extensive changes in the patterning of sarcolemmal caveolae of fast-twitch fibers from the chicken posterior latissimus dorsi (PLD) muscle, which in healthy fibers are arranged in striking bands over the myofibrillar I-bands. In dystrophic fibers the caveolae lack this patterned arrangement, and instead are dispersed over the entire sarcolemma, are irregular in shape, and are more numerous in older birds. Quantitative analysis of these differences provides three independent numerical indices of the dystrophic state and suggests that constraints responsible for normal patterning are lost in diseased fibers. These observations support theories that defects of the muscle plasma membrane are important for dystrophic pathogenesis. In contrast, the sarcolemma of slow tonic fibers from anterior latissimus dorsi (ALD) and metapatagialis latissimus dorsi (MLD) muscles have randomly dispersed caveolae whose appearance and distribution are unaffected by the disease.