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The dystrophin associated protein complex

The dystrophin associated protein complex
Dystrophin is located on the cytoplasmic face of the plasma membrane of muscle fibers. Arrows indicate the protein components mutated in various muscular dystrophies. Alterations in the dystrophin gene cause Becker and Duchenne muscular dystrophies. Mutations in the sarcoglycan proteins, caveolin-3 and dysferlin, lead to limb girdle muscular dystrophies. The laminin a2-chain is mutated in a subtype of congenital muscular dystrophy without structural brain anomalies, as is a7-integrin.
BMD: Becker muscular dystrophy; CMD: congenital muscular dystrophy; COOH: carboxy-terminal domain; CR: cysteine-rich domain; DMD: Duchenne muscular dystrophy; HH: two helices of the coiled-coil domain; LGMD: limb-girdle muscular dystrophy; MM: Miyoshi myopathy; NH2: amino-terminal domain; Syn: syntrophin-binding domain.
Courtesy of Dr. K. O'Brien and Dr. L. Kunkel, Children's Hospital, Boston, MA.
Graphic 66588 Version 1.0

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