Structural proteins of the opaque muscle fibers in Duchenne muscular dystrophy.
We studied the structural proteins of the "opaque fibers" (OFs) in muscle biopsies from 12 patients, aged 4 to 24 years, with Duchenne muscular dystrophy. The electrophoretic patterns of myofibrillar proteins indicated three groups of opaque fibers: the first (OF a) had an almost normal electrophoretogram, the second (OF b1) showed a clear decrease of 55,000 dalton protein (desmin), and the third (OF b2) exhibited a great reduction in alpha-actinin and desmin. The data suggest that some OFs are pathologic fibers in the degenerative process of Duchenne dystrophy.
D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D006801:Humans; D009124:Muscle Proteins / Q000032:analysis*; D009136:Muscular Dystrophies / Q000378:metabolism*
D009124:Muscle Proteins