Proteins of muscle subcellular fractions in Duchenne progressive muscular dystrophy stained with "stains-all" cationic carbocyanine dye and with Coomassie Blue.
The protein compositions of subcellular fractions of muscle obtained from 17 Duchenne dystrophy patients, 15 disease controls (10 different primary myopathies, 5 spinal muscular atrophy patients), and 10 normals were examined by polyacrylamide gel electrophoresis. The gels were stained with Coomassie Brilliant Blue and with "Stains-all," which stains calcium-binding proteins, sialic acid-rich glycoproteins, and phosphoproteins. In muscle membrane fractions of Duchenne dystrophy patients there was a marked reduction in the concentrations of calsequestrin and a 39 kDa protein that stained blue with "Stains-all." There were changes in the proteins of all subcellular fractions of Duchenne's patients; some of these changes appear to be specific for Duchenne dystrophy (DD). There was no apparent correlation between the protein changes observed on acrylamide gels and the age of the patients, the duration of the disease, the degree of disability, or activity of creatine kinase. A decreased level of calsequestrin in DD sarcoplasmic reticulum may contribute to an increased level of free calcium seen in muscle from these patients.
Department of Neurology, University of Wisconsin, Madison 53792.
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Niebroj-Dobosz
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Kornguth
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Schutta
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Siegel
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Hausmanowa-Petrusewicz
D002135:Calcium-Binding Proteins / Q000378:metabolism; D002155:Calsequestrin / Q000378:metabolism; D002232:Carbocyanines; D002648:Child; D002675:Child, Preschool; D004591:Electrophoresis, Polyacrylamide Gel; D005260:Female; D006801:Humans; D008297:Male; D009124:Muscle Proteins / Q000378:metabolism*; D009132:Muscles / Q000378:metabolism; D009136:Muscular Dystrophies / Q000378:metabolism*; D011804:Quinolines; D012394:Rosaniline Dyes; D012795:Sialoglycoproteins / Q000378:metabolism; D013347:Subcellular Fractions / Q000378:metabolism
D002135:Calcium-Binding Proteins; D002155:Calsequestrin; D002232:Carbocyanines; D009124:Muscle Proteins; D011804:Quinolines; D012394:Rosaniline Dyes; D012795:Sialoglycoproteins; C012040:stains-all; C004692:coomassie Brilliant Blue