Immunoblot analysis of sarcoplasmic calcium binding proteins in Duchenne muscular dystrophy.
The Western blotting technique was used to detect parvalbumin and S-100 protein in muscles from 10 Duchenne muscular dystrophy (DD) patients, 13 patients with other muscle diseases and 5 age-matched healthy subjects. DD muscles were found to contain decreased amounts of parvalbumin and the S-100 protein. The parvalbumin level did not relate to the age of the patients and the stage of the disease. The S-100 protein decreased progressively with the age of the patients. In a very advanced DD case the S-100 protein was present in trace amounts. In other primary myopathies, including Becker dystrophy, and neurogenic muscular atrophy both parvalbumin and S-100 protein levels were similar to that observed in healthy subjects. The decrease in the amount of both calcium binding proteins may contribute to the elevation of free intracellular Ca2+ level in the sarcoplasm of dystrophic muscle and would result in abnormalities in processes regulated by these proteins. The mechanism(s) responsible for the decrease of parvalbumin and S-100 protein in DD muscles are discussed.
Niebrój-Dobosz
I
I
Department of Neurology, Medical Academy, Warsaw, Poland.
Lukasiuk
M
M
D000293:Adolescent; D000328:Adult; D015153:Blotting, Western; D002135:Calcium-Binding Proteins / Q000032:analysis*; D016022:Case-Control Studies; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D008297:Male; D009135:Muscular Diseases / Q000378:metabolism; D009136:Muscular Dystrophies / Q000378:metabolism*; D010320:Parvalbumins / Q000032:analysis; D009418:S100 Proteins / Q000032:analysis; D012519:Sarcoplasmic Reticulum / Q000737:chemistry*
D002135:Calcium-Binding Proteins; D010320:Parvalbumins; D009418:S100 Proteins