Electrophoretic studies of muscle proteins in Duchenne muscular dystrophy and other neuromuscular disorders--with special reference to the change of dystrophin.
We studied total SDS-solubilized muscle proteins (TMP) of Duchenne muscular dystrophy (DMD) and other neuromuscular disorders, with special attention to the change of dystrophin suspected of being the product of DMD locus. SDS gel electrophoresis of DMD patients showed an absence of band 5 and an extreme faintness of band 2 with a decrease of band 4', 5', and 5''. Immunoblot analysis, using anti-dystrophin antibodies (anti-30 kd and anti-60 kd polyclonals), showed an absence of dystrophin in all 6 DMD cases. In other neuromuscular disorders, there was no change of TMP, and dystrophin was clearly detectable. To elucidate the degenerative mechanism of DMD muscle, further studies, including the problem of clarifying the physiological role of dystrophin, are necessary.
Uchino
M
M
Department of Internal Medicine, Kumamoto University Medical School, Japan.
Araki
S
S
Miike
T
T
D000328:Adult; D000368:Aged; D002648:Child; D002675:Child, Preschool; D064211:Connectin; D003285:Contractile Proteins / Q000378:metabolism; D016189:Dystrophin; D004591:Electrophoresis, Polyacrylamide Gel; D005260:Female; D064448:Filamins; D006801:Humans; D007223:Infant; D008297:Male; D008565:Membrane Proteins / Q000378:metabolism; D008840:Microfilament Proteins / Q000378:metabolism; D008875:Middle Aged; D009124:Muscle Proteins / Q000172:deficiency* / Q000378:metabolism; D009136:Muscular Dystrophies / Q000378:metabolism*; D009468:Neuromuscular Diseases / Q000378:metabolism*; D011494:Protein Kinases
D064211:Connectin; D003285:Contractile Proteins; D016189:Dystrophin; D064448:Filamins; D008565:Membrane Proteins; D008840:Microfilament Proteins; D009124:Muscle Proteins; C577285:TTN protein, human; C035620:nebulin; D011494:Protein Kinases