Myotonic muscular dystrophy: abnormal temperature response of membrane phosphorylation in erythrocyte membranes.
The activities of the membrane-bound protein kinases of the human erythrocytes membrane that phosphorylate spectrin, band-3 protein, and phospholipids were compared in patients with myotonic muscular dystrophy and normal age- and sex-matched controls. These activities tended to be lower in the patients, but the differences were not statistically significant. In contrast, the temperature responses (the increase in activity in response to an increase in temperature from 30 degrees C to 37 degrees C) of the spectrin and band-3 protein kinase activities were significantly lower in the patients. Although they do not eliminate an alteration of one of the substrates, these results are consistent with the proposal that differences in erythrocytes from myotonic muscular dystrophy (MyD) patients are due to a membrane lipid change. Cholesterol is unlikely to be the altered lipid, as no difference in membrane cholesterol content was found.
D000255:Adenosine Triphosphate / Q000097:blood; D000328:Adult; D000367:Age Factors; D002784:Cholesterol / Q000097:blood; D004910:Erythrocyte Membrane / Q000201:enzymology*; D004912:Erythrocytes / Q000201:enzymology*; D005260:Female; D006801:Humans; D008297:Male; D009136:Muscular Dystrophies / Q000201:enzymology*; D010743:Phospholipids / Q000097:blood; D010766:Phosphorylation; D010770:Phosphotransferases / Q000097:blood*; D011494:Protein Kinases / Q000097:blood; D012737:Sex Factors; D013049:Spectrin / Q000378:metabolism; D013696:Temperature
D010743:Phospholipids; D013049:Spectrin; D000255:Adenosine Triphosphate; D002784:Cholesterol; D010770:Phosphotransferases; D011494:Protein Kinases