Studies of selected enzymes of phospholipid metabolism in the dystrophic human muscle.
Proceeding from deviations of the phospholipid pattern in the muscle in progressive muscular dystrophy, the activities of a number of enzymes of phospholipid metabolism in the normal and dystrophic human muscle were measured. Twelve cases of the Duchenne type and four of the Becker-Kiener type were studied. The activities of CDP-choline:diglyceride-P-cholinetransferase, of CDP-choline:ceramide-P-cholinetransferase and of phosphatidylcholine degradation were determined in normal and dystrophic human muscle. A significant difference in enzyme activities between normal and dystrophic muscle could not be established. The activities of a phosphatidylethanolaminemethyltransferase and a transfer of P-choline directly from phosphatidylcholine to sphingomyelin could be excluded for normal human muscle. The significance of these results for the integration of phospholipid findings in ideas about pathogenesis of progressive muscular dystrophy is discussed.
D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D003566:Cytidine Diphosphate Choline / Q000378:metabolism; D002798:Diacylglycerol Cholinephosphotransferase / Q000378:metabolism; D006801:Humans; D008297:Male; D008780:Methyltransferases / Q000378:metabolism; D009132:Muscles / Q000201:enzymology* / Q000378:metabolism; D009136:Muscular Dystrophies / Q000201:enzymology* / Q000235:genetics; D050918:Phosphatidylethanolamine N-Methyltransferase; D010741:Phospholipases A / Q000378:metabolism; D010743:Phospholipids / Q000378:metabolism*; D010770:Phosphotransferases / Q000378:metabolism; D013109:Sphingomyelins / Q000096:biosynthesis / Q000378:metabolism; D017855:Transferases (Other Substituted Phosphate Groups)
D010743:Phospholipids; D013109:Sphingomyelins; D003566:Cytidine Diphosphate Choline; D008780:Methyltransferases; D050918:Phosphatidylethanolamine N-Methyltransferase; D010770:Phosphotransferases; D017855:Transferases (Other Substituted Phosphate Groups); D002798:Diacylglycerol Cholinephosphotransferase; C019863:ceramide cholinephosphotransferase; D010741:Phospholipases A