Kunze D, Rüstow B, Olthoff D
Clin Chim Acta. 1980 Dec 8;108(2):211-8. doi: 10.1016/0009-8981(80)90007-8.
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.
基于进行性肌营养不良患者肌肉中磷脂模式的偏差，对正常人和患病人肌肉中多种磷脂代谢酶的活性进行了测定。研究了12例杜兴型和4例贝克 - 基纳型患者。测定了正常人和患病人肌肉中CDP - 胆碱：甘油二酯 - P - 胆碱转移酶、CDP - 胆碱：神经酰胺 - P - 胆碱转移酶以及磷脂酰胆碱降解的活性。正常肌肉和患病人肌肉之间的酶活性未发现显著差异。正常人肌肉中可排除磷脂酰乙醇胺甲基转移酶的活性以及P - 胆碱直接从磷脂酰胆碱转移到鞘磷脂的情况。讨论了这些结果对于将磷脂研究结果整合到进行性肌营养不良发病机制观点中的意义。
