Membrane Abnormalities of Skeletal Muscle and Erythrocyte in Duchenne Muscular Dystrophy

概要

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Pathogenesis of Duchenne muscular dystrophy (DMD) has recently been attributed to a plasma membrane defect of muscle. Several studies on erythrocyte membranes from the patients suggest that DMD might be a generalized membrane disorder. However, other reports on the erythrocytes are against this hypothesis. This investigation was made in an attempt to clarify whether DMD is a systemic membrane disease or not, analysing the adenylate cyclase activities (AC) and phosphatidyl inositol metabolism of erythrocyte membranes. 1) Basal AC of erythrocyte membranes from mothers of DMD were higher than controls (p<0.01), but no significant correlation was obtained among the basal AC, serum creatine kinase activities and carrier states. 2) 3H-inositol was incorporated mainly into diphosphoinositide in human erythrocyte membranes. Its incorporation was markedly inhibited by Ca++and facilitated by washing the membranes in the presence of EDTA. 3) No difference was observed in the 3H-inositol incorporation between DMD and controls in the EDTA lysed membranes. 4) Neither AC were different between DMD and controls in the EDTA lysed membranes. Conditions for the preparation of erythrocyte membranes and age matched study are emphasized to be critical and important. It was not concluded that DMD might be a systemic membrane disorder from the above data. Possible factors influencing the membrane enzymes were also discussed.
Japan Society of Clinical Chemistryの論文