Demonstration of Defect of Creatinephosphokinase in Muscle of Progressive Muscular Dystrophy

Enzymological studies on serum, erythrocytes and muscle were carried out in two patients with progressive muscular dystrophy. The activities of serum aldolase, transaminase and LD were remarkably elevated. The activities of the erythrocyte enzymes such as aldolase, LD and G6PD were within normal limits. The contents of ATP and PN in erythrocytes showed no abnormal values. The glucose uptake test by the erythrocytes revealed normal findings. Analytical results of the muscle revealed that the activity of creatinephosphokinase and the contents of creatine, creatinine and creatine phosphate were definitely lower, while the content of glycogen and ATP and the activities of aldolase, transaminase and LD did not differ much from those of controls in the values per 1.0g of nitrogen in the muscle.