Phosphorylase activity and glycogen, glucose-6-phosphate, and lactic acid content of human skeletal muscle in various myopathies.
1965; National Institutes of Health; Volume: 66; Issue: 3 Linguagem: Inglês
Autores Tópico(s)
Genetic Neurodegenerative Diseases
ResumoAbstract Muscle biopsy specimens from 25 patients with and without clinical and histologic cvidence of muscle disease were studied for evidence of possible primary defects in glycolysis. Diseased muscle from patients with myasthenia gravis, lower motor neuron disease, polyarteritis, polymyositis, dermatomyositis, ocular myopathy, and progressive muscular dystrophy of the Duchenne type were assayed for phosphorylase activity and glycogen, glucose-6-phosphate, lactate, and noncollagen protein content. The data indicate that phosphorylase activity decreases in both hereditary and acquired diseases of muscle. Muscle from some patients with myasthcnia gravis showed moderately decreased phosphorylase activity. Denervating diseases produced a greater decrease, but the most marked decrease was seen in patients with primary muscle disease. Glycogen content varied considerably, both in normal and diseased muscle. Glucose-6-phosphate content also varied in the primary myopathies, but in general was decreased. On the other hand. lactate tended to accumulate, particularly in progressive muscular dystrophy. Some possible metabolic implications of these findings are discussed. Though encompassing a relatively small number of patients in each clinical group, the data do not support the presence of a primary disturbance in glycolysis in any of the conditions studied.
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