title: [Various features of muscle tissue metabolism in various muscular and neuromuscular diseases of a hereditary nature (clinico-histochemical study)].
abstract: Data of histological and histochemical examinations of 85 biopsy specimens of muscular tissue taken from patients with hereditary muscular and neuromuscular diseases are presented. The diseases were: the extremity-and-lumbar form of myopathy congenital myopathies, Charcot-Marie's neural amyotrophy, Steinert-Batten's atrophic myotonia, and other rare muscular diseases. Use was made of histochemical methods of determining the activity of the enzymes of the Krebs cycle and glycolysis. The data obtained are compared with the clinical peculiarities of each disease.
mesh_terms: D002607:Charcot-Marie-Tooth Disease / Q000201:enzymology; D008058:Dihydrolipoamide Dehydrogenase / Q000378:metabolism; D006012:Glycogen Storage Disease Type V / Q000201:enzymology; D006651:Histocytochemistry; D006801:Humans; D007770:L-Lactate Dehydrogenase / Q000378:metabolism; D009132:Muscles / Q000201:enzymology*; D009135:Muscular Diseases / Q000201:enzymology*; D009136:Muscular Dystrophies / Q000201:enzymology; D009223:Myotonic Dystrophy / Q000201:enzymology; D009468:Neuromuscular Diseases / Q000201:enzymology*
chemical_list: D007770:L-Lactate Dehydrogenase; D008058:Dihydrolipoamide Dehydrogenase
vernacular_title: Nekotorye osobennosti metabolizma myshechnoĭ tkani pri razlichnykh myshechnykh i nervno-myshechnykh zabolevaniiakh nasledstvennoĭ prirody (kliniko-gistokhimicheskoe issledovanie).