[Lysosomal hydrolases in the process of muscular atrophy and dystrophy (a histochemical study)].
Non-specific esterase, acid phosphotase, beta-glucoronidase, and N-acetyl-beta-glucosamindase were revealed using the histochemical method of asocombinations in biopsy specimens of the muscular tissue taken from 99 patients suffering from neurogenic and myogenic disorders. Biopsy specimens were taken also from 7 healthy persons. Concentrations of the granular product of the reaction correlated with autofluorescence in the same section. Some bioptic materials were investigated using electron-microscopy. It was established that activity of lysosomal hydrolases in the normal muscular tissue was low, being increased with age. Autofluorescence in the majority of cases was well noticible. In the groups of atrophic fibres in neurogenic atrophy there were observed focal elevation of the lysosomal enzymatic activity. Product of the reaction, which pointed to the activity of nonspecific esterase and acid phosphotase, was localized predominantly along the periphery of muscular fibres. Autofluorescence of the product of the reaction was considerable. On the other hand, there were extensive areas of muscular fibres in which the concentration of the reaction product did not exceed the normal level. Activity of hydrolases in cells of the mesenchyma was of a low degree. Ist type of the lysosomal activity was observed in myogenic dystrophy with an acute course of the disease; it was characterized by structural changes in the muscular tissue, activation of the all hydrolases under study, absence of autofluorescence on the areas of localization of the reaction product, participation of the mesenchyma cells in the lysosomal activation. IInd type of the lysosomal activity was noted in the muscular tissue in neurogenic atrophy. There was no difference in principle between neuro- and myogenic lesions as far as the type of lysosomal activity is concerned. Collation of the results obtained in the investigations with the data reported in available literature enabled the authors to put forward the concept of the two types of the lysosomal activity in muscular lesions: 1) diffuse elevation of the lysosome activity associated with structural changes in the muscular tissue; 2) focal elevation of the physiological activity due to formation of lipopigment.
D000118:Acetylglucosaminidase / Q000378:metabolism; D000135:Acid Phosphatase / Q000378:metabolism; D000293:Adolescent; D000328:Adult; D001706:Biopsy; D002648:Child; D002675:Child, Preschool; D004796:Clinical Enzyme Tests; D004950:Esterases / Q000378:metabolism; D005966:Glucuronidase / Q000378:metabolism; D006801:Humans; D006867:Hydrolases / Q000378:metabolism*; D007223:Infant; D008247:Lysosomes / Q000201:enzymology*; D008854:Microscopy, Electron; D008875:Middle Aged; D009133:Muscular Atrophy / Q000201:enzymology* / Q000473:pathology; D009136:Muscular Dystrophies / Q000201:enzymology* / Q000473:pathology; D009461:Neurologic Manifestations
D006867:Hydrolases; D004950:Esterases; D000135:Acid Phosphatase; D005966:Glucuronidase; D000118:Acetylglucosaminidase
Lizosomal'nye gidrolazy v protsesse myshechnykh atrofiĭ i distrofiĭ (gistokhimicheskoe issledovanie)