Lysosomal and nonlysosomal hydrolases of skeletal muscle in neuromuscular diseases

Takala, T.E.; Myllylä, V.V.; Salminen, A.; Tolonen, U.; Hassinen, I.E.; Vihko, V.

Archives of Neurology 40(9): 541-544

1983


ISSN/ISBN: 0003-9942
PMID: 6351816
DOI: 10.1001/archneur.1983.04050080041006
Accession: 043568792

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Abstract
The activities of four lysosomal and two nonlysosomal hydrolases were studied in skeletal muscle biopsy samples from patients with neuromuscular diseases and from controls. beta-Glucosaminidase activity was increased in polymyositis. beta-Glucuronidase and alkaline protease activities were elevated in muscular dystrophy in adults, whereas cathepsin D activity was increased in amyotrophic lateral sclerosis. There were significant correlations between the activities of lysosomal and nonlysosomal hydrolases. The activity of beta-glucuronidase, beta-glucosaminidase, alkaline protease, and dipeptidyl aminopeptidase IV showed a positive correlation with the severity of muscular atrophy. The activities of these hydrolases and the activity of dipeptidyl aminopeptidase I correlated positively with the activities of muscular galactosylhydroxylysyl glucosyltransferase and with the serum concentration of type III procollagen aminoterminal propeptide. The results suggest that in neuromuscular diseases the lysosomal and nonlysosomal pathways for muscle degradation are affected concomitantly with collagen biosynthesis.

Lysosomal and nonlysosomal hydrolases of skeletal muscle in neuromuscular diseases