Muscular dystrophy and activation of proteinases
- 1 July 1978
- journal article
- review article
- Published by Wiley in Muscle & Nerve
- Vol. 1 (4), 308-313
- https://doi.org/10.1002/mus.880010407
Abstract
Evidence is presented for the existence of many different systems of proteolytic enzymes in human skeletal muscle. These include the lysosomal system of cathepsins as well as proteinases and peptide hydrolases that are optimally active at neutral and alkaline pH ranges. The majority of proteolytic enzymes examined are found to show increased activity in dystrophic human muscle. Moreover, a high initial rise is observed in cathepsin B1, a thiol‐dependent endopeptidase of lysosomes, and in dipeptidyl peptidase IV, a membrane‐associated peptidase. In addition, a calcium‐activated neutral proteinase is found to be significantly elevated in muscle from patients with Duchenne dystrophy. The possible roles of these proteinases in intracellular protein catabolism and muscle wasting are discussed.Keywords
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