Cathepsin L1 plays an important role in the overall degradation of proteins in lysosomes. Its substrates include collagen and elastin, as well as α-1 protease inhibitor, a major controlling element of neutrophil elastase activity. It has been implicated in several pathologic processes, including myofibril necrosis in myopathies and in myocardial ischemia, and in the renal tubular response to proteinuria.
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