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Quality control of cytoplasmic components by lysosomal enzymes Takashi Ueno 1 , Eiki Kominami 1 1Department of Biochemistry, Juntendo University School of Medicine Keyword: リソソーム , オートファジー , カテプシン , タンパク分解 pp.27-35
Published Date 2004/2/10
DOI https://doi.org/10.11477/mf.1431100176
  • Abstract
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 Autophagy, a process by which bulk cellular proteins are turned over via the lysosomal/vacuolar system, substantially contributes to the quality control of cytoplasmic components by removing aged or injured cell constituents that are formed in cells exposed to various stimuli and stresses. In a number of inherited neuromuscular diseases, aberrant autophagic protein turnover has been shown to play a substantial role in the progression of diseases. Some diseases(Danon disease and neuronal ceroid lipofuscinosis)have been demonstrated to be due to mutation of the genes directly involved in four elementary steps of autophagy. In most other neuromuscular diseases, the responsible genes are not directly relevant to autophagy. However, abnormal accumulation of autophagsomes and autolysosomes is frequently observed as a common symptom. One common mechanism underlying these diseases is that mutations cause conformational disorders of some particular proteins. The altered proteins have a strong tendency to form insoluble aggregates. These aggregates can be sequestered into autophagosomes as the substrates of autophagy, but they are resistant to lysosomal proteinases. The accumulation of these aggregates results in a block in autophagy.

(Received:September 16, 2003)


Copyright © 2004, Igaku-Shoin Ltd. All rights reserved.

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電子版ISSN 1882-1243 印刷版ISSN 0001-8724 医学書院

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