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- Title
Pathogenic mechanisms in lysosomal disease: a reappraisal of the role of the lysosome.
- Authors
Walkley, Steven U
- Abstract
The view that lysosomes simply represent end organelles in the serial degradation of polymeric molecules derived from the cell surface and its interior has led to major misconceptions about the nature of lysosomal storage diseases and the pathogenic cascades that characterize them. Accordingly, lysosomal storage bodies are often considered 'inert', inducing cell dysfunction and death primarily through mechanical overcrowding of normal organelles or by other non-specific means leading to generalized cytotoxicity. However, modern studies of lysosomes and their component proteins provide evidence to support a far greater role for these organelles in cell metabolism. In intimate association with endosomal, autophagosomal and related vesicular systems, the greater lysosomal system can be conceptualized as a vital recycling centre that serves as a central metabolic coordinator, influencing literally every aspect of the cell, from signal transduction to regulation of gene expression.
- Publication
Acta paediatrica (Oslo, Norway : 1992), 2007, Vol 96, Issue 455, p26
- ISSN
1651-2227
- Publication type
Journal Article
- DOI
10.1111/j.1651-2227.2007.00202.x