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Jamming the endosomal system: lipid rafts and lysosomal storage diseases

Authors
Journal
Trends in Cell Biology
0962-8924
Publisher
Elsevier
Publication Date
Volume
10
Issue
11
Identifiers
DOI: 10.1016/s0962-8924(00)01847-x
Disciplines
  • Medicine

Abstract

Abstract Some lysosomal storage diseases result from the accumulation of lipids in degradative compartments of the endocytic pathway. Particularly striking is the example of the Niemann–Pick (NP) syndrome. NP syndromes types A and B are characterized by the accumulation of sphingomyelin, whereas cholesterol typically accumulates in NP type C. These two different lipids, sphingomyelin and cholesterol, are normal constituents of specific lipid microdomains called rafts. Because accumulation of raft lipids is observed not only in NP diseases but also in many other lipidoses, we forward the hypothesis that lysosomal storage diseases can be caused by the accumulation of lipid rafts in late endosomes/lysosomes.

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