Jamming the endosomal system: lipid rafts and lysosomal storage diseases.
Trends Cell Biol
; 10(11): 459-62, 2000 Nov.
Article
en En
| MEDLINE
| ID: mdl-11050411
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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Colección:
01-internacional
Banco de datos:
MEDLINE
Asunto principal:
Endosomas
/
Enfermedades por Almacenamiento Lisosomal
/
Microdominios de Membrana
/
Lisosomas
/
Lípidos de la Membrana
Tipo de estudio:
Etiology_studies
Límite:
Humans
Idioma:
En
Revista:
Trends Cell Biol
Año:
2000
Tipo del documento:
Article
País de afiliación:
Alemania