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cholesterol transport

Saturday 3 June 2006

Connections between cholesterol transport and intracellular-membrane transport

The study of Niemann-Pick disease type C provides interesting clues to the role of membrane cholesterol and membrane domains enriched with cholesterol and glycosphingolipids in vesicular transport and membrane-associated sorting events.

Niemann-Pick disease type C is characterized by the storage of lipids in various tissues, the clinical hallmark being severe neurodegeneration.

In the cells of patients with this disorder, free cholesterol is unable to exit lysosomes because of mutations in a gene that encodes NPC-1, a multimembrane-spanning protein found in late endocytic compartments.

The lysosomal accumulation of cholesterol and the resulting disturbance of cellular cholesterol homeostatic responses are likely to disrupt the organization of lipid microdomains in cellular membranes.

This effect may account for some of the observed disturbances in protein localization in affected patients and may constitute part of the molecular background of the neurodegenerative symptoms.

See also

- Cholesterol

  • cholesterol biosynthesis
  • cholesterol metabolism


- Olkkonen VM, Ikonen E. Genetic defects of intracellular-membrane transport. N Engl J Med. 2000 Oct 12;343(15):1095-104. PMID: 11027745