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Nature Medicine 8, 662 - 664 (2002)
doi:10.1038/nm0702-662
Extra Rabs unsnarl a lipid traffic jam
Martin O. Bergo1 & Stephen G. Young1
- Gladstone Institute of Cardiovascular Disease, Cardiovascular Research Institute, Department of Medicine, University of California, San Francisco San Francisco, California, USA e-mail: syoung@gladstone.ucsf.edu
Abstract
Niemann–Pick type C (NPC) disease is a fatal lipid-storage disorder caused by a 'lipid traffic jam' in late endosomes and lysosomes. A recent study indicates that overexpression of the vesicular transport proteins Rab7 or Rab9 increases the flow of traffic, allowing accumulated lipids to leave the endosomes and reach the Golgi apparatus and endoplasmic reticulum.
Niemann–Pick type C (NPC) disease is a recessive disorder characterized by the accumulation of lipids within late endosomes and lysosomes in many tissues, including the liver, spleen, and brain1, 2. The lipid accumulation in the brain causes a merciless neurodegenerative syndrome that is generally diagnosed in early preschool years and is fatal 5–10 years later.
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