FEBS Letters
Volume 580, Issue 23 , Pages 5500-5509, 9 October 2006

Diseases of intramembranous lipid transport

Edited by Gerrit van Meer

Amsterdam Liver Center, Department of Experimental Hepatology, Academic Medical Center, Meibergdreef 69-71, S-1-168, 1105 BK Amsterdam, The Netherlands

Received 2 June 2006; received in revised form 19 June 2006; accepted 20 June 2006. published online 30 June 2006.

Abstract 

The maintenance of transbilayer distribution of phospholipids is crucial for proper cell function. Intramembrane transport of lipids is mediated by three activities termed floppases, flippases, and scramblases. Members of the ATP-binding cassette transporter family and P-type ATPase superfamily have been implicated in the translocation of lipids. The importance of these activities is exemplified by several severe human inherited disorders that are caused by defects in intramembranous transport of lipids. In order to elucidate the molecular mechanisms that underlie these disorders, the combination of in vivo, biochemical, and structural analyses on intramembrane transporters is crucial.

Keywords: Transbilayer phospholipid distribution, ABC transporter, P-type ATPase, Human disease

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PII: S0014-5793(06)00784-8

doi:10.1016/j.febslet.2006.06.067

FEBS Letters
Volume 580, Issue 23 , Pages 5500-5509, 9 October 2006

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