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P4 ATPases: Flippases in Health and Disease
Tytgat Institute for Liver and Intestinal Research, Academic Medical Center, Meibergdreef 69-71, 1105 BK Amsterdam, The Netherlands
* Author to whom correspondence should be addressed.
Received: 6 March 2013; in revised form: 28 March 2013 / Accepted: 7 April 2013 / Published: 11 April 2013
Abstract: P4 ATPases catalyze the translocation of phospholipids from the exoplasmic to the cytosolic leaflet of biological membranes, a process termed “lipid flipping”. Accumulating evidence obtained in lower eukaryotes points to an important role for P4 ATPases in vesicular protein trafficking. The human genome encodes fourteen P4 ATPases (fifteen in mouse) of which the cellular and physiological functions are slowly emerging. Thus far, deficiencies of at least two P4 ATPases, ATP8B1 and ATP8A2, are the cause of severe human disease. However, various mouse models and in vitro studies are contributing to our understanding of the cellular and physiological functions of P4-ATPases. This review summarizes current knowledge on the basic function of these phospholipid translocating proteins, their proposed action in intracellular vesicle transport and their physiological role.
Keywords: P-type ATPase; P4 ATPase; flippase; phospholipid; membrane asymmetry; vesicular transport; disease; ATP8B1; CDC50
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Cite This Article
MDPI and ACS Style
van der Mark, V.A.; Elferink, R.P.O.; Paulusma, C.C. P4 ATPases: Flippases in Health and Disease. Int. J. Mol. Sci. 2013, 14, 7897-7922.
van der Mark VA, Elferink RPO, Paulusma CC. P4 ATPases: Flippases in Health and Disease. International Journal of Molecular Sciences. 2013; 14(4):7897-7922.
van der Mark, Vincent A.; Elferink, Ronald P.O.; Paulusma, Coen C. 2013. "P4 ATPases: Flippases in Health and Disease." Int. J. Mol. Sci. 14, no. 4: 7897-7922.