Yabas, MehmetCoupland, Lucy ACromer, DeborahWinterberg, MarkusTeoh, Narci CD'Rozario, JamesKirk, KiaranBröer, StefanParish, ChristopherEnders, Anselm2015-01-162015-01-160021-9258http://hdl.handle.net/1885/12545Transmembrane lipid transporters are believed to establish and maintain phospholipid asymmetry in biological membranes; however, little is known about the in vivo function of the specific transporters involved. Here, we report that developing erythrocytes from mice lacking the putative phosphatidylserine flippase ATP11Cshowed a lower rate ofPStranslocation in vitro compared with erythrocytes from wild-type littermates. Furthermore, the mutant mice had an elevated percentage of phosphatidylserineexposing mature erythrocytes in the periphery. Although erythrocyte development in ATP11C-deficient mice was normal, the mature erythrocytes had an abnormal shape (stomatocytosis), and the life span of mature erythrocytes was shortened relative to that in control littermates, resulting in anemia in the mutant mice. Thus, our findings uncover an essential role for ATP11C in erythrocyte morphology and survival and provide a new candidate for the rare inherited blood disorder stomatocytosis with uncompensated anemia.This work was supported in part by National Health and Medical Research Council Grant GNT1061288. Supported by National Health and Medical Research Council Career Development Fellowship GNT1035858 and by the Ramaciotti Foundation.8 pages© 2014 by The American Society for Biochemistry and Molecular Biology, Inc. Subject to 12 month restriction, author may post accepted version to institutional repository http://www.sherpa.ac.uk/romeo/issn/0021-9258/ Sherpa/Romeo as at 3 June 2015micephospholipidflippaseATP11Clife span2014-06-0410.1074/jbc.C114.5702672015-12-09