| Content Provider |
World Health Organization (WHO)-Global Index Medicus |
| Author |
Enders, Anselm Parish, Christopher R. Winterberg, Markus Kirk, Kiaran Yabas, Mehmet Cromer, Deborah D'rozario, James Coupland, Lucy A. Teoh, Narci C. Bröer, Stefan |
| Description |
Author Affiliation: Yabas M ( From the Ramaciotti Immunization Genomics Laboratory and.); Coupland LA ( Cancer and Vascular Biology Group, Department of Immunology, The John Curtin School of Medical Research, and the Clinical Haematology Unit, The Canberra Hospital, Canberra, Australian Capital Territory 2605, Australia.); Cromer D ( the Complex Systems in Biology Group, Centre for Vascular Research, University of New South Wales, Kensington, New South Wales 2052, Australia, and.); Winterberg M ( the Division of Biomedical Science and Biochemistry, Research School of Biology, Australian National University, Canberra, Australian Capital Territory 2601, Australia.); Teoh NC ( the Liver Research Group, Australian National University Medical School at the Canberra Hospital, Canberra, Australian Capital Territory 2605, Australia.); D'Rozario J ( the Clinical Haematology Unit, The Canberra Hospital, Canberra, Australian Capital Territory 2605, Australia.); Kirk K ( the Division of Biomedical Science and Biochemistry, Research School of Biology, Australian National University, Canberra, Australian Capital Territory 2601, Australia.); Bröer S ( the Division of Biomedical Science and Biochemistry, Research School of Biology, Australian National University, Canberra, Australian Capital Territory 2601, Australia.); Parish CR ( Cancer and Vascular Biology Group, Department of Immunology, The John Curtin School of Medical Research, and.); Enders A ( From the Ramaciotti Immunization Genomics Laboratory and anselm.enders@anu.edu.au.) |
| Abstract |
Transmembrane 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 ATP11C showed a lower rate of PS translocation in vitro compared with erythrocytes from wild-type littermates. Furthermore, the mutant mice had an elevated percentage of phosphatidylserine-exposing 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. |
| ISSN |
00219258 |
| e-ISSN |
1083351X |
| Journal |
Journal of Biological Chemistry |
| Issue Number |
28 |
| Volume Number |
289 |
| Language |
English |
| Publisher |
American Society for Biochemistry and Molecular Biology (United States) |
| Publisher Date |
2014-07-11 |
| Publisher Place |
United States |
| Access Restriction |
Open |
| Subject Keyword |
Adenosine Triphosphatases Metabolism Erythrocyte Membrane Enzymology Phospholipids Acid-Base Imbalance Genetics Pathology Anemia, Hemolytic, Congenital Animals Biological Transport, Active Cell Survival Physiology Erythrocytes, Abnormal Metabolism, Inborn Errors Mice Mice, Mutant Strains Research Support, Non-U.S. Gov't Biochemistry Molecular Biology |
| Content Type |
Text |
| Resource Type |
Article |
| Subject |
Cell Biology Biochemistry Molecular Biology |
