Mitoferrin is essential for erythroid iron assimilation
作者： George C. Shaw , John J. Cope , Liangtao Li , Kenneth Corson , Candace Hersey
DOI: 10.1038/NATURE04512
关键词:
摘要: Iron has a fundamental role in many metabolic processes, including electron transport, deoxyribonucleotide synthesis, oxygen transport and essential redox reactions involving haemoproteins Fe-S cluster proteins. Defective iron homeostasis results either deficiency or overload. Precise regulation of mitochondria is for haem biosynthesis, haemoglobin production protein assembly during red cell development. Here we describe zebrafish mutant, frascati (frs), that shows profound hypochromic anaemia erythroid maturation arrest owing to defects mitochondrial uptake. Through positional cloning, show the gene mutated frs mutant member vertebrate solute carrier family (SLC25) call mitoferrin (mfrn). mfrn highly expressed fetal adult haematopoietic tissues mouse. Erythroblasts generated from murine embryonic stem cells null Mfrn (also known as Slc25a37) with severely impaired incorporation 55Fe into haem. Disruption yeast orthologues, MRS3 MRS4, causes metabolism biogenesis. Murine rescues zebrafish, complements indicating function may be conserved. Our data functions principal importer biosynthesis erythroblasts.
nature.com
harvard.edu
sci-hub.se 参考文章(30)
S. E. Lyons, N. D. Lawson, L. Lei, P. E. Bennett, B. M. Weinstein, P. P. Liu, A nonsense mutation in zebrafish gata1 causes the bloodless phenotype in vlad tepes. Proceedings of the National Academy of Sciences of the United States of America. ,vol. 99, pp. 5454- 5459 ,(2002) , 10.1073/PNAS.082695299
Fang-Yuan Li, Barbara Leibiger, Ingo Leibiger, Catharina Larsson, Characterization of a putative murine mitochondrial transporter homology of hMRS3/4. Mammalian Genome. ,vol. 13, pp. 20- 23 ,(2002) , 10.1007/S00335-001-2094-Y
Aidas Nasevicius, Stephen C. Ekker, Effective targeted gene ‘knockdown’ in zebrafish Nature Genetics. ,vol. 26, pp. 216- 220 ,(2000) , 10.1038/79951
Cindy N. Roy, David M. Penny, John N. Feder, Caroline A. Enns, The hereditary hemochromatosis protein, HFE, specifically regulates transferrin-mediated iron uptake in HeLa cells. Journal of Biological Chemistry. ,vol. 274, pp. 9022- 9028 ,(1999) , 10.1074/JBC.274.13.9022
Ichiro Iuchi, Masamichi Yamamoto, Erythropoiesis in the developing rainbow trout, Salmo gairdneri irideus: histochemical and immunochemical detection of erythropoietic organs. Journal of Experimental Zoology. ,vol. 226, pp. 409- 417 ,(1983) , 10.1002/JEZ.1402260311
Roland Lill, Ulrich Mühlenhoff, Iron–sulfur-protein biogenesis in eukaryotes Trends in Biochemical Sciences. ,vol. 30, pp. 133- 141 ,(2005) , 10.1016/J.TIBS.2005.01.006
Quan-Zhen Li, Sarah Eckenrode, Qing-Guo Ruan, Cong-Yi Wang, Jing-Da Shi, Richard A. McIndoe, Jin-Xiong She, Rapid decrease of RNA level of a novel mouse mitochondria solute carrier protein (Mscp) gene at 4–5 weeks of age Mammalian Genome. ,vol. 12, pp. 830- 836 ,(2001) , 10.1007/S00335001-2075-1
Hartmut Wohlrab, The human mitochondrial transport protein family: identification and protein regions significant for transport function and substrate specificity. Biochimica et Biophysica Acta. ,vol. 1709, pp. 157- 168 ,(2005) , 10.1016/J.BBABIO.2005.07.003
Fang-Yuan Li, Kaisu Nikali, Juraj Gregan, Ingo Leibiger, Barbara Leibiger, Rudolf Schweyen, Catharina Larsson, Anu Suomalainen, Characterization of a novel human putative mitochondrial transporter homologous to the yeast mitochondrial RNA splicing proteins 3 and 4. FEBS Letters. ,vol. 494, pp. 79- 84 ,(2001) , 10.1016/S0014-5793(01)02319-5
Françoise Foury, Tiziana Roganti, Deletion of the mitochondrial carrier genes MRS3 and MRS4 suppresses mitochondrial iron accumulation in a yeast frataxin-deficient strain. Journal of Biological Chemistry. ,vol. 277, pp. 24475- 24483 ,(2002) , 10.1074/JBC.M111789200