Journal
MOLECULAR AND CELLULAR BIOLOGY
Volume 34, Issue 6, Pages 1077-1084Publisher
AMER SOC MICROBIOLOGY
DOI: 10.1128/MCB.00865-13
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Funding
- Ministry of Education, Culture, Sports, Science, and Technology of Japan [23701037, 22501047]
- Kodama Memorial Fund for Medical Research
- Grants-in-Aid for Scientific Research [23701037, 25430114, 22501047] Funding Source: KAKEN
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Abcb10, member 10 of the ABC transporter family, is reportedly a part of a complex in the mitochondrial inner membrane with mitoferrin-1 (Slc25a37) and ferrochelatase (Fech) and is responsible for heme biosynthesis in utero. However, it is unclear whether loss of Abcb10 causes pathological changes in adult mice. Here, we show that Abcb10(-/-) mice lack heme biosynthesis and erythropoiesis abilities and die in midgestation. Moreover, we generated Abcb10(F/-); Mx1-Cre mice, with Abcb10 in hematopoietic cells deleted, which showed accumulation of protoporphyrin IX and maturation arrest in reticulocytes. Electron microscopy images of Abcb10(-/-) hematopoietic cells showed a marked increase of iron deposits at the mitochondria. These results suggest a critical role for Abcb10 in heme biosynthesis and provide new insights into the pathogenesis of erythropoietic protoporphyria and sideroblastic anemia.
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