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BioMed Research International
Volume 2015 (2015), Article ID 278536, 9 pages
Review Article

Biology of Heme in Mammalian Erythroid Cells and Related Disorders

1Department of Hematology and Rheumatology, Tohoku University Graduate School of Medicine, Sendai 980-8575, Japan
2Molecular Hematology/Oncology, Tohoku University Graduate School of Medicine, Sendai 980-8575, Japan

Received 11 April 2015; Accepted 14 June 2015

Academic Editor: Aurora M. Cianciarullo

Copyright © 2015 Tohru Fujiwara and Hideo Harigae. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


Heme is a prosthetic group comprising ferrous iron (Fe2+) and protoporphyrin IX and is an essential cofactor in various biological processes such as oxygen transport (hemoglobin) and storage (myoglobin) and electron transfer (respiratory cytochromes) in addition to its role as a structural component of hemoproteins. Heme biosynthesis is induced during erythroid differentiation and is coordinated with the expression of genes involved in globin formation and iron acquisition/transport. However, erythroid and nonerythroid cells exhibit distinct differences in the heme biosynthetic pathway regulation. Defects of heme biosynthesis in developing erythroblasts can have profound medical implications, as represented by sideroblastic anemia. This review will focus on the biology of heme in mammalian erythroid cells, including the heme biosynthetic pathway as well as the regulatory role of heme and human disorders that arise from defective heme synthesis.