Format

Send to

Choose Destination
See comment in PubMed Commons below
Cold Spring Harb Perspect Med. 2013 Apr 1;3(4):a011676. doi: 10.1101/cshperspect.a011676.

Erythroid heme biosynthesis and its disorders.

Author information

1
Department of Microbiology, Department of Biochemistry and Molecular Biology, Biomedical and Health Sciences Institute, University of Georgia, Athens, GA 30602, USA. hdailey@uga.edu

Abstract

Heme, which is composed of iron and the small organic molecule protoporphyrin, is an essential component of hemoglobin as well as a variety of physiologically important hemoproteins. During erythropoiesis, heme synthesis is induced before, and is essential for, globin synthesis. Although all cells possess the ability to synthesize heme, there are distinct differences between regulation of the pathway in developing erythroid cells and all other types of cells. Disorders that compromise the ability of the developing red cell to synthesize heme can have profound medical implications. The biosynthetic pathway for heme and key regulatory features are reviewed herein, along with specific human genetic disorders that arise from defective heme synthesis such as X-linked sideroblastic anemia and erythropoietic protoporphyria.

PMID:
23471474
PMCID:
PMC3683999
DOI:
10.1101/cshperspect.a011676
[Indexed for MEDLINE]
Free PMC Article
PubMed Commons home

PubMed Commons

0 comments
How to join PubMed Commons

    Supplemental Content

    Full text links

    Icon for HighWire Icon for PubMed Central
    Loading ...
    Support Center