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Erythroid heme biosynthesis and its disorders
#MMPMID23471474
Dailey HA
; Meissner PN
Cold Spring Harb Perspect Med
2013[Apr]; 3
(4
): a011676
PMID23471474
show ga
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.
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