Article
Heme deficiency in erythroid lineage causes differentiation arrest and cytoplasmic iron overload.
The EMBO journal - 15 Nov 1999
Nakajima O, Takahashi S, Harigae H, Furuyama K, Hayashi N, Sassa S, Yamamoto M
Abstract excerpt
Erythroid 5-aminolevulinate synthase (ALAS-E) catalyzes the first step of heme biosynthesis in erythroid cells. Mutation of human ALAS-E causes the disorder X-linked sideroblastic anemia. To examine the roles of heme during hematopoiesis, we disrupted the mouse ALAS-E gene. ALAS-E-null embryos showed no hemoglobinized cells and died by embryonic day 11.5, indicating that ALAS-E is the principal isozyme...
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