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Down-regulation of α-globin synthesis causes α-thalassemia with underproduction of fetal (HbF, α(2)γ(2)) and adult (HbA, α(2)β(2)) hemoglobin. This article focuses on the human α-globin cluster, which has been characterized in great depth over the past 30 years. In particular the authors describe how the α genes are normally switched on during erythropoiesis and switched off as hematopoietic stem cells commit to nonerythroid lineages. In addition, the principles by which α-globin expression may be perturbed by natural mutations that cause α-thalassemia are reviewed.

Original publication

DOI

10.1016/j.hoc.2010.08.005

Type

Journal article

Journal

Hematol Oncol Clin North Am

Publication Date

12/2010

Volume

24

Pages

1033 - 1054

Keywords

Erythropoiesis, Fetal Hemoglobin, Hemoglobin A, Humans, Models, Genetic, Multigene Family, Mutation, alpha-Globins, alpha-Thalassemia