Understanding globin regulation in beta-thalassemia: it's as simple as alpha, beta, gamma, delta.
A vast excess of alpha-globin production and inadequate gamma-globin compensation lead to the development of severe anemia in human beta-thalassemia. Newly identified modifiers of alpha- and gamma-globin synthesis and insights into the mechanisms of globin regulation provide the tools for potential new approaches to treating this and other red blood cell disorders. In the study by Han and colleagues in this issue of the JCI, the activity of a heme-regulated protein, HRI, is shown to modulate the accumulation of excess alpha-globin chains in murine beta-thalassemia and to decrease the severity of the disease.
Bank
Arthur
A
Department of Medicine, Columbia University College of Physicians and Surgeons, Armand Hammer Health Science Center, New York, New York 10032, USA. ab13@columbia.edu
D000818:Animals; D002880:Chromosomes, Human, Pair 11 / Q000235:genetics / Q000378:metabolism; D002885:Chromosomes, Human, Pair 16 / Q000235:genetics / Q000378:metabolism; D005786:Gene Expression Regulation; D005914:Globins / Q000096:biosynthesis* / Q000235:genetics; D006801:Humans; D019901:Locus Control Region / Q000235:genetics*; D051379:Mice; D005810:Multigene Family / Q000235:genetics*; D017086:beta-Thalassemia / Q000235:genetics / Q000378:metabolism*; D019892:eIF-2 Kinase / Q000235:genetics / Q000378:metabolism*
D005914:Globins; D019892:eIF-2 Kinase