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Thalassemia

HRI protein has protective role in red cell disorders

Published in Blood Weekly, June 16th, 2005

Erythropoietic protoporphyria (EPP) and beta-thalassemia are two types of inherited red blood cell disorders.

In a new study appearing in the June 1, 2005, issue of the Journal of Clinical Investigation, Jane-Jane Chen and colleagues from the Massachusetts Institute of Technology demonstrate a protective role of a protein called HRI, which had never previously been shown to play a role in any known human disease, in these red cell disorders. In both diseases, HRI deficiency worsens EPP and beta-thalassemia, while HRI itself decreases disease severity in mice, by reducing the amount of a protein called alpha globin, which has toxic effects.

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