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Alpha-thalassemia

New alpha-thalassemia study results from University of Paris described

Published in Blood Weekly, February 11th, 2010

In this recent report, researchers in Le Kremlin-Bicetre, France conducted a study "To determine the role of the alpha-hemoglobin stabilizing protein (AHSP) in the clinical expression of alpha-hemoglobin (alpha-Hb) variants described as unstable, ten alpha chain variants have been studied with their chaperone. AHSP specifically binds free alpha-Hb to form a soluble heterodimer until it is replaced by the beta-Hb partner."

"In this way, AHSP prevents the precipitation of free alpha chains which might damage the membrane of erythrocyte. AHSP specifically recognizes the G and H helices of alpha-Hb that are also involved in the alpha 1 beta 1 dimer interface. AHSP may act...

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