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hemoglobin H


Definition: a homotetramer of Hb (all four polypeptides identical) of molecular formula ß4, found only when a chain synthesis is depressed and not effective in oxygen transport. Hb H disease (a-thalassemia intermedia) is a thalassemialike syndrome in people heterozygous for both severe and mild genes for a-thalassemia; moderate anemia and red blood cell abnormalities with 25–35% Hb Bart at birth, but with Hb Bart later replaced by Hb H and with Hb A2 decreased. Hb H shows no cooperativity with O2 binding and does not exhibit a Bohr effect.

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© Copyright 2018 Wolters Kluwer. All Rights Reserved. Review date: Sep 19, 2016.