hemoglobin H

he·mo·glo·bin H

[MIM*142309] a homotetramer of Hb (all four polypeptides identical) of molecular formula β₄, found only when α chain synthesis is depressed and not effective in oxygen transport. Hb H disease (α-thalassemia intermedia) is a thalassemialike syndrome in people heterozygous for both severe and mild genes for α-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.

单词	hemoglobin h
释义	DictionarySeethalassemia hemoglobin H hemoglobin H [′hē·mə‚glō·bən ′āch] (pathology) An abnormal hemoglobin migrating more rapidly than normal hemoglobin on electrophoresis, and usually associated with thalassemia. hemoglobin H he·mo·glo·bin H [MIM*142309] a homotetramer of Hb (all four polypeptides identical) of molecular formula β₄, found only when α chain synthesis is depressed and not effective in oxygen transport. Hb H disease (α-thalassemia intermedia) is a thalassemialike syndrome in people heterozygous for both severe and mild genes for α-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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