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unstable hemoglobins

 

Definition: a group of rare Hb's with amino acid substitutions (or amino acid deletions in three types) that alter the three-dimensional shape of the globin in a manner that renders the molecule unstable; they have an increased but variable tendency to autooxidation and Heinz body formation and are associated with congenital nonspherocytic hemolytic anemia. The unstable ß-chain abnormalities include Hb's Genova, Gun Hill, Hammersmith, Köln, Philly, Sabine, Santa Ana, Sydney, Wien, and Zürich; unstable a-chain abnormalities include Hb's Bibba, Sinai, and Torino.

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© Copyright 2017 Wolters Kluwer. All Rights Reserved. Review Date: Sep 19, 2016.
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