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In vitro synthesis of hemoglobin and hemoglobin chains in the BFUe‐derived colonies from persons with α‐ or β‐thalassemia
Author(s) -
Huisman T. H. J.,
Reese A. L.,
Webber B.,
Okonjo K.,
Altay C.,
Felice A. E.
Publication year - 1981
Publication title -
american journal of hematology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.456
H-Index - 105
eISSN - 1096-8652
pISSN - 0361-8609
DOI - 10.1002/ajh.2830100302
Subject(s) - alpha (finance) , alpha chain , alpha thalassemia , thalassemia , hemoglobin , reticulocyte , beta (programming language) , hereditary spherocytosis , biology , chemistry , microbiology and biotechnology , medicine , genotype , genetics , gene , rna , construct validity , nursing , computer science , patient satisfaction , programming language
Abstract The synthesis of α and non‐α chains of human hemoglobin (Hb) was studied in reticulocytes and in BFUe‐derived cell colonies from patients with α chain or β chain deficiencies. The subjects included normal adults (αα/αα) with or without a β chain variant (Hb S, Hb Leslie) or an α chain variant (Hb G‐Georgia); α‐thalassemia‐2 heterozygotes (α 0 α/αα) with an α chain variant (G‐Georgia or G‐Philadelphia); an α‐thalassemia‐1 heterozygote (α 0 α 0 /αα); α‐thalassemia‐2 homozygotes (α 0 α/α 0 α) with a β chain variant (Hb S), an α chain variant (G‐Philadelphia), a Hb S homozygosity with Hb G‐Philadelphia, or a Hb G‐Philadelphia homozygosity; and three black β + ‐thalassemia homozygotes. Data from reticulocyte in vitro synthesis analyses showed the expected deficiencies. However, similar analyses of the Hb synthesized in cell colonies (even from the black β‐thalassemia homozygotes) gave (nearly) balanced ∑α/∑non‐α ratios. It is speculated that this balanced synthesis is due to a most effective proteolysis in the immature erythroblast which rapidly removes free α or β chains. The levels of Hb F and Hb A 2 were considerably increased in these proerythroblasts; a two‐ to threefold increase in the synthesis of Hb A 2 was observed over that seen in the reticulocytes.