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In vitro synthesis of hemoglobin and hemoglobin chains in the BFUe-derived colonies form person with alpha- or beta-thalassemia.

Abstract
The synthesis of alpha and non-alpha chains of human hemoglobin (Hb) was studied in reticulocytes and in BFUe-derived cell colonies from patients with alpha chain or beta chain deficiencies. The subjects included normal adults (alpha alpha/alpha alpha) with or without a beta chain variant (Hb S, Hb Leslie) or an alpha chain variant (Hb G-Georgia); alpha-thalassemia-2 heterozygotes (alpha 0 alpha/alpha alpha) with an alpha chain variant (G-Georgia or G-Philadelphia); an alpha-thalassemia-1 heterozygote (alpha 0 alpha 0/alpha alpha); alpha-thalassemia-2 homozygotes (alpha 0 alpha 0/alpha 0 alpha) with a beta chain variant (Hb S), an alpha chain variant (G-Philadelphia), a Hb S homozygosity with Hb G-Philadelphia, or a Hb G-Philadelphia homozygosity; and three black beta +-thalassemia homozygotes. Data from reticulocyte in vitro synthesis analysis showed the expected deficiencies. However, similar analyses of the Hb synthesized in cell colonies (even from the black beta-thalassemia homozygotes) gave (nearly) balanced sigma alpha/sigma non-alpha ratios. It is speculated that this balanced synthesis is due to a most effective proteolysis in the immature erythroblast which rapidly removes free alpha or beta chains. The levels of Hb F and Hb A2 were considerably increased in these proerythroblasts; a two- to threefold increase in the synthesis of Hb A2 was observed over that seen in the reticulocytes.
AuthorsT H Huisman, A L Reese, B Webber, K Okonjo, C Altay, A E Felice
JournalAmerican journal of hematology (Am J Hematol) Vol. 10 Issue 3 Pg. 227-37 ( 1981) ISSN: 0361-8609 [Print] United States
PMID6166190 (Publication Type: Journal Article, Research Support, U.S. Gov't, P.H.S.)
Chemical References
  • Hemoglobins
  • Hemoglobin A2
  • Fetal Hemoglobin
Topics
  • Adolescent
  • Adult
  • Aged
  • Cells, Cultured
  • Child
  • Child, Preschool
  • Female
  • Fetal Hemoglobin (biosynthesis)
  • Genetic Variation
  • Hemoglobin A2 (biosynthesis)
  • Hemoglobins (biosynthesis, genetics)
  • Heterozygote
  • Homozygote
  • Humans
  • Male
  • Middle Aged
  • Reticulocytes (metabolism)
  • Thalassemia (blood, genetics)

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