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.
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Authors | T H Huisman, A L Reese, B Webber, K Okonjo, C Altay, A E Felice |
Journal | American journal of hematology
(Am J Hematol)
Vol. 10
Issue 3
Pg. 227-37
( 1981)
ISSN: 0361-8609 [Print] United States |
PMID | 6166190
(Publication Type: Journal Article, Research Support, U.S. Gov't, P.H.S.)
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Chemical References |
- Hemoglobins
- Hemoglobin A2
- Fetal Hemoglobin
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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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