PMID: 6166190Jan 1, 1981Paper

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

American Journal of Hematology
T H HuismanA E Felice

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...Continue Reading

References

Jul 1, 1978·Proceedings of the National Academy of Sciences of the United States of America·S M Hanash, D L Rucknagel
Jul 11, 1978·Molecular & General Genetics : MGG·R L Strausberg, P S Perlman
Dec 1, 1976·British Journal of Haematology·G ChalevelakisD J Weatherall
Mar 1, 1975·The Journal of Clinical Investigation·W G Wood, G Stamatoyannopoulos
May 1, 1975·British Journal of Haematology·M H SteinbergB J Dreiling
Feb 16, 1972·Nature: New Biology·J M WhiteJ Reeve
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Mar 31, 1970·Biochimica Et Biophysica Acta·T MiyataK H Stenzel
Mar 1, 1980·Pediatric Research·A E FeliceT H Huisman

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Citations

Jan 1, 1984·Hemoglobin·Y OhbaS Miwa
Jan 1, 1982·Hemoglobin·T NakatsujiT H Huisman
Jul 1, 1986·British Journal of Haematology·R S WeinbergB P Alter

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