The effect of alpha-thalassemia on the level of hybrid hemoglobin variants in heterozygotes
Date
1986Author
Lanclos, KD
Kutlar, F
Ojwang, PJ
Reese, AL
Huisman, TH
Type
ArticleLanguage
enMetadata
Show full item recordAbstract
The influence of a relative deficiency in α chain production on the amount of Hemoglobins Kenya, P-Nilotic, and Lepore was determined. The level of these hybrid hemoglobins in heterozygotes was correlated to various states of α chain deficiency by: 1) quantitation of the variants in blood samples and comparing these data with the number of α globin genes determined by gene mapping, 2) in vitro recombination experiments involving isolated non-α chains and normal α chains, and 3) in vitro heat stability analyses of the isolated hemoglobins. Hb Kenya, composed of normal α and γ-β hybrid chains, is heat labile, has a decreased ability to combine with α chains, and its level in heterozygotes is greatly decreased when a concomitant α-chain deficiency (α-thalassemia) is present. Such a posttranslational control mechanism was not observed for Hb Lepore, with normal α chains and δ-β hybrid chains, and Hb P-Nilotic, with normal α chains and β-δ hybrid chains. The latter two variants are heat stable, and their hybrid chains combine equally well as normal β chains with normal α chains. Hb P-Nilotic is more heat stable than Hb A and its in vitro formation is increased over that of Hb S, and perhaps even Hb A, in conditions of severe α chain deficiency.
URI
http://informahealthcare.com/doi/abs/10.3109/03630268608996870?journalCode=hemhttp://hdl.handle.net/11295/30561
http://www.ncbi.nlm.nih.gov/pubmed/2427479
Citation
Hemoglobin.1986, Vol. 10, No. 4 , Pages 401-416Publisher
University of Nairobi Department of medicine