The effect of αthalassemia on the level of hybrid hemoglobin variants in heterozygotes

K. D. Lanclos, A. Kutlar, F. Kutlar, P. J. Ojwang, A. L. Reese, T. H.J. Huisman

Research output: Contribution to journalArticlepeer-review

4 Scopus citations


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 γbeta; 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 αchain deficiency (αthalassemia) is present. Such a posttranslational control mechanism was not observed for Hb Lepore, with normal α chains and δbeta; hybrid chains, and Hb P-Nilotic, with normal α chains and βdelta; 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.

Original languageEnglish (US)
Pages (from-to)401-416
Number of pages16
Issue number4
StatePublished - 1986

ASJC Scopus subject areas

  • Hematology
  • Clinical Biochemistry
  • Genetics(clinical)
  • Biochemistry, medical


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