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

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

Research output: Contribution to journalArticle

4 Citations (Scopus)

Abstract

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
JournalHemoglobin
Volume10
Issue number4
DOIs
StatePublished - Jan 1 1986

Fingerprint

Thalassemia
Heterozygote
Hot Temperature
Genes
Globins
Chromosome Mapping
Genetic Recombination
Hemoglobins
Blood
hybrid hemoglobins
hemoglobin P-Nilotic
In Vitro Techniques
Experiments
hemoglobin Kenya

ASJC Scopus subject areas

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

Cite this

Lanclos, K. D., Kutlar, A., Kutlar, F., Ojwang, P. J., Reese, A. L., & Huisman, T. H. J. (1986). The effect of αthalassemia on the level of hybrid hemoglobin variants in heterozygotes. Hemoglobin, 10(4), 401-416. https://doi.org/10.3109/03630268608996870

The effect of αthalassemia on the level of hybrid hemoglobin variants in heterozygotes. / Lanclos, K. D.; Kutlar, Abdullah; Kutlar, Ferdane; Ojwang, P. J.; Reese, A. L.; Huisman, T. H.J.

In: Hemoglobin, Vol. 10, No. 4, 01.01.1986, p. 401-416.

Research output: Contribution to journalArticle

Lanclos, KD, Kutlar, A, Kutlar, F, Ojwang, PJ, Reese, AL & Huisman, THJ 1986, 'The effect of αthalassemia on the level of hybrid hemoglobin variants in heterozygotes', Hemoglobin, vol. 10, no. 4, pp. 401-416. https://doi.org/10.3109/03630268608996870
Lanclos, K. D. ; Kutlar, Abdullah ; Kutlar, Ferdane ; Ojwang, P. J. ; Reese, A. L. ; Huisman, T. H.J. / The effect of αthalassemia on the level of hybrid hemoglobin variants in heterozygotes. In: Hemoglobin. 1986 ; Vol. 10, No. 4. pp. 401-416.
@article{e7cd6e3fd3dc4574abd04a4a1bdf49ed,
title = "The effect of αthalassemia on the level of hybrid hemoglobin variants in heterozygotes",
abstract = "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.",
author = "Lanclos, {K. D.} and Abdullah Kutlar and Ferdane Kutlar and Ojwang, {P. J.} and Reese, {A. L.} and Huisman, {T. H.J.}",
year = "1986",
month = "1",
day = "1",
doi = "10.3109/03630268608996870",
language = "English (US)",
volume = "10",
pages = "401--416",
journal = "Hemoglobin",
issn = "0363-0269",
publisher = "Informa Healthcare",
number = "4",

}

TY - JOUR

T1 - The effect of αthalassemia on the level of hybrid hemoglobin variants in heterozygotes

AU - Lanclos, K. D.

AU - Kutlar, Abdullah

AU - Kutlar, Ferdane

AU - Ojwang, P. J.

AU - Reese, A. L.

AU - Huisman, T. H.J.

PY - 1986/1/1

Y1 - 1986/1/1

N2 - 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.

AB - 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.

UR - http://www.scopus.com/inward/record.url?scp=0022637932&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0022637932&partnerID=8YFLogxK

U2 - 10.3109/03630268608996870

DO - 10.3109/03630268608996870

M3 - Article

C2 - 2427479

AN - SCOPUS:0022637932

VL - 10

SP - 401

EP - 416

JO - Hemoglobin

JF - Hemoglobin

SN - 0363-0269

IS - 4

ER -