Br J Haematol. 1990 Apr;74(4):531-4.

Haemoglobin alpha 2 beta 2 23Val----Ile produced in Escherichia coli facilitates Hb S polymerization.

Pagnier J, Baudin-Chich V, Lacaze N, Bohn B, Poyart C.

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INSERM U299 Hôpital de Bicêtre, Le Kremlin-Bicêtre, France.

Abstract

The doubly substituted variant Hb S-Antilles (beta 6 Glu----Val, beta 23 Val----Ile) produces sickling in heterozygous carriers. The Csat value for pure deoxyHb S-Antilles is nearly half that of deoxyHb S. Dilute solutions of pure Hb S-Antilles have a lower oxygen affinity than those of Hb A or Hb S. The mutant Hb alpha 2 beta 2 23 Val----Ile was synthesized in E. coli. It exhibits a decreased oxygen affinity compared to Hb A and does not polymerize in 1.8 M phosphate buffer. Mixtures of equal amounts of Hb S + Hb beta 23 Val----Ile have a decreased Csat value compared to mixtures of Hb S + Hb A. The beta 23 Val in Hb S contributes to the axial contact joining molecules in each single filament. Substituting Ile for Val at this site increases the strength of this contact through hydrophobic interactions, allowing increased stability of the lateral contact between filaments in pair, which is the specific unit structure of polymers in deoxyHb S.

PMID:: 2189492; [PubMed - indexed for MEDLINE]

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[Polymerization and solubility of recombinant hemoglobins alpha 2 beta 2 6 Glu-->Ala (Hb Makassar) and alpha 2 beta 2 6 Glu-->Ala, 23 Val-->Ile]. [C R Acad Sci III. 1993]
[Polymerization and solubility of recombinant hemoglobins alpha 2 beta 2 6 Glu-->Ala (Hb Makassar) and alpha 2 beta 2 6 Glu-->Ala, 23 Val-->Ile].
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Hemoglobin S Antilles: a variant with lower solubility than hemoglobin S and producing sickle cell disease in heterozygotes.
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Cited by 1 PubMed Central article

Steric and hydrophobic determinants of the solubilities of recombinant sickle cell hemoglobins. [Protein Sci. 1992]
Steric and hydrophobic determinants of the solubilities of recombinant sickle cell hemoglobins.
Bihoreau MT, Baudin V, Marden M, Lacaze N, Bohn B, Kister J, Schaad O, Dumoulin A, Edelstein SJ, Poyart C. Protein Sci. 1992 Jan; 1(1):145-50.

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