Hemoglobin J Iran alpha 2 beta 2 77 (EF1) his----Asp in a Russian-Armenian family

A third case of Hb J Iran is reported. The propositus is of Russian-Armenian origin and was investigated for hematuria. The electrophoretic behavior and the characterization of primary structure are described. Hb J Iran is stable and has normal functional properties. High resolution Nuclear Magnetic...

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Veröffentlicht in:Hemoglobin 1986, Vol.10 (4), p.365-378
Hauptverfasser: Delanoe-Garin, J, Rhoda, M D, Craescu, C T, Bardakjian, J, Blouquit, Y, Lacombe, C, Arous, N, Poyart, C, Ganeval, D, Girot, R
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Sprache:eng
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Zusammenfassung:A third case of Hb J Iran is reported. The propositus is of Russian-Armenian origin and was investigated for hematuria. The electrophoretic behavior and the characterization of primary structure are described. Hb J Iran is stable and has normal functional properties. High resolution Nuclear Magnetic Resonance spectra suggest the presence of structural perturbations in the heme pocket of the variant. Solubility studies of Hb S/Hb J Iran mixture indicated that His beta 77 belongs to a contact region of deoxy Hb S polymers.
ISSN:0363-0269
1532-432X
DOI:10.3109/03630268608996868