Hb G-Georgia	alpha95(G2)Pro->Leu

CONTACT		alpha1beta2 contact
HEMATOLOGY		Normal in the heterozygote
ELECTROPHORESIS		Hb X moves to the position of Hb G on starch gel and slightly slower than Hb G on cellulose acetate at alkaline pH
CHROMATOGRAPHY		Hb X and Hb A can be separated by anion and cation exchange chromatography
STRUCTURE STUDIES		Tryptic peptides are separated by cation exchange chromatography; amino acid analysis
DNA ANALYSES		A CCG->CTG mutation at codon 95 of the alpha2-globin gene (Ref. 5)
FUNCTIONAL STUDIES		Increased oxygen affinity; decreased heme-heme interaction; normal Bohr effect
STABILITY		Increased dissociation into dimers (oxyHb); tetramers (deoxyHb)
OCCURRENCE		Found in several Black families and in a Portuguese family
OTHER INFORMATION		Quantity in heterozygotes 14-16%; found in combination with Hb S; also in combination with alpha-thal

REFERENCES
1.		Huisman, T.H.J., Adams, H.R., Wilson, J.B., Efremov, G.D., Reynolds, C.A., and Wrightstone, R.N.: Biochim. Biophys. Acta, 200:578, 1970.
2.		Smith, L.L., Plese, C.F., Barton, B.P., Charache, S., Wilson, J.B., and Huisman, T.H.J.: J. Biol. Chem., 247:1433, 1972.
3.		Wrightstone, R.N., Hubbard, M., and Huisman, T.H.J.: Acta Haematol., 51: 315, 1974.
4.		North, M.L., Garel, M.C., Thillet, J., Oberling, Fr., Lang, J.M., Mayer, S., and Rosa, J.: Nouv. Rev. Fr. d'Hematol., 15:460, 1975.
5.		Smetanina, N.S., Leonova, J.Y., Levy, N., and Huisman, T.H.J.: Biochim. Biophys. Acta, 1315:188, 1996

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This material is from the book A Syllabus of Human Hemoglobin Variants (1996) by Titus H.J. Huisman, Marianne F.H. Carver, and Georgi D. Efremov, published by The Sickle Cell Anemia Foundation in Augusta, GA, USA. Copyright © 1996 by Titus H.J. Huisman. All rights reserved. Neither this work nor any part may be reproduced or transmitted in any form or by any means, electronic or mechanical, microfilming and recording, or by any information storage and retrieval systems, without written permission.