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
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

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.