Hb Setif alpha94(G1)Asp->Tyr
         
CONTACT alpha1beta2 contact
HEMATOLOGY Normal in heterozygotes
ELECTROPHORESIS Hb X moves to the position of Hb S on starch gel and cellulose acetate at both alkaline and acidic pH
CHROMATOGRAPHY Hb A and Hb X separate by cation and anion exchange HPLC
STRUCTURE STUDIES Tryptic digestion; separation of peptides by cation exchange chromatography or fingerprinting; amino acid analysis; sequencing
DNA ANALYSES Not reported; presumed mutation GAC->TAC; alpha2 or alpha1
FUNCTIONAL STUDIES Oxygen affinity about normal; decreased cooperativity
STABILITY Unstable
OCCURRENCE Found in families from Algeria, Iran, Lebanon, Saudi Arabia, etc.
OTHER INFORMATION Quantity in heterozygotes 12-15%; red cells with Hb Setif "sickle" by intracellular crystallization of insoluble Hb
       
REFERENCES
1. Wajcman, H., Belkhodja, O., and Labie, D.: FEBS Lett., 27:298, 1972.
2. Debray, J., Krulik, M., Mehaut, M., Benabadji, M., Trabuchet, G., Wajcman, H., Gacon, G., and Labie, D.: Nouv. Rev. Fr. d'Hematol., 14:627, 1974.
3. Nozari, G. and Rahbar, S.: Hemoglobin, 1:289, 1977.
4. Aubert, J-P., Drupt, F., Rousseaux, J., and Loucheux-Lefebvre, M-H.: 84:375, 1977.
5. Raik, E., Powell, E., Fleming, P., and Gordon, S.: Pathology, 15:453, 1983.
6. Al-Awamy, B., Niazi, G.A., Wilson, J.B., and Huisman, T.H.J.: Hemoglobin, 9:87, 1985.
7. Charache, S., Raik, E., Holtzclaw, D., Hathaway, P.J., Powell, E., and Fleming, P.: Blood, 70:237, 1987.


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.