Hb Stanleyville-II alpha78(EF7)Asn->Lys
CONTACT External
HEMATOLOGY Normal in heterozygotes
ELECTROPHORESIS Hb X moves as Hb S on starch block, paper, starch gel, and cellulose acetate at alkaline pH; Hb X moves with Hb A on citrate agar at acidic pH
CHROMATOGRAPHY Hb X and Hb A separate by cation and anion exchange chromatography
STRUCTURE STUDIES Tryptic digestion; separation of peptides by fingerprinting; amino acid analysis
DNA ANALYSES Not reported; presumed mutation AAC->AAG; alpha2 or alpha1
OCCURRENCE Found in Black families from the Congo, Uganda, Zaire, Texas (USA), and Alsace (France)
OTHER INFORMATION Quantity in heterozygotes ~24%; in combination with Hb S it forms a hybrid Hb (alpha2St-IIbeta2S) which in contrast to normal Hb S (alpha2beta2S) does not readily sickle or form fibers (Ref. 5)
1. Dherte, P., Vandepitte, J., Ager, J.A.M., and Lehmann, H.: Br. Med. J., ii:282, 1959.
2. Schneider, R.G. and Haggard, M.E.: Br. Med. J., ii:285, 1959.
3. Van Ros, G., Beale, D., and Lehmann, H.: Br. Med. J., 4:92, 1968.
4. North, M.L., Darbre, P.D., Lehmann, H., and Juif, J.G.: Acta Haematol., 53:56, 1975.
5. Rhoda, M-D., Martin, J., Blouquit, Y., Garel, M-C., Edelstein, S.J., and Rosa, J.: Biochem. Biophys. Res. Commun., 111:8, 1983.

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.