Hb Gun Hill beta91(F7)-beta95(FG1) Leu-His-Cys-Asp-Lys->0
HEMATOLOGY Compensated hemolytic anemia with Heinz bodies after incubation with brilliant cresyl blue; reticulocytosis
ELECTROPHORESIS Hb X1 (the larger component) migrates with Hb A2 at alkaline pH
CHROMATOGRAPHY Hb X can be isolated on a DEAE-cellulose column; it elutes before Hb A
STRUCTURE STUDIES Tryptic digestion; separation of peptides by fingerprinting; amino acid analysis
DNA ANALYSES Not reported
FUNCTION STUDIES Increased oxygen affinity; decreased cooperativity; no Bohr effect
STABILITY Unstable; increased dissociation
OCCURRENCE Found in members of a Caucasian family of German-English ancestry, and in a Black female living in Alabama
OTHER INFORMATION Quantity in the heterozygote 22-26%
1. Bradley, T.B., Wohl, R.C., and Rieder, R.F.: Science, 157:1581, 1967.
2. Rieder, R.F. and Bradley, T.B.: Blood, 32:355, 1968.
3. Bradley, T.B., Parkhurst, L.J., Udem, L., and Gibson, Q.H.: Proceedings of the Inter-American Symposium on Hemoglobins, Caracas, page 168, 1969.
4. Rieder, R.F.: J. Clin. Invest., 50:388, 1971.
5. Murari, J., Smith, L.L., Wilson, J.B., Schneider, R.G., and Huisman, T.H.J.: Hemoglobin, 1:267, 1977.

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.