Hb Zürich beta63(E7)His->Arg
CONTACT External; surface crevice; "distal histidine"
HEMATOLOGY Normal in the heterozygote; certain oxidative drugs precipitate hemolytic episodes with inclusion bodies
ELECTROPHORESIS Hb X and Hb A can be separated at alkaline pH; Hb X moves slightly faster than Hb S
CHROMATOGRAPHY Hb X and Hb A can be separated by cation and anion exchange chromatography
STRUCTURE STUDIES Tryptic digestion; separation of peptides by fingerprinting; amino acid analysis; chymotryptic digestion
DNA ANALYSES Not reported; presumed mutation CAT->CGT at codon 63
FUNCTION STUDIES Increased oxygen affinity; decreased cooperativity; normal Bohr effect
STABILITY Mildly unstable
OCCURRENCE Found in families from Switzerland and from the USA
OTHER INFORMATION Quantity in the heterozygote 22-35%; stability of the variant is influenced by oxidative drugs
1. Hitzig, W.H., Frick, P.G., Betke, K., and Huisman, T.H.J.: Helv. Paediatr. Acta, 15:399, 1960.
2. Muller, C.J. and Kingma, S.: Biochim. Biophys. Acta, 40:595, 1961.
3. Frick, P.G., Hitzig, W.H., and Betke, K.: Blood, 20:261, 1962.

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.