Hb Cranston beta144+beta145 (+AA); abnormal C-terminal sequence:
HEMATOLOGY Compensated hemolytic anemia in the heterozygote
ELECTROPHORESIS Hb X moves close to Hb S at alkaline pH
CHROMATOGRAPHY Hb X was isolated on a DEAE-cellulose column
STRUCTURE STUDIES Tryptic digestion of AE-betaX chain; separation of peptides by fingerprinting; amino acid analysis; sequencing
DNA ANALYSES Not reported; presumed frameshift at codon beta145 (+AG)
FUNCTION STUDIES Increased oxygen affinity; decreased Bohr effect and coop-erativity
STABILITY Unstable; dissociation of subunits, hybrids
OCCURRENCE Found in members of an Italian-American family
OTHER INFORMATION Quantity in the heterozygote 30-40%
1. Bunn, H.F., Schmidt, G.J., Haney, D.N., and Dluhy, R.G.: Proc. Natl. Acad. Sci. USA, 72:3609, 1975.
2. McDonald, M.J., Lund, D.P., Bleichman, M., Bunn, H.F., DeYoung, A., Noble, R.W., Foster, B., and Arnone, A.: J. Mol. Biol., 140:357, 1980.
3. Shaeffer, J.R., Schmidt, G.J., Kingston, R.E., and Bunn, H.F.: J. Mol. Biol., 140: 377, 1980.

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.