Hb Catonsville Glu- inserted between
alpha37(C2)Pro and alpha38(C3)Thr
HEMATOLOGY Mild erythrocytosis; reticulocytosis; hemolytic episodes
ELECTROPHORESIS Hb X moves faster than Hb A at alkaline pH; at acidic pH Hb X moves between Hb A and Hb F
CHROMATOGRAPHY Hb X elutes faster than Hb A from a cation exchange HPLC column; no resolution of chains (alphaX and alphaA) by reversed phase HPLC
STRUCTURE STUDIES Tryptic digestion of alphaX chain; separation of peptides by reversed phase HPLC; amino acid analysis; sequencing
DNA ANALYSES Insertion of GAA between CCC [codon 37 (= Pro)] and ACC [codon 38 (= Thr)]
FUNCTION STUDIES Increased oxygen affinity; reduced cooperativity and Bohr effect
OCCURRENCE Found in a young child and his father
OTHER INFORMATION Quantity in the heterozygote 24.2%
1. Moo-Penn, W.F., Swan, D.C., Hine, T.K., Baine, R.M., Jue, D.L., Benson, J.M., Johnson, M.H., Virshup, D.M., and Zinkham, W.H.: J. Biol. Chem., 264:21454, 1989.

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.