Hb Poissy beta56(D7)Gly->Arg;
beta86(F2)Ala->Pro
         
HEMATOLOGY Erythrocytosis in the heterozygote
ELECTROPHORESIS Hb X moves like Hb Hamadan at alkaline pH, i.e. slower than Hb A and almost like Hb S
CHROMATOGRAPHY Hb X and Hb A separate by DEAE-cellulose chromatography
STRUCTURE STUDIES Tryptic digestion of AE-betaX chain; separation of peptides by fingerprinting and reversed phase HPLC; amino acid analysis
DNA ANALYSES Not reported; presumed mutation GGC->CGC and GCC->CCC at codons 56 and 86
FUNCTION STUDIES Increased oxygen affinity; decreased Bohr effect and cooperativity
STABILITY Mildly unstable; increased autooxidation
OCCURRENCE Found in a 56-year-old French male
OTHER INFORMATION The beta56 Gly->Arg replacement is found in Hb Hamadan
       
REFERENCES
1. Lacombe, C., Craescu, C.T., Blouquit, Y., Kister, J., Poyart, C., Delanoe-Garin, J., Arous, N., Bardakdjian, J., Riou, J., Rosa, J., Schaeffer, C., and Galacteros, F.: Eur. J. Biochem., 153:655, 1985.


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.