Hb Rampa alpha95(G2)Pro->Ser
CONTACT alpha1beta2 contact
HEMATOLOGY Normal in the heterozygote
ELECTROPHORESIS Hb X moves slightly faster than Hb S on starch gel and like Hb S on cellulose acetate at alkaline pH; Hb X does not separate from Hb A on agar gel at acidic pH
CHROMATOGRAPHY Hb X and Hb A were separated on a DEAE-cellulose column
STRUCTURE STUDIES Tryptic digestion; separation of peptides by fingerprinting; cation exchange chromatography; amino acid analysis; sequencing
DNA ANALYSES Not reported; presumed mutation CCG->TCG; alpha2 or alpha1
FUNCTIONAL STUDIES Increased oxygen affinity; decreased heme-heme interaction and Bohr effect
STABILITY Dissociation into dimers (oxyHb); tetramers (deoxyHb)
OCCURRENCE Found in a few Indian families and in a family of North European ancestry
OTHER INFORMATION Quantity in heterozygotes 15%
1. De Jong, W.W.W., Bernini, L.F., and Meera Khan, P.: Biochim. Biophys. Acta, 236:197, 1971.
2. Smith, L.L., Plese, C.L., Barton, B.P., Charache, S., Wilson, J.B., and Huisman, T.H.J.: J. Biol. Chem., 247:1434, 1972.

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.