Hb O-Indonesia	alpha116(GH4)Glu->Lys

ALSO KNOWN AS		Buginese-X; Oliviere
CONTACT		External
HEMATOLOGY		Normal in the heterozygote
ELECTROPHORESIS		Hb X moves slightly slower than Hb S at alkaline pH; Hb X moves as a sharp band close to but between Hb A and Hb S on citrate agar at acidic pH; excellent separation by IEF
CHROMATOGRAPHY		Hb X was isolated by cation and anion exchange HPLC
STRUCTURE STUDIES		Tryptic digestion; separation of peptides by fingerprinting; amino acid analysis
DNA ANALYSES		A GAG->AAG mutation at codon 116 of the alpha1 gene (Ref. 3)
FUNCTIONAL STUDIES		Not reported
STABILITY		Slightly less stable than Hb A
OCCURRENCE		Found in Buginese from Sulawesi (formerly Celebes), Makassars, Iranian, South African, Italian, and Chinese families
OTHER INFORMATION		Quantity in heterozygotes 21%; found in association with Hb D-Punjab and with beta-thal

REFERENCES
1.		Lie-Injo, L.E.: The Lancet, ii:1338, 1957.
2.		Baglioni, C. and Lehmann, H.: Nature, 196:229, 1962.
3.		Molchanova, T.P., Pobedimskaya, D.D., and Huisman, T.H.J.: Br. J. Haematol., 88:300, 1994.

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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.