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| HEMATOLOGY | |
Mild hemolytic anemia; more severe when drug induced or by viral infection; reticulocytosis | |
| ELECTROPHORESIS | |
Hb X moves in the position of Hb S at alkaline pH; no separation from Hb A at acidic pH | |
| CHROMATOGRAPHY | |
Hb X elutes between Hb A2 and Hb A from an anion exchange column | |
| STRUCTURE STUDIES | |
Tryptic digestion; separation of peptides by fingerprinting or cation exchange chromatography; HPLC; amino acid analysis | |
| DNA ANALYSES | |
Not reported | |
| FUNCTION STUDIES | |
Slightly increased oxygen affinity; normal Bohr effect and cooperativity; decreased response to 2,3-DPG and IHP | |
| STABILITY | |
Unstable | |
| OCCURRENCE | |
Found in a few families from The Netherlands, China, USA, South Africa, Mexico, Yugoslavia | |
| OTHER INFORMATION | |
Quantity in heterozygotes 19-31%; found in combination with betao-thal, causing severe hemolytic anemia | |
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| REFERENCES |
| 1. | |
De Jong, W.W.W., Went, L.N., and Bernini, L.F.: Nature, 220:788, 1968. | |
| 2. | |
Nagel, R.L., Rieder, R.F., Bookchin, R.M., and James, G.W.: Biochem. Biophys. Res. Commun., 53:1240, 1973. | |
| 4. | |
Rieder, R.F. and James, G.W., III: J. Clin. Invest., 54:948, 1974. | |
| 3. | |
Lie-Injo, L.E., Ganesan, J., Randhawa, Z.I., Peterson, D., and Kane, J.P.: Am. J. Hema-tol., 2:335, 1977. | |
| 4. | |
Rieder, R.F. and James, G.W.: Blood, 47:489, 1976. | |
| 5. | |
Zeng, Y-T., Huang, S-Z., Zhou, X-D., Chen, R-J., Yi, S-C., and Wang, C-J.; Chinese Med. J., 60:581, 1980. | |
| 6. | |
Perlman, M.M., Wiltshire, B.G., Stevens, K., Cassel, R., and Lehmann, H.: S.A. Med. J., 59:537, 1981. | |
| 7. | |
Juricic, D., Ruzdic, I., Beer, Z., Efremov, G.D., Casey, R., and Lehmann, H.: Hemoglobin, 7:271, 1983. | |