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HEMATOLOGY | |
Compensated hemolytic anemia with Heinz bodies after incubation with brilliant cresyl blue; reticulocytosis | |
ELECTROPHORESIS | |
Hb X1 (the larger component) migrates with Hb A2 at alkaline pH | |
CHROMATOGRAPHY | |
Hb X can be isolated on a DEAE-cellulose column; it elutes before Hb A | |
STRUCTURE STUDIES | |
Tryptic digestion; separation of peptides by fingerprinting; amino acid analysis | |
DNA ANALYSES | |
Not reported | |
FUNCTION STUDIES | |
Increased oxygen affinity; decreased cooperativity; no Bohr effect | |
STABILITY | |
Unstable; increased dissociation | |
OCCURRENCE | |
Found in members of a Caucasian family of German-English ancestry, and in a Black female living in Alabama | |
OTHER INFORMATION | |
Quantity in the heterozygote 22-26% | |
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REFERENCES |
1. | |
Bradley, T.B., Wohl, R.C., and Rieder, R.F.: Science, 157:1581, 1967. | |
2. | |
Rieder, R.F. and Bradley, T.B.: Blood, 32:355, 1968. | |
3. | |
Bradley, T.B., Parkhurst, L.J., Udem, L., and Gibson, Q.H.: Proceedings of the Inter-American Symposium on Hemoglobins, Caracas, page 168, 1969. | |
4. | |
Rieder, R.F.: J. Clin. Invest., 50:388, 1971. | |
5. | |
Murari, J., Smith, L.L., Wilson, J.B., Schneider, R.G., and Huisman, T.H.J.: Hemoglobin, 1:267, 1977. | |