Hemoglobin Evanston: alpha 14(A12) Trp arrow right Arg. A variant hemoglobin associated with alpha -thalassemia-2

• Published in: Biochimica et biophysica acta, Protein structure and molecular enzymology Vol. 747; no. 1-2; pp. 65 - 70
• Main Authors: Moo-Penn, W F, Baine, R M, Jue, D L, Johnson, M H, McGuffey, JE, Benson, J M
• Format: Journal Article
• Language: English
• Published: 01.01.1983
• Subjects: hemoglobin Evanston; man
• ISSN: 0167-4838

Hb Evanston ( alpha 14 Trp arrow right Arg) was detected on cellulose acetate at pH 8.4 as a band with an electrophoretic mobility similar to that of Hb S. In addition, a band migrating cathodic to Hb A sub(2) suggested the presence of a variant Hb A sub(2) with a substitutions in the alpha -chain, a fact that was later confirmed by structural analysis. An unusual feature of Hb Evanston is its low percentage; less than 10% occurs in the hemolysate. Studies indicate that the variant is not unstable, but there appears to be a defect in globin-chain synthesis. Gene mapping also shows that it is associated with the alpha -thalassemia-2 gene. The variant has high oxygen affinity with normal cooperativity and a normal Bohr effect. The combination of Hb Evanston with alpha -thalassemia-2 produced anemia in this black family.