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M Hirano, Y Ohba, K Imai, T Ino, Y Morishita, T Matsui, S Shimizu, H Sumi, K Yamamoto and T Miyaji
A new unstable hemoglobin with high oxygen affinity, Hb Toyoake: beta 142
(H20) Ala replaced by Pro, was found in Japanese male with a normal blood
hemoglobin level, shortened red cell survival, and increased plasma
erythropoietin. Hemoglobin studies showed heat and isopropanol instability,
and an increased tendency to heme loss and to subunit dissociation.
Electrophoresis of whole hemolysate showed inconstant abnormal bands with
reduced mobilities due to progressive heme loss during the in vitro
procedure. Isolated Hb Toyoake with normal heme content migrated slightly
faster than HbA. Oxygen affinity of red cells was elevated with P50 of 17.0
mm Hg at pH 7.4 and 37 degrees C (normal 25.0 mm Hg). Studies on hemolysate
implied that Hb Toyoake had an almost normal Bohr effect, a diminished
cooperativity, and a reduced response to inositol hexaphosphate. These
multiple abnormalities are associated with a substitution of Pro for beta
142 Ala, resulting in disruption of the H-helix and the adjacent C-terminal
portion of beta chain, which contain residues crucial for normal oxygen
binding.
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| Copyright © 1981 by American Society of Hematology Online ISSN: 1528-0020 | |||||||||