title: Hemoglobin Hope: studies of oxygen equilibrium in heterozygotes, hemoglobin S-Hope disease, and isolated hemoglobin Hope.
abstract: Hemoglobin Hope (beta(H14)136gly leads to asp), a mildly unstable variant, was found to have decreased oxygen affinity, a normal Bohr effect and diminished cooperativity. Decreased oxygen affinity of hemoglobin Hope may explain the previous failure to find an appropriate response to hemolysis in individuals studied who were heterozygous for both hemoglobin Hope and sickle hemoglobin. Salt bridge formation between NA1 valine and H14 aspartic acid may stabilize the beta Hope subunit in its deoxy form thus producing intrinsically low oxygen affinity and reduced cooperativity.
mesh_terms: D004163:Diphosphoglyceric Acids / Q000097:blood; D004912:Erythrocytes / Q000032:analysis; D006447:Hemoglobin H / Q000032:analysis* / Q000302:isolation & purification; D006451:Hemoglobin, Sickle / Q000032:analysis* / Q000302:isolation & purification; D006453:Hemoglobinopathies / Q000097:blood*; D006455:Hemoglobins, Abnormal / Q000032:analysis*; D006579:Heterozygote; D006801:Humans; D010100:Oxygen / Q000097:blood*
chemical_list: D004163:Diphosphoglyceric Acids; D006451:Hemoglobin, Sickle; D006455:Hemoglobins, Abnormal; D006447:Hemoglobin H; D010100:Oxygen