Hemoglobin Istanbul: substitution of glutamine for histidine in a proximal histidine (F8(92) ).
A presumably spontaneous mutation has resulted in the formation of Hemoglobin (Hb) Istanbul in which glutamine is substituted for histidine in the proximal position of the beta-chain (F8(92)). The anemia and other physiological effects that occur in the presence of Hb Istanbul were much ameliorated by splenectomy. Hb Istanbul is a relatively unstable molecule which produces a rather moderate case of "unstable hemoglobin hemolytic anemia."In the determination of structure, a method of preferential cleavage of an aspartyl-proline bond at residues 99-100 of the beta-chain was used.
D000328:Adult; D000595:Amino Acid Sequence; D000596:Amino Acids / Q000097:blood; D055598:Chemical Phenomena; D002621:Chemistry; D002848:Chromatography, DEAE-Cellulose; D004592:Electrophoresis, Starch Gel; D005973:Glutamine / Q000097:blood*; D006453:Hemoglobinopathies / Q000235:genetics / Q000628:therapy; D006455:Hemoglobins, Abnormal / Q000032:analysis*; D006639:Histidine / Q000097:blood*; D006358:Hot Temperature; D006801:Humans; D006863:Hydrogen-Ion Concentration; D008297:Male; D008970:Molecular Weight; D009154:Mutation; D010455:Peptides / Q000097:blood; D013156:Splenectomy; D014364:Tryptophan / Q000097:blood
D000596:Amino Acids; D006455:Hemoglobins, Abnormal; D010455:Peptides; D005973:Glutamine; D006639:Histidine; D014364:Tryptophan