Hb Fukuoka [β2(NA2)HISTYR]: A New Nutation at the 2,3-Diphosphoglycerate Binding Site

Abstract

Two abnormal hemoglobins (Hbs) with a substituted amino acid residue at the 2nd position of the B chain, a part of the 2,3-diphosphoglycerate (2,3-DPG) binding site, have been reported: Hb Deer Lodge (His+Arg) (1) and Hb Okayama (His-Gln) (2). In 1985, we found a new abnormal Hb during assay of Hb A1c and Hb A1 by high performance liquid chromatography (HPLC) in a 38-year-old Japanese female patient suffering from diabetes mellitus. This abnormal Hb was designated Hb Fukuoka [B2(NA2)HisTyr] after the name of the city where the patient lived. Herein, we describe the structural analysis and functional properties of this Hb.