Hb F-Bonaire-Ga or αAγ²39(C5) Gln→Arg, Characterized by High Pressure Liquid Chromatographic and Microsequencing Procedures

Abstract

A slowly moving γ chain variant was discovered in the cord blood of a baby of English-Vietnamese descent. The abnormality concerned the substitution of Gin residue in position 39(C5) of the Aγ chain by an Arg residue resulting in an -Arg-Arg-sequence at positions 39 and 40. The quantity of the Ay chain variant was nearly 10% of the total Hb F with 15% of the Hb F having normal Aγ chains and 75% of Hb F having Gγ chains. High pressure liquid chromatographic and microsequencing methods greatly facilitated the structural analyses.