Gamma chain abnormal human fetal hemoglobin variants

Fetal Hemoglobin with Abnormal ‘ Y . Polypeptide Chains : Hemoglobin Warren

By T. H. J. HUISMAN, A. M. Dozy, B. E. HORTON AND J. B. WILsON T HE EXISTENCE of variants of normal human fetal hemoglobin ( HbFor a2y2 ) has been suggested in different reports in the literature. Variants of fetal hemoglobin with abnormal a-polypeptide chains have been described5 ”5 and some are well characterized.’0 Only one abnormality, namely the electrophoretically fast moving hemoglobin v...

Effect of hydroxyurea on G gamma chain fetal hemoglobin synthesis by sickle-cell disease patients.

Hydroxyurea is used for sickle-cell disease patients in order to increase fetal hemoglobin synthesis and consequently decrease the severity of pain episodes. Fetal hemoglobin, which is formed by gamma-globin chains A and G, is present in a constant composition throughout fetal development: about 75% of Ggamma and 25% of Agamma. In contrast, adult red cells contain about 40% of Ggamma and 60% of...

The gamma chain heterogeneity of fetal hemoglobin in black beta-thalassemia and HPFH heterozygotes.

High pressure liquid chromatography (HPLC) was applied to the HbF isolated from blood of numerous black patients with beta-thalassemia trait or homozygosity, G gamma-delta beta-thalassemia trait, G gamma A-gamma HPFH heterozygosity, or the G gamma-[delta+ beta+]-HPFH condition. The method allowed an accurate evaluation of the relative quantities of three types of gamma-chain (G gamma, A gamma I...

Detection of abnormal hemoglobin variants during glycohemoglobin analysis.

Screening for hemoglobin variants with abnormal oxygen affinity.

To the Editor: Rai et al. (1 ) recently described an elegant method for detecting hemoglobin (Hb) variants that cause erythrocytosis, using electrospray mass spectrometry. Many of these variants are not detectable with conventional electrophoretic techniques. The authors also stated that “functional studies of oxygen saturation curves would be the ideal method to screen for Hb variants that cau...