Factors contributing to the clinical differences between sickle cell-hemoglobin C disease (SC) and the benign sickle cell trait (AS) include the higher proportion of hemoglobin (Hb) S and the higher cell Hb concentrations in SC compared with AS red cells. Reports differ, however, about whether Hb C copolymerizes more than Hb A with Hb S when measured by minimum gelling concentrations (MGCs) and...

Abnormal hemoglobins (Hbs) are the most commonly inherited disorders in humans. Their frequency and types change considerably with geographic location and ethnic group. To investigate the molecular epidemiological characterization of abnormal Hbs in eastern Guangdong of southern China, a total of 11,450 'healthy' subjects were subjected to hemoglobin electrophoresis screening. Samples of EDTA-K...

The SNO-Hb hypothesis holds that heme-bound nitric oxide (NO) present in the beta subunits of T-state hemoglobin (Hb) will be transferred to the beta-93 cysteine upon conversion to R-state Hb, thereby forming SNO-Hb. A deficiency in the ability of Hb to facilitate this intramolecular transfer has recently been purported to play a role in pulmonary hypertension and sickle cell disease. We prepar...

The SNO-Hb hypothesis holds that hemebound nitric oxide (NO) present in the subunits of T-state hemoglobin (Hb) will be transferred to the -93 cysteine upon conversion to R-state Hb, thereby forming SNO-Hb. A deficiency in the ability of Hb to facilitate this intramolecular transfer has recently been purported to play a role in pulmonary hypertension and sickle cell disease. We prepared deoxyge...

TetraPEGylated canine Hb, [SP (succinimidophenyl)-PEG5K]4-canine-Hb, with PEGylation at its four reactive cysteine residues (a111 and b93) has been prepared and characterized. The hydrodynamic volume and the molecular radius of (SP-PEG5K)4-canine-Hb are intermediate to those of di- and hexaPEGylated human Hb as expected. However, the COP (colloidal osmotic pressure) of tetraPEGylated canine Hb ...

In the sickle cell syndromes, Hb A2 measurements aid in the differential diagnosis of sickle cell anemia from sickle-beta-thalassemia. The purpose of this study is to assess the Hb A2 levels in samples containing sickle hemoglobin (Hb S) by the use of an automated high performance liquid chromatography system (HPLC-Variant beta-thalassemia Short Program). The blood samples analyzed were from in...

Seven patients of Chinese origin who had haemoglobin (Hb) Q-H disease were studied. They were found to have a similar clinical phenotype to that of patients with deletional Hb H disease, who have a near identical genotypic configuration. The complete absence of Hb A in Hb Q-H disease and the similar clinical phenotype to deletional Hb H disease lends support to the observation that Hb Q-Thailan...

By TITUS H. J. HuIS sL&rc AND RALPH C. LEE I N A PREVIOUS PAPER6 we reported the discovery of an abnormal minor hemoglobin in a Negro family ( Fam. S ) in Georgia. This hemoglobin, designated as Hb-Flatbush0 , showed the following properties : ( a ) an electrophoretic mobility ( starch gel electrophoresis at pH 8.1 ) intermediate between Hb-S and Hb-F; ( b ) a chromatographic mobility ( DEAE-ce...

By BENNETT F. HORTON, ROBERT B. THOMPSON, ANDREE M. Dozy, CARL M. NECHTMAN, EVAN NIcHOLs AND TITUS H. J. HUISMAN D URING the past few years, some human hemoglobins have been found composed of one single type of polypeptide chain in contrast to the more common hemoglobins, composed of two types of polypeptide chains. The most important of these are Hb-Bart’s, composed of four of the y chains of ...