A subject with HbG Philadelphia-HbH disease exhibited an unusually high alpha/beta synthesis ratio; when peripheral blood was tested in vitro on several occasions, ratios of 0.63 - 0.89 were obtained after incubations of 30-120 min. HbH amounted to 5%-8% of the circulating hemoglobin. Rapid destruction of excess newly synthesized beta-globin was demonstrated in kinetic and pulse-chase experimen...

By cloning into Escherichia coli and construction of isogenic mutants of Haemophilus ducreyi, we showed that the hemoglobin receptor (HgbA) is TonB dependent. An E. coli hemA tonB mutant expressing H. ducreyi hgbA grew on low levels of hemoglobin as a source of heme only when an intact H. ducreyi Ton system plasmid was present. In contrast, growth on heme by the E. coli hemA tonB mutant express...

BACKGROUND Infants with congenital heart defects (CHD) are at risk for white matter brain injury. This novel rat pup model characterizes the systemic effects of intravasal cell-free hemoglobin and hyperoxia, hypothesizing that immature endogenous scavenging systems relate to increased vulnerability to conditions present during cardiopulmonary bypass (CPB). METHODS Plasma pharmacokinetics of c...

With the technical assistance of Kaniil Tanrikulu H EMOGLOBIN E has been found with variable frequency among peopies of southeast Asia, such as the Thais,’ ’” the Burmese,2 the Malasians,3 the Indonesians,4 ”4’ the Bengalis,5#{176}’5” and Filipinos,6 and Vcddas of Ceylon.T37h Sporadic cases have been encountered in other countries.6’8 In 1955 an example of the hemoglobin E trait was discovered ...

In an English family living in the county of Norfolk, England, Ager, Lehmann, and Vella (2) have described the occurrence of an electrophoretically fast moving variant of human adult hemoglobin (hemoglobin A). This hemoglobin has been named hemoglobin Norfolk. Gammack et al. (3) have reported that hemoglobin Norfolk contains abnormal ac-peptide chains. By a microscale adaptation of the dissocia...

Hemoglobin H (HbH) disease is the severe nonfatal form of α-thalassemia syndrome. It is usually caused by molecular defects of 3 of 4 α-globin genes (--/-α) which cause α-globin expression to be decreased. HbH disease is rare in Japan. Here, we report on a 6-year-old girl with HbH disease who had profound hypochromatic and microcytic anemia. Analysis of the α-globin genes of the patient's famil...

In an effort to understand the mechanism of radical formation on heme proteins, the formation of radicals on hemoglobin was initiated by reaction with hydrogen peroxide in the presence of the spin trap 5,5-dimethyl-1-pyrroline N-oxide (DMPO). The DMPO nitrone adducts were analyzed by mass spectrometry (MS) and immuno-spin trapping. The spin-trapped protein adducts were then subjected to tryptic...

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The formation of hemoglobin AIc was studied in intact human erythrocytes in vitro. Satisfactory methods were developed for maintaining erythrocytes under physiologic conditions for greater than 8 d with less than 10% hemolysis. Hemoglobin AIc levels were determined chromatographically on erythrocyte hemolysates after removal of reversible components by incubation for 6 h at 37 degree C. Hemoglo...