OBJECTIVES The clinical analysis of hemoglobin by ion exchange chromatography can result in ambiguities in identification of the nature of the globin chain present in patient samples. LC/ESI-MS provides rapid and precise determination of globin chain masses. DESIGN AND METHODS Hemolysate of hemoglobin Q-India and hemoglobin S/D/F have been analyzed using ESI-MS. Tandem-MS has been used to est...

Artemia has a complex extracellular hemoglobin of Mr 260,000 comprising two globin chains (Mr 130,000) each of which is a polymer of eight covalently linked domains of Mr 16,000. The primary structure of this polymeric globin was studied to understand how globin folded domains are ordered within a globin chain and, in turn, how the latter associate into a functional hemoglobin molecule. Here we...

Cloning and sequencing of the gamma-globin gene of a sickle cell anemia patient homozygous for the Bantu haplotype has revealed a gene conversion that involves the replacement of an A gamma sequence by a G gamma sequence in the promoter area of the A gamma gene. This event is similar to another gene conversion believed to be responsible for the very high homology between gamma-globin genes, sug...

Inhibition of HbS polymerization is a major target for therapeutic approaches in sickle cell anemia. Toward this goal, initial efforts at pharmacological elevation of fetal hemoglobin (HbF) has shown therapeutic efficacy. In order to identify well-tolerated, novel agents that induce HbF in patients, we developed a high-throughput screening approach based on induction of g-globin gene expression...

Denatured 3H-labelled DNAs containing Xenopus or human globin sequences hybridize to RNA transcripts on a single pair of lateral loops on lampbrush chromosome IX of Triturus cristatus carnifex, and to no other loops on this chromosome or the rest of the complement. However they do so, not because of the globin sequences in the probes, but rather because the plasmids from which the probes were p...

This is a report of novel variant the α1-globin gene—(α1) α51 Gly > Cys (CE9), c.154 GGC TGC, named Hb Mazandaran, which was observed in an Iranian family. gives rise to previously undescribed haemoglobin that undetectable by capillary electrophoresis (CE). detected two cases combination with β-globin mutation, and it does not seem be associated severe haematological abnormalities carriers.

Little is known about the factors influencing the hemoglobin switch in vertebrates during development. Inasmuch as the mammalian conceptus is exposed to changing oxygen tensions in utero, we examined the effect of different oxygen concentrations on b-globin switching. We used an in vitro model of mouse embryogenesis based on the differentiation of blastocyst-derived embryonic stem cells to embr...

DGGE and SSCA. Hum Mutat 1996;7:114–22. 6. Jorge SB, Melo MB, Costa FF, Sonati MF. Screening for mutations in human -globin genes by nonradioactive single-strand conformation polymorphism. Braz J Med Biol Res 2003;36:1471–4. 7. Foglietta E, Deidda G, Graziani B, Modiano G, Bianco I. Detection of -globin gene disorders by a simple PCR methodology [Erratum published in: Haematologica 1996;81:XVI]...

background: xmn-1 polymorphism of y g globin gene ( hbg2 ) is a prominent quantitative trait loci (qtl) in β-thalassemia intermediate (β-ti). in current study, we evaluated frequency of xmn-1 polymorphism and its association with β-globin gene ( hbb ) alleles and hb f level in β-ti patients in sistan and balouchestan province, south-east of iran. methods: 45 β-ti patients were enrolled. hbb gen...

Objective: According to the characteristics of α/β-globin gene imbalance caused by α-globin gene accumulation in β-thalassemia, we designed two lentiviral vectors, that is, while we used the lentiviral to mediate full length human β-globin gene, we introduced the interference fragment of lentiviral α-globin gene to adjust the α/β chain ratio by increasing the expression of β-globin and decreasi...