globin chain
Balanced Globin Chain Synthesis in Hereditary Persistence of Fetal Hemoglobin
... g globin production and increased activity of the remaining b globin ...balanced globin synthesis in the bone ...of globin chain ...balanced globin chain synthesis and the ...
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Globin chain synthesis in the alpha thalassemia syndromes
... hemoglobin H disease, alpha thalassemia trait, and the “silent carrier” state were incubated with leucine- 14 C for definition of relative rates of production of alpha and beta chains in these disorders. The chains were ...
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Synthesis of hemoglobin Gun Hill: increased synthesis of the heme free βGH globin chain and subunit exchange with a free α chain pool
... synthesis of the abnormal b-chains than b A -chains. The ratio of the specific activities of the a-chains of hemoglobin Gun Hill to the a-chains of hemoglobin A was 20: 1. There was evidence of a free pool of a-chains in ...
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Immunostick Test for Detecting ζ-Globin Chains and Screening of the Southeast Asian α-Thalassemia 1 Deletion
... ζ globin chain mAb clone PL3 were bio- tinylated using EZ-Link ™ Sulfo-NHS-LC-Biotin according to manufacturer ...-globin chain or normal ...
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Hematologic and biosynthetic studies in homozygous hemoglobin Constant Spring
... of globin chain synthesis in vitro and the destruction of the excess beta-chains occur unusually ...intracellular globin chain precipitates and free beta-chain pool, homozygous HbCS red ...
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Two missense mutations in the beta globin gene can cause severe beta thalassemia Hemoglobin Medicine Lake (beta 32[B14]leucine >glutamine; 98 [FG5] valine >methionine)
... synthesis with early loss of beta-globin chains. An abnormal unstable hemoglobin, globin chain, or tryptic globin peptide was not present, demonstrating the extreme lability of this novel ...
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Mutant Fetal Hemoglobin Causing Cyanosis in a Newborn
... have a mutant -y-globin chain, leading to a functionally abnormal fetal hemoglobin.. A single amino acid substi- tution was found in a site consistent with known adult M hemoglobins.[r] ...
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alpha Thalassemia caused by an unstable alpha globin mutant
... mutation (CTG to CCG) in one of the genes resulted in a leucine to proline substitution. This paper describes the approach we used to detect the abnormal alpha-globin chain. The chain was identified ...
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The development of mass spectrometry based approaches for the diagnosis of hemoglobinopathies
... of globin chain(s) expression that is ...β globin chains are encoded by a single gene; α globin chains are encoded by two closely linked ...β chain, and four loci encoding the α ...
318
Globin synthesis in fractionated Normoblasts of beta thalassemia heterozygotes
... Globin chain synthesis was examined in erythroid cells of increasing maturity, fractionated from the whole bone marrow of beta-thalassemia heterozygotes by a density gradient centrifugation ...alpha/beta ...
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Mechanism of methaemoglobin breakdown by the lysine-specific gingipain of the periodontal pathogen Porphyromonas gingivalis
... and globin subunits contain numerous lysine residues, oxyhaemoglobin remains refractory to Kgp alone, resulting in the formation of a haemoglobin haemichrome which is relatively stable towards further Kgp ...
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Activation of the human beta globin promoter in K562 cells by DNA sequences 5' to the fetal gamma or embryonic zeta globin genes
... Regulatory sequences of the human fetal gamma-globin gene were studied by constructing composite gamma/beta globin promoters and comparing their function to that of intact beta promoters in human K562 ...
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A Large Cohort Study of Genotype and Phenotype Correlations of Beta- Thalassemia in Iranian Population
... Dedousis et al. 30 have studied on the presence of clinical variability in patients who were homozygote or compound heterozygote for β 0 or β + thalassemia. They have found that rising fetal hemoglobin (HbF) level ...
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Preferential Binding of βS Globin Chains Associated with Stroma in Sickle Cell Disorders
... If preferential binding of Pi chains to tive amounts of labeled globin chains present in the occurs during early erythroid cell development, supernate with globin chains associated with [r] ...
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Defects in DNA and globin messenger RNA in homozygotes for hemoglobin Lepore
... hybridization. The relative amounts of alpha- and Lepore mRNA are similar to the amounts of alpha- and Lepore globin synthesized in intact cells and by isolated mRNA in a cell-free system. It is also demonstrated ...
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An initiation codon mutation (AUG GUG) of the human alpha 1 globin gene Structural characterization and evidence for a mild thalassemic phenotype
... alpha-globin is encoded by two adjacent genes, alpha 1 and alpha 2. Recent evidence suggests that these genes are not equally expressed and that the alpha 2-globin gene encodes the majority of ...
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Molecular basis for nondeletion alpha thalassemia in American blacks Alpha 2(116GAG UAG)
... presence of a nondeletion alpha-thalassemia defect in this family which segregates with the intact alpha alpha gene cluster. An abnormally migrating and highly unstable alpha-globin gene product was demonstrated ...
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The expression of embryonic globin mRNA in a severely anemic mouse model induced by treatment with nitrogen-containing bisphosphonate
... Resident macrophages act as pivotal stromal cells during definitive erythropoiesis. In fact, erythropoiesis was inhib- ited by the elimination of BM resident macrophages in our previous studies [26, 27]. In addition, ...
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The evolution of haemoglobin gene loci in amniotes
... active globin genes (Weatherall, ...embryonic globin genes are expressed during primitive erythropoiesis in the egg yolk, foetal globin genes are expressed in the liver of the foetus, followed by ...
206
Hemoglobin Evanston (alpha 14 Trp Arg) An unstable alpha chain variant expressed as alpha thalassemia
... short-term globin synthesis studies, and from mRNA translation in vitro, suggest that the two types of alpha-chains were synthesized at relatively equal rates, with a major fraction of the newly synthesized ...
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