α globin
An erythroid chaperone that facilitates folding of α globin subunits for hemoglobin synthesis
... of α-globin successively toward formation of HbA tetramers (Figure ...free α-globin in erythroid precursors are not known but presumably are much lower, as indicated in Fig- ure ...nascent ...
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Rapid and reliable detection of α-globin copy number variations by quantitative real-time PCR
... complete α-globin gene cluster, including the upstream regulatory element HS-40, was identified in a β-thalassemia carrier with severe hemolytic anemia (Figure ...
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Endothelial cell α globin and its molecular chaperone α hemoglobin–stabilizing protein regulate arteriolar contractility
... EC α-globin deficiency or loss of AHSP (with resultant α-globin defi- ciency) inhibits TDA contractility by increasing NO diffusion to ...
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Original Article β-thalassemia for translating human β-globin gene and RNA interference of α-globin gene expression by lentivirus vectors
... reducing α-globin deposition and balancing the propor- tion of α/β-globin in patients with ...human α- andγ-globin chain and is a good cell model for research on human blood ...
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Genotype phenotype association analysis identifies the role of α globin genes in modulating disease severity of β thalassaemia intermedia in Sri Lanka
... mutations, α globin genotype and copy number and known genetic modifiers of haemoglobin F ...excess α globin ...excess α globin ...had α globin gene ...excess ...
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Processing of α-Globin and ICP0 mRNA in Cells Infected with Herpes Simplex Virus Type 1 ICP27 Mutants
... of ␣-globin pre-mRNA did not correlate with the previously described transrepression function of ICP27, that is, its ability to inhibit expression of intron-bearing reporter genes in co- transfection assays. ...
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ANALYSIS OF A MOUSE α-GLOBIN GENE MUTATION INDUCED BY ETHYLNITROSOUREA
... T h e primary structure of the mutant hemoglobin induced by ethylnitrosourea was analyzed using procedures described to ana- lyze genetic variants of the a-chains of [r] ...
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Polymorphism and Gene Conversion in Mouse α-Globin Haplotypes
... coding regions.. G., 1974 Rodent hemoglobin structure: a comparison of several species of mice. Assignment of orthol- ogous relationships among mammalian a-globin genes by ex- a[r] ...
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Free α Globin Pool in Human Bone Marrow
... of globin chain synthesis in normal patients and in the compensatory synthesis of b-chains that occurs in the bone marrow of patients heterozygous for thalassemia or for Hb ...
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Herpes Simplex Virus ICP27 Induces Cytoplasmic Accumulation of Unspliced Polyadenylated α-Globin Pre-mRNA in Infected HeLa Cells
... exon 1 (5⬘-GAGGGTGGCCTGTGGGTCCGGGCGGGCG AG-3⬘) (Fig. 3A and B). The two more slowly migrating bands present in KOS-infected cells hybridized to the intron 1 probe (Fig. 3A), while all three transcripts hybridized to the ...
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Molecular characterisation of Haemoglobin Constant Spring and Haemoglobin Quong Sze with a Combine-Amplification Refractory Mutation System
... normal α-globin gene sequence; in this reaction, forward primer CS-1 was added to 20 pmol each of reverse primers CS-N (5’-AGGAGGAACGGCTACCGAG- GCTCCAGATTA-3’) and QS-N (5’ CGGTGCT- ...
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Molecular basis of alpha-thalassemia
... no α-globin is produced, infants suffer from the Hb Bart's Hydrops Foetalis ...functional α -globin genes from both ...duplicated α -genes is responsible for a condition which lies ...
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Ineffective Erythropoiesis: Associated Factors and Their Potential as Therapeutic Targets in Beta-Thalassaemia Major
... In β-thal., release of ROS results mainly from two pathological mechanisms: excessive accumulation of the highly oxidant, unpaired α- globin chains and release of highly toxic free iron from damaged cells, ...
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Hematologic Features of Alpha Thalassemia Carriers
... ( α -thal) is relatively common ...alpha globin genes out of four functional ones, with deletions being more common than point ...of α globin ...
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A Large Cohort Study of Genotype and Phenotype Correlations of Beta- Thalassemia in Iranian Population
... Results: A total of 39 different β-globin mutations were identified. Among them, the most common were IVS IInt1 (40.33%) followed by IVS Int5 (9.56%), C30 (7.22%) and Fr8-9(7%). All patients were subjected to ...
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AHSP: a novel hemoglobin helper
... protein α hemoglobin–stabilizing protein (AHSP) was identified and found to specifically bind α-globin, stabilize its structure, and limit the toxic effects of excess α-globin, which ...
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Understanding globin regulation in β thalassemia: it’s as simple as α, β, γ, δ
... into globin polypeptides in the cell cyto- ...of globin chains associated with heme groups (Figure ...balances α-globin synthesis (Figure 1C), which results in the production of adequate ...
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The evolution of haemoglobin gene loci in amniotes
... reptilian α- and β-globin clusters, what are their locations and expression patterns, and it is not possible to deduce the structure of ancestral reptile haemoglobin gene ...reptilian globin gene ...
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Development and validation of a high throughput, closed tube method for the determination of haemoglobin alpha gene (HBA1 and HBA2) numbers by gene ratio assay copy enumeration PCR (GRACE PCR)
... the α-globin genes that affect the 3′ ends of the HBA1 or HBA2 ...the α-globin genes, the GRACE-PCR assay is a simple closed tube technique, which requires no further hands on time after the ...
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Clinical and molecular genetic features of Hb H and AE Bart’s diseases in central Thai children
... absent α -globin mRNA and α -globin chain lead- ing to the α / b globin mRNA ratio being < ...the α / b globin chain synthetic ratio in the range of ...
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