1 The structure and function of normal and abnormal haemoglobins

Huisman, T. H. J.

doi:10.1016/s0950-3536(05)80064-2

Cited by 50 publications

(29 citation statements)

References 59 publications

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“…THE SICKLE CELL ANEMIAS are genetic diseases resulting from nucleotide changes in the gene for the ␤-chain of the adult hemoglobin (HbA) (19). This results in the production of distinct functional hemoglobin variants that aggregate or polymerize when deoxygenated, resulting in decreased cellular flexibility and decreased red cell survival (29).…”

mentioning

confidence: 99%

In vivo rates of erythrocyte glutathione synthesis in adults with sickle cell disease

Reid

Badaloo²,

Forrester³

et al. 2006

American Journal of Physiology-Endocrinology and Metabolism

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Reid, Marvin, Asha Badaloo, Terrence Forrester, and Farook Jahoor. In vivo rates of erythrocyte glutathione synthesis in adults with sickle cell disease. Am J Physiol Endocrinol Metab 291: E73-E79, 2006. First published January 24, 2006 doi:10.1152/ajpendo.00287.2005.-Despite reports of lower GSH concentration in sickle cell disease (SCD), the in vivo kinetic mechanism(s) responsible for GSH deficiency is unknown. To determine whether suppressed synthesis was responsible for the lower erythrocyte GSH concentration, we used a primed intermittent infusion of [ 2 H2]glycine to measure erythrocyte GSH synthesis in vivo in 23 individuals with homozygous ␤ s SCD and 8 healthy controls. Erythrocyte cysteine concentration, the rate-limiting precursor for GSH synthesis, plasma markers of oxidant damage, and dietary intakes of energy and protein were also measured. Compared with values of controls, SCD subjects had significantly lower erythrocyte GSH (P Ͻ 0.04) and cysteine concentrations (P Ͻ 0.004) but significantly faster fractional rates of GSH synthesis (P Ͻ 0.02). The absolute rates of GSH synthesis in SCD subjects compared with control subjects was greater by ϳ57% (P ϭ 0.062). However, the concentrations of markers of oxidative damage, plasma derivatives of reactive oxygen metabolites, plasma nitrotyrosine, urinary isoprostane-to-creatinine ratio, and GSH-to-GSSG ratio, as well as dietary intakes of energy, protein, and GSH precursor amino acids, were not different between SCD subjects and controls. The findings of this study suggest that the lower erythrocyte GSH of SCD patients is not due to suppressed synthesis or impaired regeneration but rather to increased consumption. In addition, the lower erythrocyte cysteine concentration plus the faster rate of GSH synthesis strongly suggest that the endogenous cysteine supply is not sufficient to meet all anabolic demands; hence, cysteine may be a conditionally essential amino acid in individuals with SCD.

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mentioning

confidence: 99%

In vivo rates of erythrocyte glutathione synthesis in adults with sickle cell disease

Reid

Badaloo²,

Forrester³

et al. 2006

American Journal of Physiology-Endocrinology and Metabolism

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“…68 Thus, after the two introns are spliced out, two EJCs might be assembled on the transcript. 69,70 A b-globin NMD-behavior corroborating the "50-55 nt boundary rule" has been described.…”

Section: When General Rules For Nmd Are Not Obeyed: Example Of the Nomentioning

confidence: 99%

Control of human -globin mRNA stability and its impact on beta-thalassemia phenotype

Peixeiro¹,

Silva²,

Romão³

2011

Haematologica

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tion of aberrant b-globin mRNAs. In addition, we emphasize the importance of these pathways in modulating the severity of the b-thalassemia phenotype.Key words: mRNA stability, mRNA quality control, nonsense-mediated mRNA decay (NMD), beta-globin, beta-thalassemia, clinical phenotype.Citation: Peixeiro I, Silva AL and Romão L. Control of human beta-globin mRNA stability and its impact on beta-thalassemia phenotype. Haematologica 2011;96(6):905-913. doi:10.3324/haematol.2010 This is an open-access paper. ABSTRACTsense codons) or affect the termination codon, triggering accelerated mRNA decay, have also been described.

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“…Assim, é possível considerar que o processo oxidativo da Hb S depende de processos biológicos que ocorrem com especificidades individuais. Entre esses processos destacam-se a capacidade antioxidativa do eritrócito, o funcionamento das bombas de Na + , K + e Ca ++ , a oxigenação celular e a elevação da Hb fetal, principalmente (1,5,6) . Nossos resultados podem ser embasados em evidências de que, na anemia falciforme, os mecanismos redutores dos eritrócitos contra as lesões oxidativas estão alterados pela diminuição das atividades da glutationa peroxidase (GPx) e da catalase, pelo aumento da concentração de superóxido dismutase (SOD) e pela redução dos níveis de vitamina E no plasma e na membrana eritrocitária (1,5) .…”

Section: Análises Descritivas Dos Valores Deunclassified

Avaliação dos produtos da degradação oxidativa da Hb S nos genótipos SS, SF (S/beta0 talassemia) e AS, em comparação com hemoglobinas normais

Naoum¹,

Souza²

2004

J. Bras. Patol. Med. Lab.

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SF>AS>AA. É importante destacar que as análises indicaram que a simples presença de Hb S no eritrócito, como é o caso do genótipo AS, é capaz de causar elevação da concentração de metemoglobina em 52,62% das amostras analisadas e de induzir a precipitação de corpos de Heinz em 73,68% dos casos estudados. Explicações referentes aos processos oxidativos e redutores das hemoglobinas estudadas são apresentados no texto. Destaca-se, entre os resultados apresentados, a identificação por meio de eletroforese em agarose alcalina da fração de globina alfa-livre em todas as amostras do genótipo SF provenientes de pessoas com Hb S/beta0 talassemia. É proposto um esquema para explicar a origem da globina alfa-livre, especialmente para o genótipo S/beta0 talassemia, e a importância da sua identificação no diagnóstico laboratorial de Hb S/beta0 talassemia.]]>

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1 The structure and function of normal and abnormal haemoglobins

Cited by 50 publications

References 59 publications

In vivo rates of erythrocyte glutathione synthesis in adults with sickle cell disease

In vivo rates of erythrocyte glutathione synthesis in adults with sickle cell disease

Control of human -globin mRNA stability and its impact on beta-thalassemia phenotype

Avaliação dos produtos da degradação oxidativa da Hb S nos genótipos SS, SF (S/beta0 talassemia) e AS, em comparação com hemoglobinas normais

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