Potassium permeability in β-thalassemia minor red blood cells

Gunn, Robert B.; Silvers, David N.; Rosse, Wendell F.

doi:10.1172/jci106895

Cited by 53 publications

(13 citation statements)

References 22 publications

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“…However, it is unlikely that free globin chains can account for the high Na+ fluxes since the induction of Heinz bodies in erythrocytes by acetylphenylhydrazine has been shown to selectively increase K+ permeability with little effect on Na+ movements (25). Even when the K+ permeability of thalassemia minor erythrocytes is increased following metabolic depletion no change in Na+ permeability occurs (26). Moreover, globin chain precipitation in thalassemics cannot explain the observed increase in the density of cation pumps in the erythrocyte.…”

Section: Methodsmentioning

confidence: 91%

Increased Erythrocyte Cation Permeability in Thalassemia and Conditions of Marrow Stress

Wiley¹

1981

J. Clin. Invest.

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Section: Methodsmentioning

confidence: 91%

Increased Erythrocyte Cation Permeability in Thalassemia and Conditions of Marrow Stress

Wiley¹

1981

J. Clin. Invest.

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“…On the other hand, the Tm-RBCs exhibit lower aggregability [79] compared to the non-Tm-RBCs, while the altered ion homeostasis [78] leads to higher intracellular K + but loss of osmotically active intracellular components and cell shrinkage [80]. As a result of high surface-to-volume ratio [81], the Tm-RBCs are resistant to osmotic lysis [80].…”

Section: Rbc Modifications In Heterozygous Carriers Of Beta-thalassemmentioning

confidence: 99%

Donor‐variation effect on red blood cell storage lesion: A close relationship emerges

Tzounakas¹,

Kriebardis

Papassideri³

et al. 2016

Proteomics Clinical Apps

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Although the molecular pathways leading to the progressive deterioration of stored red blood cells (RBC storage lesion) and the clinical relevance of storage-induced changes remain uncertain, substantial donor-specific variability in RBC performance during storage, and posttransfusion has been established ("donor-variation effect"). In-bag hemolysis and numerous properties of the RBC units that may affect transfusion efficacy have proved to be strongly donor-specific. Donor-variation effect may lead to the production of highly unequal blood labile products even when similar storage strategy and duration are applied. Genetic, undiagnosed/subclinical medical conditions and lifestyle factors that affect RBC characteristics at baseline, including RBC lifespan, energy metabolism, and sensitivity to oxidative stress, are all likely to influence the storage capacity of individual donors' cells, although not evident by the donor's health or hematological status at blood donation. Consequently, baseline characteristics of the donors, such as membrane peroxiredoxin-2 and serum uric acid concentration, have been proposed as candidate biomarkers of storage quality. This review article focuses on specific factors that might contribute to the donor-variation effect and emphasizes the emerging need for using omics-based technologies in association with in vitro and in vivo transfusion models and clinical trials to discover biomarkers of storage quality and posttransfusion recovery in donor blood.

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“…Similarly, a primitive abnormality of membrane permeability to cations (condi tion 4) can be suspected but not demon strated; a similar increase of permeability to cations, ascribed to membrane abnormali ties, has been reported in red cells from Cooley's anaemia [Nathan et al, 1969], heterozygous beta-thalassaemia [Gunn et al, 1972;Knox-Macaulay and Weatherall, 1974;Vettore et al, 1974], and iron defi ciency anaemia [Chapman et al, 1973;Falezza et al, 1977].…”

Section: Discussionmentioning

confidence: 89%

Erythrocyte Cation Content, Globin Chain Synthesis and Glucose Metabolism in Dysmyelopoietic Syndromes

Cetto¹,

Vettore²,

Matteis³

et al. 1982

Acta Haematol

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Red blood cells (RBC) from 13 patients suffering from dysmyelopoietic syndromes (DMPS) have been studied. About half of these subjects showed an abnormal Na⁺ and K⁺ leakage of their RBC, which were incubated at 37 °C for 24 h. The mean rate of the glycolytic pathway increased significantly and the glycolysis values correlated well with the Na⁺ gain. Moreover, 9 out of the 13 patients showed an abnormal biosynthetic ratio of haemoglobin chains in their reticulocytes. Since the multiple defects in DMPS erythrocytes do not seem clearly linked by cause-effect relationships, they probably derive from several cooperating factors in pathological erythroid precursors, leading to RBC membrane damage.

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Potassium permeability in β-thalassemia minor red blood cells

Cited by 53 publications

References 22 publications

Increased Erythrocyte Cation Permeability in Thalassemia and Conditions of Marrow Stress

Increased Erythrocyte Cation Permeability in Thalassemia and Conditions of Marrow Stress

Donor‐variation effect on red blood cell storage lesion: A close relationship emerges

Erythrocyte Cation Content, Globin Chain Synthesis and Glucose Metabolism in Dysmyelopoietic Syndromes

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