ArticleEBioMedicine2015
Cation Homeostasis in Red Cells From Patients With Sickle Cell Disease Heterologous for HbS and HbC (HbSC Genotype).
Article in EBioMedicine, 2015. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 22 papers.
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Who cites it
22 citing papers in PubMed, 35 citations in OpenAlex.
- Across species: A comparative perspective on red cell homeostasis and its influence on our understanding of human physiology and disease.British journal of haematology · 2026Review
- A mouse model for hemoglobin SC disease recapitulates characteristic human pathologies.Blood advances · 2025Article
- A tale of 2 mice.Blood advances · 2025Article
- Enhanced PIEZO1 function contributes to the pathogenesis of sickle cell disease.Proceedings of the National Academy of Sciences of the United States of America · 2025Article
- The antisickling agent, 5-hydroxymethyl-2-furfural: Other potential pharmacological applications.Medicinal research reviews · 2024Review
- Article
- The pleiotropic effects of α-thalassemia on HbSS and HbSC sickle cell disease: Reduced erythrocyte cation co-transport activity, serum erythropoietin, and transfusion burden, do not translate into increased survival.American journal of hematology · 2022Article
- Sickle cell nephropathy: A review of novel biomarkers and their potential roles in early detection of renal involvement.World journal of clinical pediatrics · 2022Review
- The protective effect of the spleen in sickle cell patients. A comparative study between patients with asplenia/hyposplenism and hypersplenism.Frontiers in physiology · 2022Article
- Impact of a submaximal mono-articular exercise on the skeletal muscle function of patients with sickle cell disease.European journal of applied physiology · 2021Article
- Is Skeletal Muscle Dysfunction a Limiting Factor of Exercise Functional Capacity in Patients with Sickle Cell Disease?Journal of clinical medicine · 2021Article
- Curative vs targeted therapy for SCD: does it make more sense to address the root cause than target downstream events?Blood advances · 2020Article
- Clinical Diagnosis of Red Cell Membrane Disorders: Comparison of Osmotic Gradient Ektacytometry and Eosin Maleimide (EMA) Fluorescence Test for Red Cell Band 3 (AE1, SLC4A1) Content for Clinical Diagnosis.Frontiers in physiology · 2020Article
- A gain of function variant in PIEZO1 (E756del) and sickle cell disease.Haematologica · 2019Article
- The Effect of Antioxidants on the Properties of Red Blood Cells From Patients With Sickle Cell Anemia.Frontiers in physiology · 2019Article
- Serum Potassium, Sodium, and Chloride Levels in Sickle Cell Disease Patients and Healthy Controls: A Case-Control Study at Korle-Bu Teaching Hospital, Accra.Biomarker insights · 2019Article
- Retinal thickness measurements in sickle cell patients with HbSS and HbSC genotype.Canadian journal of ophthalmology. Journal canadien d'ophtalmologie · 2018Article
- The effect of xanthine oxidase and hypoxanthine on the permeability of red cells from patients with sickle cell anemia.Physiological reports · 2018Article
- Disorders of erythrocyte hydration.Blood · 2017Review
- Early Markers of Sickle Nephropathy in Children With Sickle Cell Anemia Are Associated With Red Cell Cation Transport Activity.HemaSphere · 2017Article
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Authors and funding
4 authors at 2 institutions in 1 country.
Funding
Abstract
Sickle cell disease (SCD) in patients of HbSC genotype is considered similar, albeit milder, to that in homozygous HbSS individuals--but with little justification. In SCD, elevated red cell cation permeability is critical as increased solute loss causes dehydration and encourages sickling. Recently, we showed that the KCl cotransporter (KCC) activity in red cells from HbSC patients correlated significantly with disease severity, but that in HbSS patients did not. Two transporters involved in red cell dehydration, the conductive channels Psickle and the Gardos channel, behaved similarly in red cells from the two genotypes, but were significantly less active in HbSC patients. By contrast, KCC activity was quantitatively greater in HbSC red cells. Results suggest that KCC is likely to have greater involvement in red cell dehydration in HbSC patients, which could explain its association with disease severity in this genotype. This work supports the hypothesis that SCD in HbSC patients is a distinct disease entity to that in HbSS patients. Results suggest the possibility of designing specific treatments of particular benefit to HbSC patients and a rationale for the development of prognostic markers, to inform early treatment of children likely to develop more severe complications of the disease.
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Registered trials
Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.