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dc.contributor.authorHannemann, Ankeen
dc.contributor.authorRees, DCen
dc.contributor.authorTewari, Sen
dc.contributor.authorGibson, Johnen
dc.date.accessioned2015-09-16T10:32:52Z
dc.date.available2015-09-16T10:32:52Z
dc.date.issued2015-09-18en
dc.identifier.citationEBioMedicine 2015, 2(11): 1669–1676. doi:10.1016/j.ebiom.2015.09.026en
dc.identifier.issn2352-3964
dc.identifier.urihttps://www.repository.cam.ac.uk/handle/1810/250588
dc.description.abstractSickle 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
dc.description.sponsorshipThe authors are very grateful to the Medical Research Council and Action Medical Research for financial support.
dc.languageEnglishen
dc.language.isoenen
dc.publisherElsevier
dc.rights.urihttp://creativecommons.org/licenses/by/4.0/
dc.rights.uriCreative Commons Attribution 4.0 International License
dc.subjectSickle cell diseaseen
dc.subjectHbSCen
dc.subjectred cellsen
dc.subjectpotassium permeabilityen
dc.subjectKCl cotransporten
dc.titleCation Homeostasis in Red Cells From Patients With Sickle Cell Disease Heterologous for HbS and HbC (HbSC Genotype)en
dc.typeArticle
dc.description.versionThis is the final version of the article. It was first available from Elsevier via http://dx.doi.org/10.1016/j.ebiom.2015.09.026en
prism.endingPage1676
prism.publicationDate2015en
prism.publicationNameEBioMedicineen
prism.startingPage1669
prism.volume2en
dc.rioxxterms.funderMRC
dcterms.dateAccepted2015-09-15en
rioxxterms.versionofrecord10.1016/j.ebiom.2015.09.026en
rioxxterms.licenseref.urihttp://www.rioxx.net/licenses/all-rights-reserveden
rioxxterms.licenseref.startdate2015-09-18en
dc.contributor.orcidHannemann, Anke [0000-0002-2925-1124]
dc.contributor.orcidGibson, John [0000-0001-6145-9139]
dc.identifier.eissn2352-3964
rioxxterms.typeJournal Article/Reviewen
pubs.funder-project-idMRC (G0901177)


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