{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,3,27]],"date-time":"2026-03-27T00:55:55Z","timestamp":1774572955396,"version":"3.50.1"},"reference-count":46,"publisher":"Wiley","issue":"2","license":[{"start":{"date-parts":[[2017,7,12]],"date-time":"2017-07-12T00:00:00Z","timestamp":1499817600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"name":"His Majesty Sultan Qaboos's 1000 grants"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Br J Haematol"],"published-print":{"date-parts":[[2017,10]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>Studying different sickle cell genotypes may throw light on the pathogenesis of sickle cell disease (<jats:styled-content style=\"fixed-case\">SCD<\/jats:styled-content>). Here, the clinical profile, red cell sickling and K<jats:sup>+<\/jats:sup> permeability in 29 <jats:styled-content style=\"fixed-case\">SCD<\/jats:styled-content> patients (15 patients with severe disease and 14 with a milder form) of HbA\/S\u2010Oman genotype were analysed. The super sickling nature of this Hb variant was confirmed. The red cell membrane permeability to K<jats:sup>+<\/jats:sup> was markedly abnormal with elevated activities of P<jats:sub>sickle<\/jats:sub>, Gardos channel and <jats:styled-content style=\"fixed-case\">KC<\/jats:styled-content>l cotransporter (<jats:styled-content style=\"fixed-case\">KCC<\/jats:styled-content>). Results were consistent with Ca<jats:sup>2+<\/jats:sup> entry and Mg<jats:sup>2+<\/jats:sup> loss via P<jats:sub>sickle<\/jats:sub> stimulating Gardos channel and <jats:styled-content style=\"fixed-case\">KCC<\/jats:styled-content> activities. The abnormal red cell behaviour was similar to that in the commonest genotype of <jats:styled-content style=\"fixed-case\">SCD<\/jats:styled-content>, Hb<jats:styled-content style=\"fixed-case\">SS<\/jats:styled-content>, in which the level of mutated Hb is considerably higher. Although activities of all three K<jats:sup>+<\/jats:sup> transporters also correlated with the level of HbS\u2010Oman, there was no association between transport phenotype and disease severity. The super sickling behaviour of HbS\u2010Oman may obviate the need for solute loss and red cell dehydration to encourage Hb polymerisation, required in other <jats:styled-content style=\"fixed-case\">SCD<\/jats:styled-content> genotypes. Disease severity was reduced by concurrent \u03b1 thalassaemia, as observed in other <jats:styled-content style=\"fixed-case\">SCD<\/jats:styled-content> genotypes, and represents an obvious genetic marker for prognostic tests of severity in young <jats:styled-content style=\"fixed-case\">SCD<\/jats:styled-content> patients of the HbA\/S\u2010Oman genotype.<\/jats:p>","DOI":"10.1111\/bjh.14851","type":"journal-article","created":{"date-parts":[[2017,7,12]],"date-time":"2017-07-12T11:22:55Z","timestamp":1499858575000},"page":"256-265","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":7,"title":["The super sickling haemoglobin HbS\u2010Oman: a study of red cell sickling, K<sup>+<\/sup> permeability and associations with disease severity in patients heterozygous for HbA and HbS\u2010Oman (HbA\/S\u2010Oman genotype)"],"prefix":"10.1111","volume":"179","author":[{"given":"Halima W. 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