{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,27]],"date-time":"2026-01-27T15:53:55Z","timestamp":1769529235094,"version":"3.49.0"},"reference-count":15,"publisher":"Wiley","issue":"1","license":[{"start":{"date-parts":[[2017,2,1]],"date-time":"2017-02-01T00:00:00Z","timestamp":1485907200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000050","name":"National Heart, Lung, and Blood Institute","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100000050","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Br J Haematol"],"published-print":{"date-parts":[[2017,4]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>The aetiology of paroxysmal nocturnal haemoglobinuria (<jats:styled-content style=\"fixed-case\">PNH<\/jats:styled-content>) is a somatic mutation in the X\u2010linked phosphatidylinositol glycan class A gene (<jats:italic><jats:styled-content style=\"fixed-case\">PIGA<\/jats:styled-content><\/jats:italic>), resulting in global deficiency of glycosyl phosphatidylinositol\u2013anchored proteins (<jats:styled-content style=\"fixed-case\">GPI<\/jats:styled-content>\u2010<jats:styled-content style=\"fixed-case\">AP<\/jats:styled-content>s). This study applied <jats:styled-content style=\"fixed-case\">RNA<\/jats:styled-content>\u2010sequencing to examine functional effects of the <jats:italic><jats:styled-content style=\"fixed-case\">PIGA<\/jats:styled-content><\/jats:italic> mutation in human granulocytes. <jats:italic><jats:styled-content style=\"fixed-case\">CXCR<\/jats:styled-content>2<\/jats:italic> expression was increased in <jats:styled-content style=\"fixed-case\">GPI<\/jats:styled-content>\u2010<jats:styled-content style=\"fixed-case\">AP<\/jats:styled-content><jats:sup>\u2010<\/jats:sup> compared to <jats:styled-content style=\"fixed-case\">GPI<\/jats:styled-content>\u2010<jats:styled-content style=\"fixed-case\">AP<\/jats:styled-content><jats:sup>+<\/jats:sup> granulocytes. Macrophage migration inhibitory factor, a <jats:styled-content style=\"fixed-case\">CXCR<\/jats:styled-content>2 agonist, was significantly higher in plasma of <jats:styled-content style=\"fixed-case\">PNH<\/jats:styled-content> patients. Nuclear factor\u2010\u03baB phosphorylation was upregulated in <jats:styled-content style=\"fixed-case\">GPI<\/jats:styled-content>\u2010<jats:styled-content style=\"fixed-case\">AP<\/jats:styled-content><jats:sup>\u2212<\/jats:sup> compared with <jats:styled-content style=\"fixed-case\">GPI<\/jats:styled-content>\u2010<jats:styled-content style=\"fixed-case\">AP<\/jats:styled-content><jats:sup>+<\/jats:sup> granulocytes. Our data suggest novel mechanisms in <jats:styled-content style=\"fixed-case\">PNH<\/jats:styled-content>, not obviously predicted by decreased production of the <jats:styled-content style=\"fixed-case\">GPI<\/jats:styled-content> moiety.<\/jats:p>","DOI":"10.1111\/bjh.14502","type":"journal-article","created":{"date-parts":[[2017,2,1]],"date-time":"2017-02-01T16:04:42Z","timestamp":1485965082000},"page":"136-141","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":7,"title":["Whole transcriptome sequencing identifies increased <i><scp>CXCR<\/scp>2<\/i> expression in <scp>PNH<\/scp> granulocytes"],"prefix":"10.1111","volume":"177","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3423-1523","authenticated-orcid":false,"given":"Kohei","family":"Hosokawa","sequence":"first","affiliation":[{"name":"Hematology Branch National Heart, Lung, and Blood Institute (NHLBI) NIH Bethesda MD 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Autoimmunity and Inflammation NIH Bethesda MD USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Danielle M.","family":"Townsley","sequence":"additional","affiliation":[{"name":"Hematology Branch National Heart, Lung, and Blood Institute (NHLBI) NIH Bethesda MD USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Xingmin","family":"Feng","sequence":"additional","affiliation":[{"name":"Hematology Branch National Heart, Lung, and Blood Institute (NHLBI) NIH Bethesda MD USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Neal S.","family":"Young","sequence":"additional","affiliation":[{"name":"Hematology Branch National Heart, Lung, and Blood Institute (NHLBI) NIH Bethesda MD 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