{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2024,6,9]],"date-time":"2024-06-09T08:40:03Z","timestamp":1717922403728},"reference-count":26,"publisher":"Wiley","issue":"9","license":[{"start":{"date-parts":[[2015,5,29]],"date-time":"2015-05-29T00:00:00Z","timestamp":1432857600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"NIH","award":["2R01 DK074720","R01HL112788","RO1 AI44970"],"award-info":[{"award-number":["2R01 DK074720","R01HL112788","RO1 AI44970"]}]},{"name":"Stella and Henry Endowment"},{"name":"Maestro","award":["2011\/02\/A\/NZ4\/00035"],"award-info":[{"award-number":["2011\/02\/A\/NZ4\/00035"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J Cellular Molecular Medi"],"published-print":{"date-parts":[[2015,9]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:p>The glycolipid glycosylphosphatidylinositol anchor (<jats:styled-content style=\"fixed-case\">GPI<\/jats:styled-content>\u2010A) plays an important role in lipid raft formation, which is required for proper expression on the cell surface of two inhibitors of the complement cascade,<jats:styled-content style=\"fixed-case\">CD<\/jats:styled-content>55 and<jats:styled-content style=\"fixed-case\">CD<\/jats:styled-content>59. The absence of these markers from the surface of blood cells, including erythrocytes, makes the cells susceptible to complement lysis, as seen in patients suffering from paroxysmal nocturnal haemoglobinuria (<jats:styled-content style=\"fixed-case\">PNH<\/jats:styled-content>). However, the explanation for why<jats:styled-content style=\"fixed-case\">PNH<\/jats:styled-content>\u2010affected hematopoietic stem\/progenitor cells (<jats:styled-content style=\"fixed-case\">HSPC<\/jats:styled-content>s) expand over time in<jats:styled-content style=\"fixed-case\">BM<\/jats:styled-content>is still unclear. Here, we propose an explanation for this phenomenon and provide evidence that a defect in lipid raft formation in<jats:styled-content style=\"fixed-case\">HSPC<\/jats:styled-content>s leads to defective<jats:styled-content style=\"fixed-case\">CXCR<\/jats:styled-content>4\u2010 and<jats:styled-content style=\"fixed-case\">VLA<\/jats:styled-content>\u20104\u2010mediated retention of these cells in<jats:styled-content style=\"fixed-case\">BM<\/jats:styled-content>. In support of this possibility,<jats:styled-content style=\"fixed-case\">BM<\/jats:styled-content>\u2010isolated<jats:styled-content style=\"fixed-case\">CD<\/jats:styled-content>34<jats:sup>+<\/jats:sup>cells from<jats:styled-content style=\"fixed-case\">PNH<\/jats:styled-content>patients show a defect in the incorporation of<jats:styled-content style=\"fixed-case\">CXCR<\/jats:styled-content>4 and<jats:styled-content style=\"fixed-case\">VLA<\/jats:styled-content>\u20104 into membrane lipid rafts, respond weakly to<jats:styled-content style=\"fixed-case\">SDF<\/jats:styled-content>\u20101 stimulation, and show defective adhesion to fibronectin. Similar data were obtained with the<jats:styled-content style=\"fixed-case\">GPI<\/jats:styled-content>\u2010A<jats:sup>\u2212<\/jats:sup>Jurkat cell line. Moreover, we also report that chimeric mice transplanted with<jats:styled-content style=\"fixed-case\">CD<\/jats:styled-content>55<jats:sup>\u2212\/\u2212<\/jats:sup>\u00a0<jats:styled-content style=\"fixed-case\">CD<\/jats:styled-content>59<jats:sup>\u2212\/\u2212<\/jats:sup><jats:styled-content style=\"fixed-case\">BM<\/jats:styled-content>cells but with proper<jats:styled-content style=\"fixed-case\">GPI<\/jats:styled-content>\u2010A expression do not expand over time in transplanted hosts. On the basis of these findings, we propose that a defect in lipid raft formation in<jats:styled-content style=\"fixed-case\">PNH<\/jats:styled-content>\u2010mutated<jats:styled-content style=\"fixed-case\">HSPC<\/jats:styled-content>s makes these cells more mobile, so that they expand and out\u2010compete normal<jats:styled-content style=\"fixed-case\">HSPC<\/jats:styled-content>s from their<jats:styled-content style=\"fixed-case\">BM<\/jats:styled-content>niches over time.<\/jats:p>","DOI":"10.1111\/jcmm.12605","type":"journal-article","created":{"date-parts":[[2015,5,29]],"date-time":"2015-05-29T17:23:53Z","timestamp":1432920233000},"page":"2193-2201","source":"Crossref","is-referenced-by-count":8,"title":["Further evidence that paroxysmal nocturnal haemoglobinuria is a disorder of defective cell membrane lipid rafts"],"prefix":"10.1111","volume":"19","author":[{"given":"Mariusz Z.","family":"Ratajczak","sequence":"first","affiliation":[{"name":"Stem Cell Institute at James Graham 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