{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,1]],"date-time":"2026-09-01T22:59:43Z","timestamp":1788303583947,"version":"build-2803163510"},"reference-count":24,"publisher":"Wiley","issue":"9","license":[{"start":{"date-parts":[[2017,4,18]],"date-time":"2017-04-18T00:00:00Z","timestamp":1492473600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100000054","name":"National Cancer Institute","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100000054","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["HHSN261200800001E"],"award-info":[{"award-number":["HHSN261200800001E"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100006492","name":"Division of Intramural Research, National Institute of Allergy and Infectious Diseases","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100006492","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Allergy"],"published-print":{"date-parts":[[2017,9]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Familial eosinophilia (<jats:styled-content style=\"fixed-case\">FE<\/jats:styled-content>) is a rare autosomal dominant inherited disorder characterized by the presence of lifelong peripheral eosinophilia (&gt;1500\/\u03bcL). Mapped to chromosome 5q31\u2010q33, the genetic cause of <jats:styled-content style=\"fixed-case\">FE<\/jats:styled-content> is unknown, and prior studies have failed to demonstrate a primary abnormality in the eosinophil lineage.<\/jats:p><\/jats:sec><jats:sec><jats:title>Objective<\/jats:title><jats:p>The aim of this study was to identify the cells driving the eosinophilia in <jats:styled-content style=\"fixed-case\">FE<\/jats:styled-content>.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Microarray analysis and real\u2010time <jats:styled-content style=\"fixed-case\">PCR<\/jats:styled-content> were used to examine transcriptional differences in peripheral blood mononuclear cells (<jats:styled-content style=\"fixed-case\">PBMC<\/jats:styled-content>), and in purified cell subsets from affected and unaffected family members belonging to a single large kindred. Cytokine levels in serum and <jats:styled-content style=\"fixed-case\">PBMC<\/jats:styled-content> culture supernatants were assessed by suspension array multiplexed immunoassays.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Whereas <jats:italic><jats:styled-content style=\"fixed-case\">IL<\/jats:styled-content>\u20105<\/jats:italic> m<jats:styled-content style=\"fixed-case\">RNA<\/jats:styled-content> expression was significantly increased in freshly isolated <jats:styled-content style=\"fixed-case\">PBMC<\/jats:styled-content> from affected family members, this was not accompanied by increased m<jats:styled-content style=\"fixed-case\">RNA<\/jats:styled-content> expression of other Th2 cytokines (<jats:italic><jats:styled-content style=\"fixed-case\">IL<\/jats:styled-content>\u20104<\/jats:italic> or <jats:italic><jats:styled-content style=\"fixed-case\">IL<\/jats:styled-content>\u201013<\/jats:italic>). Serum levels of <jats:styled-content style=\"fixed-case\">IL<\/jats:styled-content>\u20105 and <jats:styled-content style=\"fixed-case\">IL<\/jats:styled-content>\u20105 receptor \u03b1, but not IgE, were similarly increased in affected family members. Of note, <jats:italic><jats:styled-content style=\"fixed-case\">IL<\/jats:styled-content>\u20105<\/jats:italic> m<jats:styled-content style=\"fixed-case\">RNA<\/jats:styled-content> expression was significantly increased in purified <jats:styled-content style=\"fixed-case\">CD<\/jats:styled-content>3+ <jats:styled-content style=\"fixed-case\">CD<\/jats:styled-content>4+, <jats:styled-content style=\"fixed-case\">CD<\/jats:styled-content>14+, <jats:styled-content style=\"fixed-case\">CD<\/jats:styled-content>19+, and <jats:styled-content style=\"fixed-case\">ILC<\/jats:styled-content>2 cells from affected family members, as were <jats:styled-content style=\"fixed-case\">IL<\/jats:styled-content>\u20105 protein levels in supernatants from both stimulated <jats:styled-content style=\"fixed-case\">PBMC<\/jats:styled-content> and <jats:styled-content style=\"fixed-case\">ILC<\/jats:styled-content>2 cultures.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>These data are consistent with the hypothesis that the eosinophilia in <jats:styled-content style=\"fixed-case\">FE<\/jats:styled-content> is secondary to dysregulation of <jats:styled-content style=\"fixed-case\">IL<\/jats:styled-content>\u20105 production in <jats:styled-content style=\"fixed-case\">PBMC<\/jats:styled-content> (and their component subsets).<\/jats:p><\/jats:sec>","DOI":"10.1111\/all.13146","type":"journal-article","created":{"date-parts":[[2017,2,22]],"date-time":"2017-02-22T23:06:49Z","timestamp":1487804809000},"page":"1338-1345","source":"Crossref","is-referenced-by-count":21,"title":["Dysregulation of interleukin 5 expression in familial eosinophilia"],"prefix":"10.1111","volume":"72","author":[{"given":"S.","family":"Prakash Babu","sequence":"first","affiliation":[{"name":"Laboratory of Parasitic Diseases National Institute of Allergy and Infectious Diseases National Institutes of Health Bethesda MD USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Y.\u2010Y. K.","family":"Chen","sequence":"additional","affiliation":[{"name":"Laboratory of Parasitic Diseases National Institute of Allergy and Infectious Diseases National Institutes of Health Bethesda MD USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"S.","family":"Bonne\u2010Annee","sequence":"additional","affiliation":[{"name":"Laboratory of Parasitic Diseases National Institute of Allergy and Infectious Diseases National Institutes of Health Bethesda MD USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J.","family":"Yang","sequence":"additional","affiliation":[{"name":"Laboratory of Human Retrovirology and Immunoinformatics Applied and Developmental Research Directorate Leidos Biomedical Research, Inc. 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