Functional defects of dendritic cells in patients with CD40 deficiency

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dc.contributor.authorFontana, S
dc.contributor.authorMoratto, D
dc.contributor.authorMangal, S
dc.contributor.authorDe Francesco, M
dc.contributor.authorVermi, W
dc.contributor.authorFerrari, S
dc.contributor.authorFacchetti, F
dc.contributor.authorKutukculer, N
dc.contributor.authorFiorini, C
dc.contributor.authorDuse, M
dc.contributor.authorDas, PK
dc.contributor.authorNotarangelo, LD
dc.contributor.authorPlebani, A
dc.contributor.authorBadolato, R
dc.date.accessioned2019-10-27T18:37:57Z
dc.date.available2019-10-27T18:37:57Z
dc.date.issued2003
dc.departmentEge Üniversitesien_US
dc.descriptionJoint Meeting of the International-Cytokine-Society/International-Society-for-Interferon-and-CytokineResearch/European-Cytokine-Society/Society-for-Leukocyte-Biology -- OCT 06-10, 2002 -- TURIN, ITALYen_US
dc.description.abstractWe have recently identified 2 patients with a rare autosomal recessive form of hyper IgM disease, known as HIGM3, caused by mutations in the CD40 gene. These patients had opportunistic infections observed on X-linked hyper IgM syndrome (HIGM), suggesting that the CD40-CD40 ligand interaction is important for promoting T-cell-mediated immunity. To evaluate whether innate immunity signals may substitute CD154 for inducing the maturation of dendritic cells (DCs), we analyzed monocyte-derived DCs in these patients. Monocyte-derived DCs of HIGM3 subjects on ex vivo stimulation with tumor necrosis factor-alpha (TNP-alpha) or lipopolysaccharide (LIPS) combined with interferon-gamma (IFN-gamma) normally express all the markers of mature DCs, such as CD83 and DC-LAMP. However, cell surface levels of HLA-DR in mature DCs are reduced, as is costimulatory activity of these cells for allogeneic naive T cells. In addition, CD40-deficient DCs secrete lower amounts of interieukin-12 (IL-12) but larger quantities of IL-10 than control subjects. Finally, analysis of circulating plasmacytoid DCs demonstrates a normal percentage of this subset in CD40-deficient cells, but IFN-alpha secretion in response to herpes simplex virus 1 (HSV-1) infection is severely reduced in patients. These observations suggest that the severe impairment of DC maturation may contribute to the defect of T-cell-mediated immunity observed in HIGM3 patients. (C) 2003 by The American Society of Hematology.en_US
dc.description.sponsorshipInt Cytokine Soc, Int Soc Interferon Cytokine res, Euro Cytokine Soc, Soc Leukocyte Bioen_US
dc.identifier.doi10.1182/blood-2003-04-1244en_US
dc.identifier.endpage4106en_US
dc.identifier.issn0006-4971
dc.identifier.issue12en_US
dc.identifier.pmid12893749en_US
dc.identifier.startpage4099en_US
dc.identifier.urihttps://doi.org/10.1182/blood-2003-04-1244
dc.identifier.urihttps://hdl.handle.net/11454/36511
dc.identifier.volume102en_US
dc.identifier.wosWOS:000186987200042en_US
dc.identifier.wosqualityQ1en_US
dc.indekslendigikaynakWeb of Scienceen_US
dc.indekslendigikaynakPubMeden_US
dc.language.isoenen_US
dc.publisherAmer Soc Hematologyen_US
dc.relation.ispartofBlooden_US
dc.relation.publicationcategoryMakale - Uluslararası Hakemli Dergi - Kurum Öğretim Elemanıen_US
dc.rightsinfo:eu-repo/semantics/openAccessen_US
dc.titleFunctional defects of dendritic cells in patients with CD40 deficiencyen_US
dc.typeArticleen_US

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