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Lipid Antigen Presentation by CD1b and CD1d in Lysosomal Storage Disease Patients

2019Journal article Open access
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dc.contributor.authorPereira, C.
dc.contributor.authorPérez-Cabezas, B.
dc.contributor.authorRibeiro, H.
dc.contributor.authorMaia, M.
dc.contributor.authorCardoso, M.
dc.contributor.authorDias, A.
dc.contributor.authorAzevedo, O.
dc.contributor.authorFerreira, M.
dc.contributor.authorGarcia, P.
dc.contributor.authorRodrigues, E.
dc.contributor.authorCastro-Chaves, P.
dc.contributor.authorMartins, Esmeralda
dc.contributor.authorAguiar, P.
dc.contributor.authorPineda, M.
dc.contributor.authorAmraoui, Y.
dc.contributor.authorFecarotta, S.
dc.contributor.authorLeão-Teles, E.
dc.contributor.authorDeng, S.
dc.contributor.authorSavage, P.
dc.contributor.authorMacedo, M.
dc.date.accessioned2020-05-12T09:52:06Z
dc.date.available2020-05-12T09:52:06Z
dc.date.issued2019
dc.description.abstractThe lysosome has a key role in the presentation of lipid antigens by CD1 molecules. While defects in lipid antigen presentation and in invariant Natural Killer T (iNKT) cell response were detected in several mouse models of lysosomal storage diseases (LSD), the impact of lysosomal engorgement in human lipid antigen presentation is poorly characterized. Here, we analyzed the capacity of monocyte-derived dendritic cells (Mo-DCs) from Fabry, Gaucher, Niemann Pick type C and Mucopolysaccharidosis type VI disease patients to present exogenous antigens to lipid-specific T cells. The CD1b- and CD1d-restricted presentation of lipid antigens by Mo-DCs revealed an ability of LSD patients to induce CD1-restricted T cell responses within the control range. Similarly, freshly isolated monocytes from Fabry and Gaucher disease patients had a normal ability to present α-Galactosylceramide (α-GalCer) antigen by CD1d. Gaucher disease patients' monocytes had an increased capacity to present α-Gal-(1-2)-αGalCer, an antigen that needs internalization and processing to become antigenic. In summary, our results show that Fabry, Gaucher, Niemann Pick type C, and Mucopolysaccharidosis type VI disease patients do not present a decreased capacity to present CD1d-restricted lipid antigens. These observations are in contrast to what was observed in mouse models of LSD. The percentage of total iNKT cells in the peripheral blood of these patients is also similar to control individuals. In addition, we show that the presentation of exogenous lipids that directly bind CD1b, the human CD1 isoform with an intracellular trafficking to the lysosome, is normal in these patients.pt_PT
dc.description.sponsorshipThis work was financed by Gaucher Generation Program, supported by Sanofi-Genzyme and by Norte-01-0145-FEDER-000012—Structured program on bioengineered therapies for infectious diseases and tissue regeneration, supported by the Norte Portugal Regional Operational Programme (NORTE 2020), under the PORTUGAL 2020 Partnership Agreement, through the European Regional Development Fund (FEDER). BP-C was temporally supported by a grant from the American Portuguese Biomedical Research Fund (APBRF).pt_PT
dc.description.versioninfo:eu-repo/semantics/publishedVersionpt_PT
dc.identifier.citationPereira CS, Pérez-Cabezas B, Ribeiro H, et al. Lipid Antigen Presentation by CD1b and CD1d in Lysosomal Storage Disease Patients. Front Immunol. 2019;10:1264. Published 2019 Jun 4. doi:10.3389/fimmu.2019.01264pt_PT
dc.identifier.doi10.3389/fimmu.2019.01264pt_PT
dc.identifier.issn1664-3224
dc.identifier.urihttp://hdl.handle.net/10400.16/2380
dc.language.isoengpt_PT
dc.peerreviewedyespt_PT
dc.publisherFrontiers Mediapt_PT
dc.relation.publisherversionhttps://www.frontiersin.org/articles/10.3389/fimmu.2019.01264/fullpt_PT
dc.rights.urihttp://creativecommons.org/licenses/by/4.0/pt_PT
dc.subjectCD1bpt_PT
dc.subjectCD1dpt_PT
dc.subjectdendritic cellspt_PT
dc.subjectlipid antigen presentationpt_PT
dc.subjectlysosomal storage diseasespt_PT
dc.subjectmonocytespt_PT
dc.subjectnatural killer T cellspt_PT
dc.titleLipid Antigen Presentation by CD1b and CD1d in Lysosomal Storage Disease Patientspt_PT
dc.typejournal article
dspace.entity.typePublication
oaire.citation.conferencePlaceSwitzerlandpt_PT
oaire.citation.startPage1264pt_PT
oaire.citation.titleFrontiers in immunologypt_PT
oaire.citation.volume10pt_PT
person.familyNameMartins
person.givenNameEsmeralda
person.identifier.ciencia-idBF1C-DAE5-FAEA
person.identifier.orcid0000-0002-9247-9391
rcaap.rightsopenAccesspt_PT
rcaap.typearticlept_PT
relation.isAuthorOfPublication7f4a528c-bf0f-4895-ac98-4353fe38c68a
relation.isAuthorOfPublication.latestForDiscovery7f4a528c-bf0f-4895-ac98-4353fe38c68a

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