Lipid antigen presentation by CD1b and CD1d in lysosomal storage disease patients
Autor(a) principal: | |
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Data de Publicação: | 2019 |
Outros Autores: | , , , , , , , , , , , , , , , , , , |
Tipo de documento: | Artigo |
Idioma: | eng |
Título da fonte: | Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
Texto Completo: | https://hdl.handle.net/10216/136361 |
Resumo: | The 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 a-Galactosylceramide (a-GalCer) antigen by CD1d. Gaucher disease patients' monocytes had an increased capacity to present a-Gal-(1-2)-aGalCer, 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. |
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Lipid antigen presentation by CD1b and CD1d in lysosomal storage disease patientsCD1bCD1dDendritic cellsLipid antigen presentationLysosomal storage diseasesMonocytesNatural killer T cellsThe 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 a-Galactosylceramide (a-GalCer) antigen by CD1d. Gaucher disease patients' monocytes had an increased capacity to present a-Gal-(1-2)-aGalCer, 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.Frontiers Media20192019-01-01T00:00:00Zinfo:eu-repo/semantics/publishedVersioninfo:eu-repo/semantics/articleapplication/pdfhttps://hdl.handle.net/10216/136361eng1664-322410.3389/fimmu.2019.01264Pereira, CSPerez-Cabezas, BRibeiro, HMaia, MCardoso, MDias, AFAzevedo, OFerreira, MGarcia, PRodrigues, ECastro-Chaves, PMartins, EAguiar, PPineda, MAmraoui, YFecarotta, SLeão-Teles, EDeng, SSavage, PMacedo, MFinfo:eu-repo/semantics/openAccessreponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos)instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãoinstacron:RCAAP2023-11-29T14:01:01Zoai:repositorio-aberto.up.pt:10216/136361Portal AgregadorONGhttps://www.rcaap.pt/oai/openaireopendoar:71602024-03-19T23:52:31.440675Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informaçãofalse |
dc.title.none.fl_str_mv |
Lipid antigen presentation by CD1b and CD1d in lysosomal storage disease patients |
title |
Lipid antigen presentation by CD1b and CD1d in lysosomal storage disease patients |
spellingShingle |
Lipid antigen presentation by CD1b and CD1d in lysosomal storage disease patients Pereira, CS CD1b CD1d Dendritic cells Lipid antigen presentation Lysosomal storage diseases Monocytes Natural killer T cells |
title_short |
Lipid antigen presentation by CD1b and CD1d in lysosomal storage disease patients |
title_full |
Lipid antigen presentation by CD1b and CD1d in lysosomal storage disease patients |
title_fullStr |
Lipid antigen presentation by CD1b and CD1d in lysosomal storage disease patients |
title_full_unstemmed |
Lipid antigen presentation by CD1b and CD1d in lysosomal storage disease patients |
title_sort |
Lipid antigen presentation by CD1b and CD1d in lysosomal storage disease patients |
author |
Pereira, CS |
author_facet |
Pereira, CS Perez-Cabezas, B Ribeiro, H Maia, M Cardoso, M Dias, AF Azevedo, O Ferreira, M Garcia, P Rodrigues, E Castro-Chaves, P Martins, E Aguiar, P Pineda, M Amraoui, Y Fecarotta, S Leão-Teles, E Deng, S Savage, P Macedo, MF |
author_role |
author |
author2 |
Perez-Cabezas, B Ribeiro, H Maia, M Cardoso, M Dias, AF Azevedo, O Ferreira, M Garcia, P Rodrigues, E Castro-Chaves, P Martins, E Aguiar, P Pineda, M Amraoui, Y Fecarotta, S Leão-Teles, E Deng, S Savage, P Macedo, MF |
author2_role |
author author author author author author author author author author author author author author author author author author author |
dc.contributor.author.fl_str_mv |
Pereira, CS Perez-Cabezas, B Ribeiro, H Maia, M Cardoso, M Dias, AF Azevedo, O Ferreira, M Garcia, P Rodrigues, E Castro-Chaves, P Martins, E Aguiar, P Pineda, M Amraoui, Y Fecarotta, S Leão-Teles, E Deng, S Savage, P Macedo, MF |
dc.subject.por.fl_str_mv |
CD1b CD1d Dendritic cells Lipid antigen presentation Lysosomal storage diseases Monocytes Natural killer T cells |
topic |
CD1b CD1d Dendritic cells Lipid antigen presentation Lysosomal storage diseases Monocytes Natural killer T cells |
description |
The 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 a-Galactosylceramide (a-GalCer) antigen by CD1d. Gaucher disease patients' monocytes had an increased capacity to present a-Gal-(1-2)-aGalCer, 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. |
publishDate |
2019 |
dc.date.none.fl_str_mv |
2019 2019-01-01T00:00:00Z |
dc.type.status.fl_str_mv |
info:eu-repo/semantics/publishedVersion |
dc.type.driver.fl_str_mv |
info:eu-repo/semantics/article |
format |
article |
status_str |
publishedVersion |
dc.identifier.uri.fl_str_mv |
https://hdl.handle.net/10216/136361 |
url |
https://hdl.handle.net/10216/136361 |
dc.language.iso.fl_str_mv |
eng |
language |
eng |
dc.relation.none.fl_str_mv |
1664-3224 10.3389/fimmu.2019.01264 |
dc.rights.driver.fl_str_mv |
info:eu-repo/semantics/openAccess |
eu_rights_str_mv |
openAccess |
dc.format.none.fl_str_mv |
application/pdf |
dc.publisher.none.fl_str_mv |
Frontiers Media |
publisher.none.fl_str_mv |
Frontiers Media |
dc.source.none.fl_str_mv |
reponame:Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) instname:Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação instacron:RCAAP |
instname_str |
Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação |
instacron_str |
RCAAP |
institution |
RCAAP |
reponame_str |
Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
collection |
Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) |
repository.name.fl_str_mv |
Repositório Científico de Acesso Aberto de Portugal (Repositórios Cientìficos) - Agência para a Sociedade do Conhecimento (UMIC) - FCT - Sociedade da Informação |
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1799135845631918080 |