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DC Field | Value | Language |
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dc.contributor.author | Lee, Danyel | - |
dc.contributor.author | Le Pen, Jérémie | - |
dc.contributor.author | Yatim, Ahmad | - |
dc.contributor.author | Dong, Beihua | - |
dc.contributor.author | Aquino, Yann | - |
dc.contributor.author | Ogishi, Masato | - |
dc.contributor.author | Pescarmona, Rémi | - |
dc.contributor.author | Talouarn, Estelle | - |
dc.contributor.author | Rinchai, Darawan | - |
dc.contributor.author | Zhang, Peng | - |
dc.contributor.author | Perret, Magali | - |
dc.contributor.author | Dalgard, Clifford L. | - |
dc.contributor.author | Bustamante, Jacinta | - |
dc.contributor.author | Puel, Anne | - |
dc.contributor.author | Boisson-Dupuis, Stéphanie | - |
dc.contributor.author | Boisson, Bertrand | - |
dc.contributor.author | Maniatis, Tom | - |
dc.contributor.author | Zhang, Qian | - |
dc.contributor.author | Bastard, Paul | - |
dc.contributor.author | Notarangelo, Luigi D. | - |
dc.contributor.author | Béziat, Vivien | - |
dc.contributor.author | Perez De Diego, Rebeca | - |
dc.contributor.author | Rodriguez Gallego, Carlos | - |
dc.contributor.author | Su, Helen C. | - |
dc.contributor.author | Lifton, Richard P. | - |
dc.contributor.author | Jouanguy, Emmanuelle | - |
dc.contributor.author | Cobat, Aurélie | - |
dc.contributor.author | Alsina, Laia | - |
dc.contributor.author | Keles, Sevgi | - |
dc.contributor.author | Haddad, Elie | - |
dc.contributor.author | Casanova, Jean-Laurent | - |
dc.contributor.author | Abel, Laurent | - |
dc.contributor.author | Belot, Alexandre | - |
dc.contributor.author | Zhang, Shen-Ying | - |
dc.contributor.author | Quintana Murci, Lluis | - |
dc.contributor.author | Silverman, Robert H. | - |
dc.contributor.author | Rice, Charles M. | - |
dc.contributor.author | Liu, Zhiyong | - |
dc.contributor.author | Jordan Garcia, Iolanda | - |
dc.contributor.author | Elmas Bozdemir, Sefika | - |
dc.contributor.author | Bayhan, Gulsum Iclal | - |
dc.contributor.author | Beaufils, Camille | - |
dc.contributor.author | Bizien, Lucy | - |
dc.contributor.author | Bisiaux, Aurelie | - |
dc.contributor.author | Lei, Weite | - |
dc.contributor.author | Hasan, Milena | - |
dc.contributor.author | Chen, Jie | - |
dc.contributor.author | Gaughan, Christina | - |
dc.contributor.author | Asthana, Abhishek | - |
dc.contributor.author | Libri, Valentina | - |
dc.contributor.author | Luna, Joseph M. | - |
dc.contributor.author | Jaffré, Fabrice | - |
dc.contributor.author | Hoffmann, Heinrich H. | - |
dc.contributor.author | Michailidis, Eleftherios | - |
dc.contributor.author | Moreews, Marion | - |
dc.contributor.author | Seeleuthner, Yoann | - |
dc.contributor.author | Bilguvar, Kaya | - |
dc.contributor.author | Mane, Shrikant | - |
dc.contributor.author | Flores, Carlos | - |
dc.contributor.author | Zhang, Yu | - |
dc.contributor.author | Arias, Andrés A. | - |
dc.contributor.author | Bailey, Rasheed | - |
dc.contributor.author | Schlüter, Agatha | - |
dc.contributor.author | Milisavljevic, Baptiste | - |
dc.contributor.author | Bigio, Benedetta | - |
dc.contributor.author | Anton Lopez, Jordi | - |
dc.contributor.author | Le Voyer, Tom | - |
dc.contributor.author | Materna, Marie | - |
dc.contributor.author | Gervais, Adrian | - |
dc.contributor.author | Moncada Velez, Marcela | - |
dc.contributor.author | Pala, Francesca | - |
dc.contributor.author | Lazarov, Tomi | - |
dc.contributor.author | Levy, Romain | - |
dc.contributor.author | Neehus, Anna-Lena | - |
dc.contributor.author | Rosain, Jérémie | - |
dc.contributor.author | Peel, Jessica | - |
dc.contributor.author | Chan, Yi-Hao | - |
dc.contributor.author | Morin, Marie-Paule | - |
dc.contributor.author | Pino Ramírez, Rosa Maria | - |
dc.contributor.author | Belkaya, Serkan | - |
dc.contributor.author | Lorenzo, Lazaro | - |
dc.contributor.author | Delafontaine, Selket | - |
dc.contributor.author | Toubiana, Julie | - |
dc.contributor.author | Bajolle, Fanny | - |
dc.contributor.author | Fumadó, Victoria | - |
dc.contributor.author | DeDiego, Marta L. | - |
dc.contributor.author | Fidouh, Nadhira | - |
dc.contributor.author | Rozenberg, Flore | - |
dc.contributor.author | Pérez Tur, Jordi | - |
dc.contributor.author | Chen, Shuibing | - |
dc.contributor.author | Evans, Todd | - |
dc.contributor.author | Geissmann, Frédéric | - |
dc.contributor.author | Lebon, Pierre | - |
dc.contributor.author | Weiss, Susan R. | - |
dc.contributor.author | Bonnet, Damien | - |
dc.contributor.author | Duval, Xavier | - |
dc.contributor.author | CoV-Contact Cohort | - |
dc.contributor.author | COVID Human Genetic Effort | - |
dc.contributor.author | Pan-Hammarström, Qiang | - |
dc.contributor.author | Planas Obradors, Anna M. | - |
dc.contributor.author | Meyts, Isabelle | - |
dc.contributor.author | Haerynck, Filomeen | - |
dc.contributor.author | Pujol Onofre, Aurora | - |
dc.contributor.author | Sancho Shimizu, Vanessa | - |
dc.date.accessioned | 2023-09-21T14:21:09Z | - |
dc.date.available | 2023-09-21T14:21:09Z | - |
dc.date.issued | 2023-02-10 | - |
dc.identifier.issn | 0036-8075 | - |
dc.identifier.uri | http://hdl.handle.net/2445/202161 | - |
dc.description.abstract | Multisystem inflammatory syndrome in children (MIS-C) is a rare and severe condition that follows benign COVID-19. We report autosomal recessive deficiencies of OAS1, OAS2, or RNASEL in five unrelated children with MIS-C. The cytosolic double-stranded RNA (dsRNA)-sensing OAS1 and OAS2 generate 2'-5'-linked oligoadenylates (2-5A) that activate the single-stranded RNA-degrading ribonuclease L (RNase L). Monocytic cell lines and primary myeloid cells with OAS1, OAS2, or RNase L deficiencies produce excessive amounts of inflammatory cytokines upon dsRNA or severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) stimulation. Exogenous 2-5A suppresses cytokine production in OAS1-deficient but not RNase L-deficient cells. Cytokine production in RNase L-deficient cells is impaired by MDA5 or RIG-I deficiency and abolished by mitochondrial antiviral-signaling protein (MAVS) deficiency. Recessive OAS-RNase L deficiencies in these patients unleash the production of SARS-CoV-2-triggered, MAVS-mediated inflammatory cytokines by mononuclear phagocytes, thereby underlying MIS-C. | - |
dc.format.extent | 23 p. | - |
dc.format.mimetype | application/pdf | - |
dc.language.iso | eng | - |
dc.publisher | American Association for the Advancement of Science | - |
dc.relation.isformatof | Reproducció del document publicat a: https://doi.org/10.1126/science.abo3627 | - |
dc.relation.ispartof | Science, 2023, vol. 379, num. 6632, p. eabo3627 | - |
dc.relation.uri | https://doi.org/10.1126/science.abo3627 | - |
dc.rights | cc by (c) Lee, Danyel et al., 2023 | - |
dc.rights.uri | http://creativecommons.org/licenses/by/3.0/es/ | * |
dc.source | Articles publicats en revistes (Cirurgia i Especialitats Medicoquirúrgiques) | - |
dc.subject.classification | COVID-19 | - |
dc.subject.classification | SARS-CoV-2 | - |
dc.subject.classification | Inflamació | - |
dc.subject.classification | Infants | - |
dc.subject.other | COVID-19 | - |
dc.subject.other | SARS-CoV-2 | - |
dc.subject.other | Inflammation | - |
dc.subject.other | Children | - |
dc.title | Inborn errors of OAS-RNase L in SARS-CoV-2-related multisystem inflammatory syndrome in children | - |
dc.type | info:eu-repo/semantics/article | - |
dc.type | info:eu-repo/semantics/publishedVersion | - |
dc.identifier.idgrec | 732180 | - |
dc.date.updated | 2023-09-21T14:21:09Z | - |
dc.rights.accessRights | info:eu-repo/semantics/openAccess | - |
dc.identifier.pmid | 36538032 | - |
Appears in Collections: | Articles publicats en revistes (Cirurgia i Especialitats Medicoquirúrgiques) Articles publicats en revistes (Institut d'lnvestigació Biomèdica de Bellvitge (IDIBELL)) |
Files in This Item:
File | Description | Size | Format | |
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049311-053503.pdf | 5.87 MB | Adobe PDF | View/Open |
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