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oai:riubu.ubu.es:10259/100362025-01-25T01:05:15Zcom_10259_6688com_10259_5086com_10259_2604col_10259_6689

00925njm 22002777a 4500 dc Lee, Danyel author Le Pen, Jérémie author Yatim, Ahmad author Dong, Beihua author Aquino, Yann author Ogishi, Masato author Pescarmona, Rémi author Talouarn, Estelle author Rinchai, Darawan author Zhang, Peng author Perret, Magali author Liu, Zhiyong author Jordan, Iolanda author Elmas Bozdemir, Sefika author Bayhan, Gulsum Iclal author Beaufils, Camille author Bizien, Lucy author Bisiaux, Aurelie author Lei, Weite author Hasan, Milena author Chen, Jie author Gaughan, Christina author Asthana, Abhishek author Libri, Valentina author Luna, Joseph M. author Jaffré, Fabrice author Hoffmann, H.-Heinrich author Michailidis, Eleftherios author Moreews, Marion author Seeleuthner, Yoann author Bilguvar, Kaya author Mane, Shrikant author Flores, Carlos author Zhang, Yu author Arias, Andrés A. author Bailey, Rasheed author Schlüter, Agatha author Milisavljevic, Baptiste author Bigio, Benedetta author Le Voyer, Tom author Materna, Marie author Gervais, Adrian author Moncada-Velez, Marcela author Pala, Francesca author Lazarov, Tomi author Levy, Romain author Neehus, Anna-Lena author Rosain, Jérémie author Peel, Jessica author Chan, Yi-Hao author Morin, Marie-Paule author Pino-Ramirez, Rosa Maria author Belkaya, Serkan author Lorenzo, Lazaro author Anton, Jordi author Delafontaine, Selket author Toubiana, Julie author Bajolle, Fanny author Fumadó, Victoria author DeDiego, Marta L. author Fidouh, Nadhira author Rozenberg, Flore author Pérez-Tur, Jordi author Chen, Shuibing author Evans, Todd author Geissmann, Frédéric author Lebon, Pierre author Weiss, Susan R. author Bonnet, Damien author Duval, Xavier author Pan-Hammarström, Qiang author Planas, Anna M. author Meyts, Isabelle author Haerynck, Filomeen author Pujol, Aurora author Sancho-Shimizu, Vanessa author Dalgard, Clifford L. author Bustamante, Jacinta author Puel, Anne author Boisson-Dupuis, Stéphanie author Boisson, Bertrand author Maniatis, Tom author Zhang, Qian author Bastard, Paul author Notarangelo, Luigi author Béziat, Vivien author Perez de Diego, Rebeca author Rodriguez-Gallego, Carlos author Su, Helen C. author Lifton, Richard P. author Jouanguy, Emmanuelle author Cobat, Aurélie author Alsina, Laia author Keles, Sevgi author Haddad, Elie author Abel, Laurent author Belot, Alexandre author Quintana-Murci, Lluis author Rice, Charles M. author Silverman, Robert H. author Zhang, Shen-Ying author Casanova, Jean-Laurent author Alavoine, Loubna author Behillil, Sylvie author Burdet, Charles author Charpentier, Charlotte author Dechanet, Aline author Descamps, Diane author Ecobichon, Jean-Luc author Enouf, Vincent author Frezouls, Wahiba author Houhou, Nadhira author Kafif, Ouifiya author Lehacaut, Jonathan author Letrou, Sophie author Lina, Bruno author Lucet, Jean-Christophe author Manchon, Pauline author Nouroudine, Mariama author Piquard, Valentine author Quintin, Caroline author Thy, Michael author Tubiana, Sarah author van der Werf, Sylvie author Vignali, Valérie author Visseaux, Benoit author Yazdanpanah, Yazdan author Chahine, Abir author Waucquier, Nawal author Migaud, Maria-Claire author Deplanque, Dominique author Djossou, Félix author Mergeay-Fabre, Mayka author Lucarelli, Aude author Demar, Magalie author Bruneau, Léa author Gérardin, Patrick author Maillot, Adrien author Payet, Christine author Laviolle, Bruno author Laine, Fabrice author Paris, Christophe author Desille-Dugast, Mireille author Fouchard, Julie author Malvy, Denis author Nguyen, Duc author Pistone, Thierry author Perreau, Pauline author Gissot, Valérie author Le Goas, Carole author Montagne, Samatha author Richard, Lucie author Chirouze, Catherine author Bouiller, Kévin author Desmarets, Maxime author Meunier, Alexandre author Lefèvre, Benjamin author Jeulin, Hélène author Legrand, Karine author Lomazzi, Sandra author Tardy, Bernard author Gagneux-Brunon, Amandine author Bertholon, Frédérique author Botelho-Nevers, Elisabeth author Christelle, Kouakam author Nicolas, Leturque author Roufai, Layidé author Amat, Karine author Couffin-Cadiergues, Sandrine author Espérou, Hélène author Hendou, Samia author Abolhassani, Hassan author Aguilera-Albesa, Sergio author Aiuti, Alessandro author Akcan, Ozge Metin author Akcay, Nihal author Alkan, Gulsum author Alkhater, Suzan A. author Allende, Luis Miguel author Alper, Yosunkaya author Amenzoui, Naima author Anderson, Mark S. author Arkin, Lisa author Aubart, Melodie author Avramenko, Iryna author Aydemir, Şehnaz author Gayretli Aydin, Zeynep Gökçe author Aytekin, Caner author Aytekin, Gökhan author Erol Aytekin, Selma author Bando, Silvia Yumi author Beland, Kathie author Biggs, Catherine M. author Bilbao Aburto, Agurtzane author Blanchard-Rohner, Geraldine author Blázquez-Gamero, Daniel author Bloomfield, Marketa author Bogunovic, Dusan author Bondarenko, Anastasia author Borghesi, Alessandro author Bousfiha, Amed Aziz author Boyarchuk, Oksana author Brodin, Petter author Bryceson, Yenan author Bucciol, Giorgia author Calcaterra, Valeria author Casari, Giorgio author Cavalcanti, Andre author Celik, Jale Bengi author Chrousos, George P. author Colobran, Roger author Condino-Neto, Antonio author Conti, Francesca author Cooper, Megan author Coskuner, Taner author Cyrus, Cyril author D’Auria, Enza author Drolet, Beth A. author Bursal Duramaz, Burcu author El Zein, Loubna author Elnagdy, Marwa H. author Emiroglu, Melike author Erdeniz, Emine Hafize author Fabi, Marianna author Baris Feldman, Hagit author Fellay, Jacques author Fencl, Filip author Filippatos, Filippos author Freiss, Julie author Fremuth, Jiri author Gagro, Alenka author Garcia-Solis, Blanca author Vergine, Gianluca author González-Montelongo, Rafaela author Gul, Yahya author Gülhan, Belgin author Gultekin, Sara Sebnem Kilic author Gut, Marta author Halwani, Rabih author Hammarström, Lennart author Hatipoğlu, Nevin author Heath, James author Henrickson, Sarah E. author Hernandez-Brito, Elisa author Hoffman, Ilse author Hoste, Levi author Hsieh, Elena author Íñigo-Campos, Antonio author Itan, Yuval author Jabandziev, Petr author Kandemir, Bahar author Kanık-Yüksek, Saliha author Kapakli, Hasan author Karbuz, Adem author Kasapcopur, Ozgur author Kechiche, Robin author Kendir Demirkol, Yasemin author Kilic, Omer author Hansen, Stella Kim author Klocperk, Adam author Lau, Yu-Lung author Lebl, Jan author Lorenzo-Salazar, José M. author Lucas, Carrie L. author Maglorius, Majistor author Marque, Laura author Novoa Medina, Yeray author Montesdeoca Melián, Abián author Mentis, Alexios-Fotios A. author Pato, Michele T. author Michos, Athanasios author Milner, Joshua D. author Mogensen, Trine H. author Muñoz-Barrera, Adrián author Nepesov, Serdar author Farela Neves, João author Ng, Ashley author Ng, Lisa F. P. author Novelli, Antonio author Novelli, Giuseppe author Oz, Fatma Nur author Ocejo-Viñals, J. Gonzalo author Okada, Satoshi author Orbak, Zerrin author Kilic, Ahmet Osman author Ouair, Hind author Öz, Şadiye Kübra Tüter author Özçelik, Tayfun author Özkan, Esra Akyüz author Parlakay, Aslınur Özkaya author Pato, Carlos N. author Paz-Artal, Estela author Pelham, Simon author Pellier, Isabelle author Philippot, Quentin author Planas-Serra, Laura author Plassart, Samira author Pokorna, Petra author Polat, Meltem author Poli, Cecilia author Prando, Carolina author Renia, Laurent author Rivière, Jacques G. author Rodríguez-Palmero, Agustí author Roussel, Lucie author Rubio-Rodriguez, Luis A. author Salifu, Moro author Sasek, Lumir author Sasia, Laura author Scherbina, Anna author Schmitt, Erica author Sediva, Anna author Sevketoglu, Esra author Slaba, Katerina author Slaby, Ondrej author Sobh, Ali author Solé-Violán, Jordi author Soler-Palacin, Pere author De Somer, Lien author Sözeri, Betül author Spaan, András N. author Stepanovskiy, Yuriy author Tangye, Stuart G. author Tanir, Gonul author Tatsi, Elizabeth Barbara author Thorball, Christian W. author Hancerli Torun, Selda author Turvey, Stuart author Uddin, Mohammed J. author Uyar, Emel author Valencia Ramos, Juan author Van Den Rym, Ana Maria author Vatansev, Hulya author Castillo de Vera, Martín author Vermeulen, François author Vinh, Donald C. author Volokha, Alla author von Bernuth, Horst author Wouters, Carine author Yahşi, Aysun author Yarar, Volkan author Yesilbas, Osman author Yıldız, Mehmet author Zatz, Mayana author Zawadzki, Pawel author Zuccotti, Gianvincenzo author 2023-02 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. 0036-8075 http://hdl.handle.net/10259/10036 10.1126/SCIENCE.ABO3627 1095-9203 Inborn errors of OAS–RNase L in SARS-CoV-2–related multisystem inflammatory syndrome in children