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Journal Articles Science Year : 2023

Inborn errors of OAS–RNase L in SARS-CoV-2–related multisystem inflammatory syndrome in children

Danyel Lee (1, 2) , Jérémie Le Pen , Ahmad Yatim (3) , Beihua Dong , Yann Aquino (4) , Masato Ogishi (1) , Rémi Pescarmona (5, 6, 7, 8) , Estelle Talouarn (9) , Darawan Rinchai (10) , Peng Zhang (1, 2) , Magali Perret (11) , Zhiyong Liu (12) , Iolanda Jordan (13) , Sefika Elmas Bozdemir , Gulsum Iclal Bayhan , Camille Beaufils , Lucy Bizien (2, 14, 15) , Aurelie Bisiaux (16) , Weite Lei , Milena Hasan (17) , Jie Chen (1, 18) , Christina Gaughan , Abhishek Asthana (19) , Valentina Libri (20) , Joseph Luna , Fabrice Jaffré , H.-Heinrich Hoffmann , Eleftherios Michailidis , Marion Moreews (5) , Yoann Seeleuthner , Kaya Bilguvar , Shrikant Mane , Carlos Flores , Yu Zhang , Andrés Arias , Rasheed Bailey , Agatha Schlüter , Baptiste Milisavljevic , Benedetta Bigio , Tom Le Voyer , Marie Materna , Adrian Gervais , Marcela Moncada-Velez , Francesca Pala , Tomi Lazarov , Romain Levy , Anna-Lena Neehus , Jérémie Rosain , Jessica Peel , Yi-Hao Chan , Marie-Paule Morin , Rosa Maria Pino-Ramirez , Serkan Belkaya , Lazaro Lorenzo , Jordi Anton , Selket Delafontaine , Julie Toubiana (21) , Fanny Bajolle , Victoria Fumadó , Marta Dediego , Nadhira Fidouh , Flore Rozenberg , Jordi Pérez-Tur , Shuibing Chen , Todd Evans , Frédéric Geissmann , Pierre Lebon , Susan Weiss , Damien Bonnet , Xavier Duval (22) , Qiang Pan-Hammarström , Anna Planas , Isabelle Meyts , Filomeen Haerynck , Aurora Pujol , Vanessa Sancho-Shimizu , Clifford Dalgard , Jacinta Bustamante , Anne Puel , Stéphanie Boisson-Dupuis , Bertrand Boisson , Tom Maniatis , Qian Zhang , Paul Bastard , Luigi Notarangelo , Vivien Béziat , Rebeca Perez de Diego , Carlos Rodriguez-Gallego , Helen Su , Richard Lifton , Emmanuelle Jouanguy , Aurélie Cobat , Laia Alsina , Sevgi Keles , Elie Haddad , Laurent Abel , Alexandre Belot (7) , Lluis Quintana-Murci (16) , Charles Rice , Robert Silverman , Shen-Ying Zhang , Jean-Laurent Casanova , Loubna Alavoine (23) , Sylvie Behillil , Charles Burdet , Charlotte Charpentier , Aline Dechanet , Diane Descamps , Xavier Duval , Jean-Luc Ecobichon , Vincent Enouf , Wahiba Frezouls , Nadhira Houhou , Ouifiya Kafif , Jonathan Lehacaut , Sophie Letrou , Bruno Lina (5) , Jean-Christophe Lucet , Pauline Manchon , Mariama Nouroudine , Valentine Piquard , Caroline Quintin , Michael Thy , Sarah Tubiana (24) , Sylvie van der Werf , Valérie Vignali , Benoit Visseaux , Yazdan Yazdanpanah , Abir Chahine , Nawal Waucquier , Maria-Claire Migaud (25) , Dominique Deplanque , Félix Djossou , Mayka Mergeay-Fabre (26) , Aude Lucarelli , Magalie Demar , Léa Bruneau , Patrick Gérardin , Adrien Maillot (27) , Christine Payet , Bruno Laviolle (28, 29) , Fabrice Laine (28, 29) , Christophe Paris (28, 29) , Mireille Desille-Dugast (28, 29) , Julie Fouchard (28, 29) , Denis Malvy , Duc Nguyen , Thierry Pistone , Pauline Perreau , Valérie Gissot , Carole Le Goas , Samatha Montagne , Lucie Richard , Catherine Chirouze , Kévin Bouiller , Maxime Desmarets , Alexandre Meunier , Benjamin Lefèvre , Hélène Jeulin , Karine Legrand , Sandra Lomazzi , Bernard Tardy , Amandine Gagneux-Brunon , Frédérique Bertholon , Elisabeth Botelho-Nevers , Kouakam Christelle , Leturque Nicolas , Layidé Roufai , Karine Amat , Sandrine Couffin-Cadiergues , Hélène Espérou , Samia Hendou , Laurent Abel , Hassan Abolhassani , Sergio Aguilera-Albesa , Alessandro Aiuti , Ozge Metin Akcan , Nihal Akcay , Gulsum Alkan , Suzan Alkhater , Luis Miguel Allende , Yosunkaya Alper , Naima Amenzoui , Mark Anderson , Lisa Arkin , Melodie Aubart , Iryna Avramenko , Şehnaz Aydemir , Zeynep Gökçe Gayretli Aydin , Caner Aytekin , Gökhan Aytekin , Selma Erol Aytekin , Silvia Yumi Bando , Kathie Beland , Serkan Belkaya , Catherine Biggs , Agurtzane Bilbao Aburto , Geraldine Blanchard-Rohner , Daniel Blázquez-Gamero , Marketa Bloomfield , Dusan Bogunovic , Anastasia Bondarenko , Alessandro Borghesi , Amed Aziz Bousfiha , Oksana Boyarchuk , Petter Brodin , Yenan Bryceson , Giorgia Bucciol , Valeria Calcaterra , Giorgio Casari , Andre Cavalcanti , Jale Bengi Celik , George Chrousos , Roger Colobran , Antonio Condino-Neto , Francesca Conti , Megan Cooper , Taner Coskuner , Cyril Cyrus , Enza D’auria , Selket Delafontaine , Beth Drolet , Burcu Bursal Duramaz , Loubna El Zein , Marwa Elnagdy , Melike Emiroglu , Emine Hafize Erdeniz , Marianna Fabi , Hagit Baris Feldman , Jacques Fellay , Filip Fencl , Filippos Filippatos , Julie Freiss , Jiri Fremuth , Alenka Gagro , Blanca Garcia-Solis , Gianluca Vergine , Rafaela González-Montelongo , Yahya Gul , Belgin Gülhan , Sara Sebnem Kilic Gultekin , Marta Gut , Rabih Halwani , Lennart Hammarström , Nevin Hatipoğlu , James Heath , Sarah Henrickson , Elisa Hernandez-Brito , Ilse Hoffman , Levi Hoste , Elena Hsieh , Antonio Íñigo-Campos , Yuval Itan , Petr Jabandziev , Bahar Kandemir , Saliha Kanık-Yüksek , Hasan Kapakli , Adem Karbuz , Ozgur Kasapcopur , Robin Kechiche , Yasemin Kendir Demirkol , Omer Kilic , Stella Kim Hansen , Adam Klocperk , Yu-Lung Lau , Jan Lebl , José Lorenzo-Salazar , Carrie Lucas , Majistor Maglorius , Laura Marque , Yeray Novoa Medina , Abián Montesdeoca Melián , Alexios-Fotios Mentis , Michele Pato , Athanasios Michos , Joshua Milner , Trine Mogensen , Adrián Muñoz-Barrera , Serdar Nepesov , João Farela Neves , Ashley Ng , Lisa Ng , Antonio Novelli , Giuseppe Novelli , Fatma Nur Oz , J. Gonzalo Ocejo-Viñals , Satoshi Okada , Zerrin Orbak , Ahmet Osman Kilic , Hind Ouair , Şadiye Kübra Tüter Öz , Tayfun Özçelik , Esra Akyüz Özkan , Aslınur Özkaya Parlakay , Carlos Pato , Estela Paz-Artal , Simon Pelham , Isabelle Pellier , Quentin Philippot , Laura Planas-Serra , Samira Plassart , Petra Pokorna , Meltem Polat , Cecilia Poli , Carolina Prando , Laurent Renia , Jacques Rivière , Agustí Rodríguez-Palmero , Lucie Roussel , Luis Rubio-Rodriguez , Moro Salifu , Lumir Sasek , Laura Sasia , Anna Scherbina , Erica Schmitt , Anna Sediva , Esra Sevketoglu , Katerina Slaba , Ondrej Slaby , Ali Sobh , Jordi Solé-Violán , Pere Soler-Palacin , Lien de Somer , Betül Sözeri , András Spaan , Yuriy Stepanovskiy , Stuart Tangye , Gonul Tanir , Elizabeth Barbara Tatsi , Christian Thorball , Selda Hancerli Torun , Stuart Turvey , Mohammed Uddin , Emel Uyar , Juan Valencia-Ramos , Ana Maria van den Rym , Hulya Vatansev , Martín Castillo de Vera , François Vermeulen , Donald Vinh , Alla Volokha , Horst von Bernuth , Carine Wouters , Aysun Yahşi , Volkan Yarar , Osman Yesilbas , Mehmet Yıldız , Mayana Zatz , Pawel Zawadzki , Gianvincenzo Zuccotti , Shen-Ying Zhang , Jean-Laurent Casanova
1 Rockefeller University [New York]
2 Imagine - U1163 - Imagine - Institut des maladies génétiques (IHU)
3 IGH - Institut de génétique humaine
4 GEMS - Génomique évolutive, modélisation et santé
5 CIRI - Centre International de Recherche en Infectiologie
6 RAISE - Centre de référence des rhumatismes inflammatoires et maladies auto-immunes systémiques rares de l’enfant / National Referee Centre for Rheumatic and AutoImmune and Systemic Diseases in Children [Lyon]
7 HCL - Hospices Civils de Lyon
8 CHLS - Centre Hospitalier Lyon Sud [CHU - HCL]
9 GenPhySE - Génétique Physiologie et Systèmes d'Elevage
10 Sidra Medicine [Doha, Qatar]
11 BIOASTER Technology Research Institute, Lyon, France
12 St. Giles Laboratory of Human Genetics of Infectious Diseases
13 Department of Paediatrics and Intensive Care
14 Equipe Inserm U1163 - Human genetics of infectious diseases : Mendelian predisposition
15 Département de Pédiatrie et maladies infectieuses [CHU Necker]
16 Génétique Evolutive Humaine - Human Evolutionary Genetics
17 CRT - Centre de Recherche Translationnelle - Center for Translational Science
18 Shangaï Jiao Tong University [Shangaï]
19 Sheffield Hallam University
20 IP - Institut Pasteur [Paris]
21 CHU Necker - Enfants Malades [AP-HP]
22 IAME (UMR_S_1137 / U1137) - Infection, Anti-microbiens, Modélisation, Evolution
23 CIC 1425 - Centre d'investigation Clinique [CHU Bichat] - Épidémiologie clinique
24 Biodiversité et Epidémiologie des Bactéries pathogènes - Biodiversity and Epidemiology of Bacterial Pathogens
25 CIC Lille - Centre d'Investigation Clinique - Innovation Technologique de Lille - CIC 1403 - CIC 9301
26 CIC - Antilles Guyane - Centre d'Investigation Clinique Antilles-Guyane
27 CIC 1410 - Centre d'Investigation Clinique de La Réunion - INSERM
28 CIC - Centre d'Investigation Clinique [Rennes]
29 CHU Pontchaillou [Rennes]
Jérémie Le Pen
Beihua Dong
  • Function : Author
Masato Ogishi
Darawan Rinchai
Sefika Elmas Bozdemir
Gulsum Iclal Bayhan
Camille Beaufils
Weite Lei
Christina Gaughan
Abhishek Asthana
Valentina Libri
Joseph Luna
Fabrice Jaffré
H.-Heinrich Hoffmann
Eleftherios Michailidis
Yoann Seeleuthner
  • Function : Author
Kaya Bilguvar
Shrikant Mane
Carlos Flores
Yu Zhang
Andrés Arias
Rasheed Bailey
  • Function : Author
Agatha Schlüter
Baptiste Milisavljevic
  • Function : Author
Benedetta Bigio
  • Function : Author
Tom Le Voyer
Marie Materna
Adrian Gervais
Marcela Moncada-Velez
Francesca Pala
Tomi Lazarov
Romain Levy
Anna-Lena Neehus
Jérémie Rosain
Jessica Peel
  • Function : Author
Yi-Hao Chan
Marie-Paule Morin
  • Function : Author
Rosa Maria Pino-Ramirez
Serkan Belkaya
Lazaro Lorenzo
Jordi Anton
Selket Delafontaine
Fanny Bajolle
Victoria Fumadó
  • Function : Author
Marta Dediego
  • Function : Author
Nadhira Fidouh
  • Function : Author
Flore Rozenberg
  • Function : Author
Jordi Pérez-Tur
Shuibing Chen
Todd Evans
Frédéric Geissmann
Pierre Lebon
  • Function : Author
Susan Weiss
Damien Bonnet
Qiang Pan-Hammarström
Anna Planas
Isabelle Meyts
Filomeen Haerynck
Aurora Pujol
Vanessa Sancho-Shimizu
Clifford Dalgard
Jacinta Bustamante
Anne Puel
Stéphanie Boisson-Dupuis
Bertrand Boisson
Tom Maniatis
Qian Zhang
Paul Bastard
Luigi Notarangelo
Vivien Béziat
Rebeca Perez de Diego
Carlos Rodriguez-Gallego
  • Function : Author
Helen Su
Richard Lifton
  • Function : Author
Emmanuelle Jouanguy
Aurélie Cobat
Laia Alsina
Sevgi Keles
  • Function : Author
Elie Haddad
Laurent Abel
Alexandre Belot
Charles Rice
Robert Silverman
Shen-Ying Zhang
Jean-Laurent Casanova
Sylvie Behillil
  • Function : Author
Charles Burdet
  • Function : Author
Charlotte Charpentier
  • Function : Author
Aline Dechanet
  • Function : Author
Diane Descamps
  • Function : Author
Xavier Duval
  • Function : Author
Jean-Luc Ecobichon
  • Function : Author
Vincent Enouf
  • Function : Author
Wahiba Frezouls
  • Function : Author
Nadhira Houhou
  • Function : Author
Ouifiya Kafif
  • Function : Author
Jonathan Lehacaut
  • Function : Author
Sophie Letrou
  • Function : Author
Jean-Christophe Lucet
  • Function : Author
Pauline Manchon
  • Function : Author
Mariama Nouroudine
  • Function : Author
Valentine Piquard
  • Function : Author
Caroline Quintin
  • Function : Author
Michael Thy
  • Function : Author
Sylvie van der Werf
Valérie Vignali
  • Function : Author
Benoit Visseaux
  • Function : Author
Yazdan Yazdanpanah
  • Function : Author
Abir Chahine
  • Function : Author
Nawal Waucquier
  • Function : Author
Dominique Deplanque
  • Function : Author
Félix Djossou
  • Function : Author
Aude Lucarelli
  • Function : Author
Magalie Demar
  • Function : Author
Léa Bruneau
  • Function : Author
Patrick Gérardin
  • Function : Author
Christine Payet
  • Function : Author
Denis Malvy
  • Function : Author
Duc Nguyen
  • Function : Author
Thierry Pistone
  • Function : Author
Pauline Perreau
  • Function : Author
Valérie Gissot
  • Function : Author
Carole Le Goas
  • Function : Author
Samatha Montagne
  • Function : Author
Lucie Richard
  • Function : Author
Catherine Chirouze
  • Function : Author
Kévin Bouiller
  • Function : Author
Maxime Desmarets
  • Function : Author
Alexandre Meunier
  • Function : Author
Benjamin Lefèvre
  • Function : Author
Hélène Jeulin
Karine Legrand
  • Function : Author
Sandra Lomazzi
  • Function : Author
Bernard Tardy
  • Function : Author
Amandine Gagneux-Brunon
  • Function : Author
Frédérique Bertholon
  • Function : Author
Elisabeth Botelho-Nevers
  • Function : Author
Kouakam Christelle
  • Function : Author
Leturque Nicolas
  • Function : Author
Layidé Roufai
  • Function : Author
Karine Amat
  • Function : Author
Sandrine Couffin-Cadiergues
  • Function : Author
Hélène Espérou
  • Function : Author
Samia Hendou
  • Function : Author
Laurent Abel
  • Function : Author
Hassan Abolhassani
  • Function : Author
Sergio Aguilera-Albesa
  • Function : Author
Alessandro Aiuti
  • Function : Author
Ozge Metin Akcan
  • Function : Author
Nihal Akcay
  • Function : Author
Gulsum Alkan
  • Function : Author
Suzan Alkhater
  • Function : Author
Luis Miguel Allende
  • Function : Author
Yosunkaya Alper
  • Function : Author
Naima Amenzoui
  • Function : Author
Mark Anderson
  • Function : Author
Lisa Arkin
  • Function : Author
Melodie Aubart
  • Function : Author
Iryna Avramenko
  • Function : Author
Şehnaz Aydemir
  • Function : Author
Zeynep Gökçe Gayretli Aydin
  • Function : Author
Caner Aytekin
  • Function : Author
Gökhan Aytekin
  • Function : Author
Selma Erol Aytekin
  • Function : Author
Silvia Yumi Bando
  • Function : Author
Kathie Beland
  • Function : Author
Serkan Belkaya
  • Function : Author
Catherine Biggs
  • Function : Author
Agurtzane Bilbao Aburto
  • Function : Author
Geraldine Blanchard-Rohner
  • Function : Author
Daniel Blázquez-Gamero
  • Function : Author
Marketa Bloomfield
  • Function : Author
Dusan Bogunovic
  • Function : Author
Anastasia Bondarenko
  • Function : Author
Alessandro Borghesi
  • Function : Author
Amed Aziz Bousfiha
  • Function : Author
Oksana Boyarchuk
  • Function : Author
Petter Brodin
  • Function : Author
Yenan Bryceson
  • Function : Author
Giorgia Bucciol
  • Function : Author
Valeria Calcaterra
  • Function : Author
Giorgio Casari
  • Function : Author
Andre Cavalcanti
  • Function : Author
Jale Bengi Celik
  • Function : Author
George Chrousos
  • Function : Author
Roger Colobran
  • Function : Author
Antonio Condino-Neto
  • Function : Author
Francesca Conti
  • Function : Author
Megan Cooper
  • Function : Author
Taner Coskuner
  • Function : Author
Cyril Cyrus
  • Function : Author
Enza D’auria
  • Function : Author
Selket Delafontaine
  • Function : Author
Beth Drolet
  • Function : Author
Burcu Bursal Duramaz
  • Function : Author
Loubna El Zein
  • Function : Author
Marwa Elnagdy
  • Function : Author
Melike Emiroglu
  • Function : Author
Emine Hafize Erdeniz
  • Function : Author
Marianna Fabi
  • Function : Author
Hagit Baris Feldman
  • Function : Author
Jacques Fellay
  • Function : Author
Filip Fencl
  • Function : Author
Filippos Filippatos
  • Function : Author
Julie Freiss
  • Function : Author
Jiri Fremuth
  • Function : Author
Alenka Gagro
  • Function : Author
Blanca Garcia-Solis
  • Function : Author
Gianluca Vergine
  • Function : Author
Rafaela González-Montelongo
  • Function : Author
Yahya Gul
  • Function : Author
Belgin Gülhan
  • Function : Author
Sara Sebnem Kilic Gultekin
  • Function : Author
Marta Gut
  • Function : Author
Rabih Halwani
  • Function : Author
Lennart Hammarström
  • Function : Author
Nevin Hatipoğlu
  • Function : Author
James Heath
  • Function : Author
Sarah Henrickson
  • Function : Author
Elisa Hernandez-Brito
  • Function : Author
Ilse Hoffman
  • Function : Author
Levi Hoste
  • Function : Author
Elena Hsieh
  • Function : Author
Antonio Íñigo-Campos
  • Function : Author
Yuval Itan
  • Function : Author
Petr Jabandziev
  • Function : Author
Bahar Kandemir
  • Function : Author
Saliha Kanık-Yüksek
  • Function : Author
Hasan Kapakli
  • Function : Author
Adem Karbuz
  • Function : Author
Ozgur Kasapcopur
  • Function : Author
Robin Kechiche
  • Function : Author
Yasemin Kendir Demirkol
  • Function : Author
Omer Kilic
  • Function : Author
Stella Kim Hansen
  • Function : Author
Adam Klocperk
  • Function : Author
Yu-Lung Lau
  • Function : Author
Jan Lebl
  • Function : Author
José Lorenzo-Salazar
  • Function : Author
Carrie Lucas
  • Function : Author
Majistor Maglorius
  • Function : Author
Laura Marque
  • Function : Author
Yeray Novoa Medina
  • Function : Author
Abián Montesdeoca Melián
  • Function : Author
Alexios-Fotios Mentis
  • Function : Author
Michele Pato
  • Function : Author
Athanasios Michos
  • Function : Author
Joshua Milner
  • Function : Author
Trine Mogensen
  • Function : Author
Adrián Muñoz-Barrera
  • Function : Author
Serdar Nepesov
  • Function : Author
João Farela Neves
  • Function : Author
Ashley Ng
  • Function : Author
Lisa Ng
  • Function : Author
Antonio Novelli
  • Function : Author
Giuseppe Novelli
  • Function : Author
Fatma Nur Oz
  • Function : Author
J. Gonzalo Ocejo-Viñals
  • Function : Author
Satoshi Okada
  • Function : Author
Zerrin Orbak
  • Function : Author
Ahmet Osman Kilic
  • Function : Author
Hind Ouair
  • Function : Author
Şadiye Kübra Tüter Öz
  • Function : Author
Tayfun Özçelik
  • Function : Author
Esra Akyüz Özkan
  • Function : Author
Aslınur Özkaya Parlakay
  • Function : Author
Carlos Pato
  • Function : Author
Estela Paz-Artal
  • Function : Author
Simon Pelham
  • Function : Author
Isabelle Pellier
  • Function : Author
Quentin Philippot
  • Function : Author
Laura Planas-Serra
  • Function : Author
Samira Plassart
  • Function : Author
Petra Pokorna
  • Function : Author
Meltem Polat
  • Function : Author
Cecilia Poli
  • Function : Author
Carolina Prando
  • Function : Author
Laurent Renia
  • Function : Author
Jacques Rivière
  • Function : Author
Agustí Rodríguez-Palmero
  • Function : Author
Lucie Roussel
  • Function : Author
Luis Rubio-Rodriguez
  • Function : Author
Moro Salifu
  • Function : Author
Lumir Sasek
  • Function : Author
Laura Sasia
  • Function : Author
Anna Scherbina
  • Function : Author
Erica Schmitt
  • Function : Author
Anna Sediva
  • Function : Author
Esra Sevketoglu
  • Function : Author
Katerina Slaba
  • Function : Author
Ondrej Slaby
  • Function : Author
Ali Sobh
  • Function : Author
Jordi Solé-Violán
  • Function : Author
Pere Soler-Palacin
  • Function : Author
Lien de Somer
  • Function : Author
Betül Sözeri
  • Function : Author
András Spaan
  • Function : Author
Yuriy Stepanovskiy
  • Function : Author
Stuart Tangye
  • Function : Author
Gonul Tanir
  • Function : Author
Elizabeth Barbara Tatsi
  • Function : Author
Christian Thorball
  • Function : Author
Selda Hancerli Torun
  • Function : Author
Stuart Turvey
  • Function : Author
Mohammed Uddin
  • Function : Author
Emel Uyar
  • Function : Author
Juan Valencia-Ramos
  • Function : Author
Ana Maria van den Rym
  • Function : Author
Hulya Vatansev
  • Function : Author
Martín Castillo de Vera
  • Function : Author
François Vermeulen
  • Function : Author
Donald Vinh
  • Function : Author
Alla Volokha
  • Function : Author
Horst von Bernuth
  • Function : Author
Carine Wouters
  • Function : Author
Aysun Yahşi
  • Function : Author
Volkan Yarar
  • Function : Author
Osman Yesilbas
  • Function : Author
Mehmet Yıldız
  • Function : Author
Mayana Zatz
  • Function : Author
Pawel Zawadzki
  • Function : Author
Gianvincenzo Zuccotti
  • Function : Author
Shen-Ying Zhang
  • Function : Author
Jean-Laurent Casanova
  • Function : Author

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.
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hal-04089777 , version 1 (31-05-2023)

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Danyel Lee, Jérémie Le Pen, Ahmad Yatim, Beihua Dong, Yann Aquino, et al.. Inborn errors of OAS–RNase L in SARS-CoV-2–related multisystem inflammatory syndrome in children. Science, 2023, 379 (6632), pp.eabo3627. ⟨10.1126/science.abo3627⟩. ⟨hal-04089777⟩
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