Scopus
🔓 Açık Erişim YÖKSİS Eşleşti
Inborn errors of OAS-RNase L in SARS-CoV-2-related multisystem inflammatory syndrome in children
Science · Şubat 2023
YÖKSİS Kayıtları
Inborn errors of OAS–RNase L in SARS-CoV-2–related multisystem inflammatory syndrome in children
American Association for the Advancement of Science (AAAS) · 2023 SCI-Expanded
DOÇENT MELİKE EMİROĞLU →
Inborn errors of OAS–RNase L in SARS-CoV-2–related multisystem inflammatory syndrome in children
Science · 2023 SCI-Expanded
DOÇENT GÜLSÜM ALKAN →
Inborn errors of OAS–RNase L in SARS-CoV-2–related multisystem inflammatory syndrome in children
Science · 2023 SCI-Expanded
PROFESÖR JALE BENGİ ÇELİK →
Inborn errors of OAS–RNase L in SARS-CoV-2–related multisystem inflammatory syndrome in children
Science · 2023 SCI-Expanded
PROFESÖR JALE BENGİ ÇELİK →
Inborn errors of OAS–RNase L in SARS-CoV-2–related multisystem inflammatory syndrome in children
Science · 2023 SCI-Expanded
DOÇENT MELİKE EMİROĞLU →
Makale Bilgileri
DergiScience
Yayın TarihiŞubat 2023
Cilt / Sayfa379
Scopus ID2-s2.0-85147787850
Erişim🔓 Açık Erişim
Özet
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.
Yazarlar (100)
1
Danyel Lee
ORCID: 0000-0003-3124-0245
2
Jérémie Le Pen
3
Ahmad Yatim
4
Beihua Dong
5
Yann Aquino
6
Masato Ogishi
7
Rémi Pescarmona
8
Estelle Talouarn
ORCID: 0000-0002-5016-0446
9
Darawan Rinchai
10
Peng Zhang
11
Magali Perret
12
Zhiyong Liu
ORCID: 0000-0001-6473-348X
13
Iolanda Jordan
14
Sefika Elmas Bozdemir
ORCID: 0000-0003-1058-0320
15
Gulsum Iclal Bayhan
ORCID: 0000-0002-1423-4348
16
Camille Beaufils
17
Lucy Bizien
18
Aurelie Bisiaux
19
Weite Lei
20
Milena Hasan
21
Jie Chen
22
Christina Gaughan
23
Abhishek Asthana
24
Valentina Libri
25
Joseph M. Luna
26
Fabrice Jaffré
27
H. Heinrich Hoffmann
28
Eleftherios Michailidis
29
Marion Moreews
30
Yoann Seeleuthner
ORCID: 0000-0002-1878-8567
31
Kaya Bilguvar
32
Shrikant Mane
33
Carlos Flores
34
Yu Zhang
35
Andrés Augusto Arias
36
Rasheed Bailey
37
Agatha Schlüter
38
Baptiste Milisavljevic
39
Benedetta Bigio
40
Tom Le Voyer
ORCID: 0000-0002-7253-3135
41
Marie Materna
42
Adrian Gervais
43
Marcela Moncada-Velez
44
Francesca Pala
45
Tomi Lazarov
46
Romain Lévy
47
Anna Lena Neehus
48
Jérémie Rosain
49
Jessica Peel
50
Yi Hao Chan
ORCID: 0000-0003-0329-238X
51
Marie Paule Morin
ORCID: 0000-0003-1358-6429
52
Rosa Maria Pino-Ramirez
53
Serkan Belkaya
54
Lazaro Lorenzo
55
Jordi Anton
56
Selket Delafontaine
57
Julie Toubiana
ORCID: 0000-0001-9561-669X
58
Fanny Bajolle
59
Victoria Fumadó
60
Marta L. DeDiego
61
Nadhira Fidouh
62
Flore Rozenberg
63
Jordi Perez-Tur
64
Shuibing Chen
65
Todd Evans
66
Frédéric Geissmann
67
Pierre Lebon
68
Susan R. Weiss
69
Damien Bonnet
70
Xavier Duval
71
Qiang Pan-Hammarström
72
Anna M. Planas
73
Isabelle Meyts
74
Filomeen Haerynck
ORCID: 0000-0001-9161-7361
75
Aurora Pujol
ORCID: 0000-0002-9606-0600
76
Vanessa Sancho-Shimizu
ORCID: 0000-0002-3519-0727
77
Clifton L. Dalgard
78
Jacinta Bustamante
79
Anne Puel
80
Stéphanie Boisson-Dupuis
81
Bertrand Boisson
82
Tom Maniatis
83
Qian Zhang
84
Paul Bastard
85
Luigi Notarangelo
ORCID: 0000-0002-8335-0262
86
Vivien Béziat
87
Rebeca Pérez de Diego
ORCID: 0000-0001-8426-8765
88
Carlos Rodriguez-Gallego
89
Helen C. Su
90
Richard P. Lifton
91
Emmanuelle Jouanguy
ORCID: 0000-0002-7358-9157
92
Aurélie Cobat
ORCID: 0000-0001-7209-6257
93
Laia Alsina
94
Sevgi Keles
95
Elie Haddad
ORCID: 0000-0003-2446-6879
96
Laurent Abel
ORCID: 0000-0001-7016-6493
97
Alexandre Belot
ORCID: 0000-0003-4902-5332
98
Lluis Quintana-Murci
99
Charles M. Rice
100
Robert H. Silverman
Kurumlar
Acıbadem Mehmet Ali Aydınlar Üniversitesi
Istanbul Turkey
Ankara Yildirim Beyazit University
Ankara Turkey
Bilkent Üniversitesi
Ankara Turkey
Bursa City Hospital
Bursa Türkiye
CASE School of Medicine
Cleveland United States
Centre Hospitalier Lyon-Sud
Lyon France
Centro de Investigación Biomédica en Red de Enfermedades Neurodegenerativas
Madrid Spain
Centro de Investigación Biomédica en Red de Enfermedades Raras
Madrid Spain
Centro de Investigación Biomédica en Red de Enfermedades Respiratorias
Madrid Spain
Centro de Investigación Biomédica en Red de Epidemiología y Salud Pública
Madrid Spain
CHU de Lyon
Lyon France
CHU Sainte-Justine - Le Centre Hospitalier Universitaire Mère-Enfant
Montreal Canada
Cleveland Clinic Foundation
Cleveland United States
Collège de France
Paris France
CSIC - Centro Nacional de Biotecnologia (CNB)
Madrid Spain
CSIC - Instituto de Biomedicina de Valencia (IBV)
Valencia Spain
CSIC - Instituto de Investigaciones Biomedicas de Barcelona (IIBB)
Barcelona Spain
Emory University School of Medicine
Atlanta United States
Hôpital Bichat-Claude-Bernard AP-HP
Paris France
Hopital Cochin AP-HP
Paris France
Hôpital Necker Enfants Malades
Paris France
Hospital de Gran Canaria Dr. Negrin
Las Palmas de Gran Canaria Spain
Hospital Duran i Reynals
Barcelona Spain
Hospital Sant Joan de Déu Barcelona
Esplugues de Llobregat Spain
Hospital Universitario Nuestra Senora de Candelaria
Santa Cruz de Tenerife Spain
Imperial College Faculty of Medicine
London United Kingdom
Imperial College London
London United Kingdom
Inserm
Paris France
Institut de Recerca Sant Joan de Déu
Barcelona Spain
Institut d'Investigacions Biomèdiques August Pi i Sunyer - IDIBAPS
Barcelona Spain
Institute of Technology and Renewable Energies (ITER)
Granadilla de Abona Spain
Instituto de Investigación Sanitaria del Hospital Universitario La Paz
Madrid Spain
Instituto de Investigación Sanitaria La Fe
Valencia Spain
Institut Pasteur, Paris
Paris France
Interdepartmental Group of Immunodeficiencies
Madrid Spain
Karolinska Institutet
Stockholm Sweden
Kids Corona Platform
Barcelona Spain
KU Leuven– University Hospital Leuven
Leuven Belgium
l'Institut des Maladies Génétiques Imagine
Paris France
Memorial Sloan-Kettering Cancer Center
New York United States
National Institute of Allergy and Infectious Diseases (NIAID)
Bethesda United States
Necmettin Erbakan Üniversitesi
Meram Turkey
New York Genome Center
New York United States
Rockefeller University
New York United States
Sorbonne Université
Paris France
Uniformed Services University of the Health Sciences
Bethesda United States
Universidad de Antioquia
Medellin Colombia
Universidad Fernando Pessoa Canarias
Santa Maria de Guia de Gran Canaria Spain
Universitair Ziekenhuis Gent
Ghent Belgium
Universitat de Barcelona, Facultad de Medicina y Ciencias de la Salud
Barcelona Spain
Université Claude Bernard Lyon 1
Villeurbanne France
Université Paris Cité
Paris France
University of Paris City
Paris France
University of Pennsylvania Perelman School of Medicine
Philadelphia United States
Weill Cornell Medicine
New York United States
Yale School of Medicine
New Haven United States
Metrikler
89
Atıf
100
Yazar