Scopus
🔓 Açık Erişim YÖKSİS DOI Eşleşti
SJR Q1
Inborn errors of OAS-RNase L in SARS-CoV-2-related multisystem inflammatory syndrome in children
Science · Şubat 2023
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.
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
Prof. Dr. MELİKE EMİROĞLU →
Inborn errors of OAS–RNase L in SARS-CoV-2–related multisystem inflammatory syndrome in children
Science · 2023 SCI-Expanded
Prof. Dr. JALE BENGİ ÇELİK →
Inborn errors of OAS–RNase L in SARS-CoV-2–related multisystem inflammatory syndrome in children
Science · 2023 SCI-Expanded
Doç. Dr. GÜLSÜM ALKAN →
Inborn errors of OAS–RNase L in SARS-CoV-2–related multisystem inflammatory syndrome in children
Science · 2023 SCI-Expanded
Prof. Dr. JALE BENGİ ÇELİK →
Inborn errors of OAS–RNase L in SARS-CoV-2–related multisystem inflammatory syndrome in children
Science · 2023 SCI-Expanded
Prof. Dr. JALE BENGİ ÇELİK →
Inborn errors of OAS–RNase L in SARS-CoV-2–related multisystem inflammatory syndrome in children
Science · 2023 SCI-Expanded
Prof. Dr. MELİKE EMİROĞLU →
YÖKSİS Kayıtları — ISSN Eşleşmesi
Bu dergide (ISSN eşleşmesi) kurumun 11 kaydı bulundu.
YÖKSİS Kayıtları — ISSN Eşleşmesi
Bu dergide (ISSN eşleşmesi) kurumun 11 kaydı bulundu.
Autoantibodies against type I IFNs in patients with life-threatening COVID-19
2020 ISSN: 0036-8075 SCI
Prof. Dr. FİKRET KANAT →
Inborn errors of type I IFN immunity in patients with life-threatening COVID-19
2020 ISSN: 0036-8075 SCI
Prof. Dr. FİKRET KANAT →
Inborn errors of type I IFN immunity in patients with life-threatening COVID-19
2020 ISSN: 0036-8075 SCI-Expanded
Prof. Dr. MELİKE EMİROĞLU →
Inborn errors of OAS–RNase L in SARS-CoV-2–related multisystem inflammatory syndrome in children
2023 ISSN: 0036-8075 SCI-Expanded Q1
Prof. Dr. MELİKE EMİROĞLU →
Inborn errors of OAS–RNase L in SARS-CoV-2–related multisystem inflammatory syndrome in children
2023 ISSN: 0036-8075 SCI-Expanded Q1
Doç. Dr. GÜLSÜM ALKAN →
Autoantibodies against type I IFNs in patients with life-threatening COVID-19
2020 ISSN: 0036-8075 SCI-Expanded Q1
Doç. Dr. GÜLSÜM ALKAN →
Inborn errors of type I IFN immunity in patients with life-threatening COVID-19
2020 ISSN: 0036-8075 SCI-Expanded Q1
Prof. Dr. MELİKE EMİROĞLU →
Inborn errors of OAS–RNase L in SARS-CoV-2–related multisystem inflammatory syndrome in children
2023 ISSN: 0036-8075 SCI-Expanded Q1
Prof. Dr. MELİKE EMİROĞLU →
Inborn errors of type I IFN immunity in patients with life-threatening COVID-19
2020 ISSN: 0036-8075 SCI-Expanded Q1
Doç. Dr. GÜLSÜM ALKAN →
Inborn errors of OAS–RNase L in SARS-CoV-2–related multisystem inflammatory syndrome in children
2023 ISSN: 0036-8075 SCI-Expanded Q1
Prof. Dr. JALE BENGİ ÇELİK →
Inborn errors of OAS–RNase L in SARS-CoV-2–related multisystem inflammatory syndrome in children
2023 ISSN: 0036-8075 SCI-Expanded Q1
Prof. Dr. JALE BENGİ ÇELİK →
Makale Bilgileri
Dergi
Science
Toplam Atıf
142 atıf
· Scopus
ISSN00368075
Yayın TarihiŞubat 2023
Cilt / Sayfa379
Scopus ID2-s2.0-85147787850
Erişim🔓 Açık Erişim
Kurumlar
Acıbadem Mehmet Ali Aydınlar Üniversitesi
Istanbul Turkey
Ankara Yildirim Beyazit University
Ankara Turkey
Bilkent Üniversitesi
Ankara Turkey
Bursa City Hospital
Bursa Turkey
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 Cente
New York United States
Paris City University
Paris France
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 Pennsylvania Perelman School of Medicine
Philadelphia United States
Weill Cornell Medicine
New York United States
Yale School of Medicine
New Haven United States
Son Atıflar
Therapy-induced modulation of cellular response in Multisystem Inflammatory Syndrome in Children (MIS-C) - insights into selected immune cells’ markers expression
Scientific Reports
· 2026 · DOI
Neonatal multisystem inflammatory syndrome (MIS-N) associated with prenatal maternal SARS-CoV-2: a single-center experience
BMC Pediatrics
· 2026 · DOI
Screening of autoinflammatory genes in patients with SARS-CoV-2-associated MIS-C
Human Genomics
· 2026 · DOI
Alternative splicing broadens antiviral diversity at the human OAS2 locus
EMBO Journal
· 2026 · DOI
Tumor cell intrinsic dsRNA innate immune response triggered by PARP inhibitor is compromised in BRCA1-deficient breast cancer by repressing IRF3
Protein and Cell
· 2026 · DOI
Stalled translation on transcripts cleaved by RNase L activates signaling important for innate immunity
RNA
· 2026 · DOI
Toward a monogenic architecture of human infections: From 1996 to 2026
Journal of Human Immunity
· 2026 · DOI
Non-canonical proteolytic activation of RNase L by SARS-CoV-2 3CLprooffsets inactivation of OAS1 p46 antiviral signaling
Cell Reports
· 2026 · DOI
Predisposition to viral infection of the fetus and neonate
Current Opinion in Virology
· 2026 · DOI
Cytoplasmic mRNA decay by the antiviral nuclease RNase L promotes transcriptional repression
Cell Reports
· 2026 · DOI
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Scimago Dergi (ISSN Eşleşmesi)
Science
Q1
OA
SJR Skoru10,948
H-Index1433
YayıncıAmerican Association for the Advancement of Science
ÜlkeUnited States
History and Philosophy of Science (Q1)
Multidisciplinary (Q1)
Metrikler
142
Atıf