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Lee, Danyel;
Le Pen, Jérémie;
Yatim, Ahmad;
Dong, Beihua;
Aquino, Yann;
Ogishi, Masato;
Pescarmona, Rémi;
Talouarn, Estelle;
Rinchai, Darawan;
Zhang, Peng;
Perret, Magali;
Liu, Zhiyong;
Jordan, Iolanda;
Elmas Bozdemir, Sefika;
Bayhan, Gulsum Iclal;
Beaufils, Camille;
Bizien, Lucy;
Bisiaux, Aurelie;
Lei, Weite;
Hasan, Milena;
Chen, Jie;
Gaughan, Christina;
Asthana, Abhishek;
Libri, Valentina;
[...]
Inborn errors of OAS–RNase L in SARS-CoV-2–related multisystem inflammatory syndrome in children
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- Medientyp: E-Artikel
- Titel: Inborn errors of OAS–RNase L in SARS-CoV-2–related multisystem inflammatory syndrome in children
- Beteiligte: Lee, Danyel; Le Pen, Jérémie; Yatim, Ahmad; Dong, Beihua; Aquino, Yann; Ogishi, Masato; Pescarmona, Rémi; Talouarn, Estelle; Rinchai, Darawan; Zhang, Peng; Perret, Magali; Liu, Zhiyong; Jordan, Iolanda; Elmas Bozdemir, Sefika; Bayhan, Gulsum Iclal; Beaufils, Camille; Bizien, Lucy; Bisiaux, Aurelie; Lei, Weite; Hasan, Milena; Chen, Jie; Gaughan, Christina; Asthana, Abhishek; Libri, Valentina; [...]
- Erschienen: American Association for the Advancement of Science (AAAS), 2023
- Erschienen in: Science
- Sprache: Englisch
- DOI: 10.1126/science.abo3627
- ISSN: 0036-8075; 1095-9203
- Entstehung:
- Anmerkungen:
- Beschreibung: <jats:p> Multisystem inflammatory syndrome in children (MIS-C) is a rare and severe condition that follows benign COVID-19. We report autosomal recessive deficiencies of <jats:italic>OAS1</jats:italic> , <jats:italic>OAS2</jats:italic> , or <jats:italic>RNASEL</jats:italic> 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. </jats:p>