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Inborn errors of OAS-RNase L in SARS-CoV-2-related multisystem inflammatory syndrome in children
Science (American Association for the Advancement of Science), 2023-02, Vol.379 (6632), p.eabo3627-eabo3627
2023

Details

Autor(en) / Beteiligte
Titel
Inborn errors of OAS-RNase L in SARS-CoV-2-related multisystem inflammatory syndrome in children
Ist Teil von
  • Science (American Association for the Advancement of Science), 2023-02, Vol.379 (6632), p.eabo3627-eabo3627
Ort / Verlag
United States: The American Association for the Advancement of Science
Erscheinungsjahr
2023
Link zum Volltext
Quelle
American Association for the Advancement of Science
Beschreibungen/Notizen
  • Multisystem inflammatory syndrome in children (MIS-C) is a rare and severe condition that follows benign COVID-19. We report autosomal recessive deficiencies of , , or 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.
Sprache
Englisch
Identifikatoren
ISSN: 0036-8075, 1095-9203
eISSN: 1095-9203
DOI: 10.1126/science.abo3627
Titel-ID: cdi_swepub_primary_oai_prod_swepub_kib_ki_se_236538032
Format
–
Schlagworte
Asymptomatic, Biomarkers, CD4 antigen, CD8 antigen, Cell activation, Cell differentiation, Cell lines, Child, Children, Coding, Coronaviruses, COVID-19, COVID-19 - immunology, Cytokines, Cytokines - genetics, Cytokines - immunology, Defects, Dendritic cells, Double-stranded RNA, Endoribonucleases - genetics, Endoribonucleases - metabolism, Epithelial cells, Epithelium, Errors, Etiology, Fibroblasts, Gene sequencing, Genes, Genetics, Genomes, Heart diseases, Homogeneity, Humans, Immunology, Individualized Instruction, Infections, Inflammation, Interferon, Kawasaki disease, Leukocytes (mononuclear), Life Sciences, Lymphadenopathy, Lymphocytes, Lymphocytes T, Medicin och hälsovetenskap, Melanoma, Mucocutaneous lymph node syndrome, Multisystem inflammatory syndrome in children, Pain, Patients, Phagocytes, Phenotypes, Pneumonia, Proteins, Receptors, Respiratory diseases, Retinoic acid, Ribonucleic acid, RNA, RNA, Double-Stranded, SARS-CoV-2 - genetics, Severe acute respiratory syndrome coronavirus 2, Signal transduction, Signaling, Systemic Inflammatory Response Syndrome - genetics, T cell receptors, Viral diseases

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