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http://purl.uniprot.org/citations/34380029http://www.w3.org/1999/02/22-rdf-syntax-ns#typehttp://purl.uniprot.org/core/Journal_Citation
http://purl.uniprot.org/citations/34380029http://www.w3.org/2000/01/rdf-schema#comment"Loss of function of adenosine deaminase acting on double-stranded RNA (dsRNA)-1 (ADAR1) causes the severe autoinflammatory disease Aicardi-Goutières syndrome (AGS). ADAR1 converts adenosines into inosines within dsRNA. This process called A-to-I editing masks self-dsRNA from detection by the antiviral dsRNA sensor MDA5. ADAR1 binds to dsRNA in both the canonical A-form and the poorly defined Z conformation (Z-RNA). Mutations in the Z-RNA-binding Zα domain of ADAR1 are common in patients with AGS. How loss of ADAR1/Z-RNA interaction contributes to disease development is unknown. We demonstrate that abrogated binding of ADAR1 to Z-RNA leads to reduced A-to-I editing of dsRNA structures formed by base pairing of inversely oriented short interspersed nuclear elements. Preventing ADAR1 binding to Z-RNA triggers an MDA5/MAVS-mediated type I interferon response and leads to the development of lethal autoinflammation in mice. This shows that the interaction between ADAR1 and Z-RNA restricts sensing of self-dsRNA and prevents AGS development."xsd:string
http://purl.uniprot.org/citations/34380029http://purl.org/dc/terms/identifier"doi:10.1016/j.celrep.2021.109500"xsd:string
http://purl.uniprot.org/citations/34380029http://purl.uniprot.org/core/author"Vandenabeele P."xsd:string
http://purl.uniprot.org/citations/34380029http://purl.uniprot.org/core/author"Maelfait J."xsd:string
http://purl.uniprot.org/citations/34380029http://purl.uniprot.org/core/author"Staes K."xsd:string
http://purl.uniprot.org/citations/34380029http://purl.uniprot.org/core/author"Van Nieuwerburgh F."xsd:string
http://purl.uniprot.org/citations/34380029http://purl.uniprot.org/core/author"Lambrecht B.N."xsd:string
http://purl.uniprot.org/citations/34380029http://purl.uniprot.org/core/author"de Reuver R."xsd:string
http://purl.uniprot.org/citations/34380029http://purl.uniprot.org/core/author"Dierick E."xsd:string
http://purl.uniprot.org/citations/34380029http://purl.uniprot.org/core/author"Botzki A."xsd:string
http://purl.uniprot.org/citations/34380029http://purl.uniprot.org/core/author"De Meester E."xsd:string
http://purl.uniprot.org/citations/34380029http://purl.uniprot.org/core/author"Wiernicki B."xsd:string
http://purl.uniprot.org/citations/34380029http://purl.uniprot.org/core/author"Muyldermans T."xsd:string
http://purl.uniprot.org/citations/34380029http://purl.uniprot.org/core/author"Seys L."xsd:string
http://purl.uniprot.org/citations/34380029http://purl.uniprot.org/core/date"2021"xsd:gYear
http://purl.uniprot.org/citations/34380029http://purl.uniprot.org/core/name"Cell Rep"xsd:string
http://purl.uniprot.org/citations/34380029http://purl.uniprot.org/core/pages"109500"xsd:string
http://purl.uniprot.org/citations/34380029http://purl.uniprot.org/core/title"ADAR1 interaction with Z-RNA promotes editing of endogenous double-stranded RNA and prevents MDA5-dependent immune activation."xsd:string
http://purl.uniprot.org/citations/34380029http://purl.uniprot.org/core/volume"36"xsd:string
http://purl.uniprot.org/citations/34380029http://www.w3.org/2004/02/skos/core#exactMatchhttp://purl.uniprot.org/pubmed/34380029
http://purl.uniprot.org/citations/34380029http://xmlns.com/foaf/0.1/primaryTopicOfhttps://pubmed.ncbi.nlm.nih.gov/34380029
http://purl.uniprot.org/uniprot/#_A0A0A6YXF5-mappedCitation-34380029http://www.w3.org/1999/02/22-rdf-syntax-ns#objecthttp://purl.uniprot.org/citations/34380029
http://purl.uniprot.org/uniprot/#_A0A024DAK3-mappedCitation-34380029http://www.w3.org/1999/02/22-rdf-syntax-ns#objecthttp://purl.uniprot.org/citations/34380029
http://purl.uniprot.org/uniprot/#_D3YYR0-mappedCitation-34380029http://www.w3.org/1999/02/22-rdf-syntax-ns#objecthttp://purl.uniprot.org/citations/34380029