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http://purl.uniprot.org/citations/26320659http://www.w3.org/1999/02/22-rdf-syntax-ns#typehttp://purl.uniprot.org/core/Journal_Citation
http://purl.uniprot.org/citations/26320659http://www.w3.org/2000/01/rdf-schema#comment"TREX1 is an endoplasmic reticulum (ER)-associated negative regulator of innate immunity. TREX1 mutations are associated with autoimmune and autoinflammatory diseases. Biallelic mutations abrogating DNase activity cause autoimmunity by allowing immunogenic self-DNA to accumulate, but it is unknown how dominant frameshift (fs) mutations that encode DNase-active but mislocalized proteins cause disease. We found that the TREX1 C terminus suppressed immune activation by interacting with the ER oligosaccharyltransferase (OST) complex and stabilizing its catalytic integrity. C-terminal truncation of TREX1 by fs mutations dysregulated the OST complex, leading to free glycan release from dolichol carriers, as well as immune activation and autoantibody production. A connection between OST dysregulation and immune disorders was demonstrated in Trex1(-/-) mice, TREX1-V235fs patient lymphoblasts, and TREX1-V235fs knock-in mice. Inhibiting OST with aclacinomycin corrects the glycan and immune defects associated with Trex1 deficiency or fs mutation. This function of the TREX1 C terminus suggests a potential therapeutic option for TREX1-fs mutant-associated diseases."xsd:string
http://purl.uniprot.org/citations/26320659http://purl.org/dc/terms/identifier"doi:10.1016/j.immuni.2015.07.022"xsd:string
http://purl.uniprot.org/citations/26320659http://purl.uniprot.org/core/author"Jiang S."xsd:string
http://purl.uniprot.org/citations/26320659http://purl.uniprot.org/core/author"Miyazaki T."xsd:string
http://purl.uniprot.org/citations/26320659http://purl.uniprot.org/core/author"Sakai T."xsd:string
http://purl.uniprot.org/citations/26320659http://purl.uniprot.org/core/author"Hasan M."xsd:string
http://purl.uniprot.org/citations/26320659http://purl.uniprot.org/core/author"Gao N."xsd:string
http://purl.uniprot.org/citations/26320659http://purl.uniprot.org/core/author"Yan N."xsd:string
http://purl.uniprot.org/citations/26320659http://purl.uniprot.org/core/author"Atkinson J.P."xsd:string
http://purl.uniprot.org/citations/26320659http://purl.uniprot.org/core/author"Li Q.Z."xsd:string
http://purl.uniprot.org/citations/26320659http://purl.uniprot.org/core/author"Lehrman M.A."xsd:string
http://purl.uniprot.org/citations/26320659http://purl.uniprot.org/core/author"Morse H.C."xsd:string
http://purl.uniprot.org/citations/26320659http://purl.uniprot.org/core/author"Fermaintt C.S."xsd:string
http://purl.uniprot.org/citations/26320659http://purl.uniprot.org/core/date"2015"xsd:gYear
http://purl.uniprot.org/citations/26320659http://purl.uniprot.org/core/name"Immunity"xsd:string
http://purl.uniprot.org/citations/26320659http://purl.uniprot.org/core/pages"463-474"xsd:string
http://purl.uniprot.org/citations/26320659http://purl.uniprot.org/core/title"Cytosolic Nuclease TREX1 Regulates Oligosaccharyltransferase Activity Independent of Nuclease Activity to Suppress Immune Activation."xsd:string
http://purl.uniprot.org/citations/26320659http://purl.uniprot.org/core/volume"43"xsd:string
http://purl.uniprot.org/citations/26320659http://www.w3.org/2004/02/skos/core#exactMatchhttp://purl.uniprot.org/pubmed/26320659
http://purl.uniprot.org/citations/26320659http://xmlns.com/foaf/0.1/primaryTopicOfhttps://pubmed.ncbi.nlm.nih.gov/26320659
http://purl.uniprot.org/uniprot/Q91XB0#attribution-8A4ECCAF46F365B3486BD0ABEFBA3110http://purl.uniprot.org/core/sourcehttp://purl.uniprot.org/citations/26320659
http://purl.uniprot.org/uniprot/Q91XB0#attribution-B4AEA9B4E5E32A4C62662BCE1E151D31http://purl.uniprot.org/core/sourcehttp://purl.uniprot.org/citations/26320659
http://purl.uniprot.org/uniprot/#_A0A125R9I3-mappedCitation-26320659http://www.w3.org/1999/02/22-rdf-syntax-ns#objecthttp://purl.uniprot.org/citations/26320659
http://purl.uniprot.org/uniprot/#_D3Z5R0-mappedCitation-26320659http://www.w3.org/1999/02/22-rdf-syntax-ns#objecthttp://purl.uniprot.org/citations/26320659