http://purl.uniprot.org/citations/26320659 | http://www.w3.org/1999/02/22-rdf-syntax-ns#type | http://purl.uniprot.org/core/Journal_Citation |
http://purl.uniprot.org/citations/26320659 | http://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/26320659 | http://purl.org/dc/terms/identifier | "doi:10.1016/j.immuni.2015.07.022"xsd:string |
http://purl.uniprot.org/citations/26320659 | http://purl.uniprot.org/core/author | "Jiang S."xsd:string |
http://purl.uniprot.org/citations/26320659 | http://purl.uniprot.org/core/author | "Miyazaki T."xsd:string |
http://purl.uniprot.org/citations/26320659 | http://purl.uniprot.org/core/author | "Sakai T."xsd:string |
http://purl.uniprot.org/citations/26320659 | http://purl.uniprot.org/core/author | "Hasan M."xsd:string |
http://purl.uniprot.org/citations/26320659 | http://purl.uniprot.org/core/author | "Gao N."xsd:string |
http://purl.uniprot.org/citations/26320659 | http://purl.uniprot.org/core/author | "Yan N."xsd:string |
http://purl.uniprot.org/citations/26320659 | http://purl.uniprot.org/core/author | "Atkinson J.P."xsd:string |
http://purl.uniprot.org/citations/26320659 | http://purl.uniprot.org/core/author | "Li Q.Z."xsd:string |
http://purl.uniprot.org/citations/26320659 | http://purl.uniprot.org/core/author | "Lehrman M.A."xsd:string |
http://purl.uniprot.org/citations/26320659 | http://purl.uniprot.org/core/author | "Morse H.C."xsd:string |
http://purl.uniprot.org/citations/26320659 | http://purl.uniprot.org/core/author | "Fermaintt C.S."xsd:string |
http://purl.uniprot.org/citations/26320659 | http://purl.uniprot.org/core/date | "2015"xsd:gYear |
http://purl.uniprot.org/citations/26320659 | http://purl.uniprot.org/core/name | "Immunity"xsd:string |
http://purl.uniprot.org/citations/26320659 | http://purl.uniprot.org/core/pages | "463-474"xsd:string |
http://purl.uniprot.org/citations/26320659 | http://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/26320659 | http://purl.uniprot.org/core/volume | "43"xsd:string |
http://purl.uniprot.org/citations/26320659 | http://www.w3.org/2004/02/skos/core#exactMatch | http://purl.uniprot.org/pubmed/26320659 |
http://purl.uniprot.org/citations/26320659 | http://xmlns.com/foaf/0.1/primaryTopicOf | https://pubmed.ncbi.nlm.nih.gov/26320659 |
http://purl.uniprot.org/uniprot/Q91XB0#attribution-8A4ECCAF46F365B3486BD0ABEFBA3110 | http://purl.uniprot.org/core/source | http://purl.uniprot.org/citations/26320659 |
http://purl.uniprot.org/uniprot/Q91XB0#attribution-B4AEA9B4E5E32A4C62662BCE1E151D31 | http://purl.uniprot.org/core/source | http://purl.uniprot.org/citations/26320659 |
http://purl.uniprot.org/uniprot/#_A0A125R9I3-mappedCitation-26320659 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/26320659 |
http://purl.uniprot.org/uniprot/#_D3Z5R0-mappedCitation-26320659 | http://www.w3.org/1999/02/22-rdf-syntax-ns#object | http://purl.uniprot.org/citations/26320659 |