Tonic prime-boost of STING signalling mediates Niemann-Pick disease type C.

Chu, Ting-Ting; Tu, Xintao; Yang, Kun; Wu, Jianjun; Repa, Joyce J; Yan, Nan

Chu, Ting-Ting; Tu, Xintao; Yang, Kun; Wu, Jianjun; Repa, Joyce J; Yan, Nan.

Affiliation

Chu TT; Department of Immunology, University of Texas Southwestern Medical Center, Dallas, TX, USA.
Tu X; Department of Immunology, University of Texas Southwestern Medical Center, Dallas, TX, USA.
Yang K; Department of Immunology, University of Texas Southwestern Medical Center, Dallas, TX, USA.
Wu J; Department of Immunology, University of Texas Southwestern Medical Center, Dallas, TX, USA.
Repa JJ; Department of Physiology, University of Texas Southwestern Medical Center, Dallas, TX, USA.
Yan N; Department of Internal Medicine, University of Texas Southwestern Medical Center, Dallas, TX, USA.

Nature ; 596(7873): 570-575, 2021 08.

Article in En | MEDLINE | ID: mdl-34290407

Subject(s)

Membrane Proteins/metabolism; Models, Biological; Niemann-Pick Disease, Type C/metabolism; Signal Transduction; Animals; Cell Line; Cerebellum/pathology; Endoplasmic Reticulum/metabolism; Golgi Apparatus/metabolism; Humans; Interferon Regulatory Factor-3/metabolism; Interferon Type I/immunology; Lysosomes/metabolism; Membrane Proteins/deficiency; Membrane Proteins/genetics; Mice; Mice, Inbred BALB C; Mice, Inbred C57BL; Microglia/metabolism; Motor Skills; Neuroinflammatory Diseases; Niemann-Pick C1 Protein/deficiency; Niemann-Pick C1 Protein/genetics; Niemann-Pick C1 Protein/metabolism; Niemann-Pick Disease, Type C/pathology; Nucleotides, Cyclic/metabolism; Nucleotidyltransferases/metabolism; Protein Serine-Threonine Kinases/metabolism; Protein Transport; Proteolysis; Purkinje Cells/metabolism; Sterol Regulatory Element Binding Protein 2/metabolism

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Full text: 1 Collection: 01-internacional Database: MEDLINE Main subject: Signal Transduction / Niemann-Pick Disease, Type C / Membrane Proteins / Models, Biological Type of study: Prognostic_studies Limits: Animals / Humans Language: En Journal: Nature Year: 2021 Document type: Article Affiliation country: United States

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