Augmentation of Stimulator of Interferon Genes-Induced Type I Interferon Production in COPA Syndrome.
Takashi KatoMasaki YamamotoYoshitaka HondaTakashi OrimoIzumi SasakiKohei MurakamiHiroaki HemmiYuri Fukuda-OhtaKyoichi IsonoSaki TakayamaHidenori NakamuraYoshiro OtsukiToshiaki MiyamotoJunko TakitaTakahiro YasumiRyuta NishikomoriTadashi MatsubayashiKazushi IzawaTsuneyasu KaishoPublished in: Arthritis & rheumatology (Hoboken, N.J.) (2021)
V242G, a novel COPA variant, was found in 4 patients from one family. In gene-targeted mice with the V242G variant, interstitial lung disease was recapitulated and augmented responses of the STING pathway, leading to an increase in type I IFN production, were demonstrated.
Keyphrases
- interstitial lung disease
- systemic sclerosis
- dendritic cells
- end stage renal disease
- genome wide
- ejection fraction
- newly diagnosed
- chronic kidney disease
- rheumatoid arthritis
- immune response
- genome wide identification
- diabetic rats
- dna methylation
- cancer therapy
- metabolic syndrome
- drug induced
- patient reported outcomes
- oxidative stress
- stress induced