A defect in COPI-mediated transport of STING causes immune dysregulation in COPA syndrome

Pathogenic COPA variants cause a Mendelian syndrome of immune dysregulation with elevated type I interferon signaling. COPA is a subunit of coat protein complex I (COPI) that mediates Golgi to ER transport. Missense mutations of the COPA WD40 domain impair binding and sorting of proteins targeted fo...

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Detalles Bibliográficos
Autores principales: Deng, Zimu, Chong, Zhenlu, Law, Christopher S., Mukai, Kojiro, Ho, Frances O., Martinu, Tereza, Backes, Bradley J., Eckalbar, Walter L., Taguchi, Tomohiko, Shum, Anthony K.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Rockefeller University Press 2020
Materias:
Brief Definitive Report
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7596814/
https://www.ncbi.nlm.nih.gov/pubmed/32725126
http://dx.doi.org/10.1084/jem.20201045

Internet

https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7596814/
https://www.ncbi.nlm.nih.gov/pubmed/32725126
http://dx.doi.org/10.1084/jem.20201045

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