Article

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

2020-05-20

Abstract excerpt

Pathogenic COPA variants cause a Mendelian syndrome of immune dysregulation with elevated type I interferon signaling 1,2 . COPA is a subunit of coat protein complex I (COPI) that mediates Golgi to ER transport 3 . Missense mutations that disrupt the COPA WD40 domain impair binding and sorting of proteins targeted for retrieval to the ER but how this causes disease remains unknown 1,4 . Given the importance of...

Topics

Open a Topic to create a Post that cites this publication.

Identifiers and source

Literature Corpus work: e0f6fbaa-9b01-5983-b327-0e533842852c
DOI: 10.1101/2020.05.20.106500

Open publication

Related research

Semantic proximity does not establish scientific evidence.

Click a neighbor to travelStep 1 · 12 closest

A defect in COPI-mediated transport of STING causes immune dysregulation in COPA syndromeDOI 10.1101/2020.05.20.106500Read

Activation of STING due to COPI-deficiencySemantic proximity Read

Homeostatic regulation of STING by Golgi-to-ER membrane trafficSemantic proximity Read

STING trafficking as a new dimension of immune signalingSemantic proximity Read

Heterozygous mutations in the C-terminal domain of COPA underlie a complex autoinﬂammatory syndrome.Semantic proximity Read

STING-Mediated Lung Inflammation and Beyond.Semantic proximity Read

Augmentation of Stimulator of Interferon Genes-Induced Type I Interferon Production in COPA Syndrome.Semantic proximity Read

The common HAQ STING allele prevents clinical penetrance of COPA syndrome.Semantic proximity Read

The HAQ STING mutation interacts with COPA causing allosteric structural rearrangements in COPA that may protect against COPA diseaseSemantic proximity Read

STING: a multifaced player in cellular homeostasis.Semantic proximity Read

COPA mutations impair ER-Golgi transport and cause hereditary autoimmune-mediated lung disease and arthritis.Semantic proximity Read

Membrane traffic governs the STING inflammatory signallingSemantic proximity Read

YIPF5 Is Essential for Innate Immunity to DNA Virus and Facilitates COPII-Dependent STING TraffickingSemantic proximity Read