Article
Distinct pathogenic mutations in ARF1 allow dissection of its dual role in cGAS-STING signalling.
EMBO reports - 1 May 2025
Lang Johannes, Bergner Tim, Zinngrebe Julia, Lepelley Alice, Vill Katharina, Leiz Steffen, Wlaschek Meinhard, Wagner Matias, Scharffetter-Kochanek Karin, Fischer-Posovszky Pamela, Read Clarissa, Crow Yanick J, Hirschenberger Maximilian, Sparrer Konstantin M J
Abstract excerpt
Tight control of cGAS-STING-mediated DNA sensing is crucial to avoid auto-inflammation. The GTPase ADP-ribosylation factor 1 (ARF1) is crucial to maintain cGAS-STING homeostasis and various pathogenic ARF1 variants are associated with type I interferonopathies. Functional ARF1 inhibits STING activity by maintaining mitochondrial integrity and facilitating COPI-mediated retrograde STING trafficking and...
Topics
- Humans
- Nucleotidyltransferases
- ADP-Ribosylation Factor 1
- Signal Transduction
- Membrane Proteins
- Mutation
- Fibroblasts
- Interferon Type I
- Mitochondria
- HEK293 Cells