Evidence map›Paper›PMID 35484149›Full record

ArticleNature communications2022

Deficiency in coatomer complex I causes aberrant activation of STING signalling.

Annemarie Steiner, Katja Hrovat-Schaale, Ignazia Prigione, Chien-Hsiung Yu, Pawat Laohamonthonkul, Cassandra R Harapas, Ronnie Ren Jie Low, Dominic De Nardo, Laura F Dagley, Michael J Mlodzianoski and 9 more

Open access · goldAbstract read
In one paragraph

Article in Nature communications, 2022. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 65 papers.

0numbers the graph read from it
0cells of the map it votes in
65citing papers in PubMed
7.1field-weighted citation impact, top 2% of its field
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

65 citing papers in PubMed, 90 citations in OpenAlex.

  1. COPI Coatomer Regulates Several Steps of HDL Metabolism.Arteriosclerosis, thrombosis, and vascular biology · 2026
    Article
  2. Article
  3. Review
  4. Article
  5. Article
  6. Interstitial lung disease and the STING pathway.The Journal of clinical investigation · 2026
    Review
  7. Cell autonomous inflammation in VEXAS is mediated by cGAS-STING.bioRxiv : the preprint server for biology · 2026
    Article
  8. Article
  9. Article
  10. Article
  11. Article
  12. STING Innate Immunity Signalling from the Golgi.Sub-cellular biochemistry · 2026
    Review
  13. Genetic Familial Interstitial Lung Disease.Clinics in chest medicine · 2025
    Review
  14. Article
  15. Review
  16. Review
  17. Article
  18. STING COPII ER Export Trafficking and Signaling Primed by Phosphorylation Switches.Advanced science (Weinheim, Baden-Wurttemberg, Germany) · 2025
    Article
  19. Article
  20. NADCell death & disease · 2025
    Article

5 more citing papers are in PubMed but not listed here.

4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

19 authors at 6 institutions in 4 countries.

Annemarie SteinerInflammation Division, The Walter and Eliza Hall Institute of Medical Research, Parkville, VIC, 3052, Australia.
Katja Hrovat-SchaaleInflammation Division, The Walter and Eliza Hall Institute of Medical Research, Parkville, VIC, 3052, Australia.ORCID 0000-0002-0398-5015
Ignazia PrigioneCentre for Autoinflammatory Diseases and Primary Immunodeficiencies, IRCCS Istituto Giannina Gaslini, 16147, Genoa, Italy.
Chien-Hsiung YuInflammation Division, The Walter and Eliza Hall Institute of Medical Research, Parkville, VIC, 3052, Australia.ORCID 0000-0003-1744-3791
Pawat LaohamonthonkulInflammation Division, The Walter and Eliza Hall Institute of Medical Research, Parkville, VIC, 3052, Australia.
Cassandra R HarapasInflammation Division, The Walter and Eliza Hall Institute of Medical Research, Parkville, VIC, 3052, Australia.
Ronnie Ren Jie LowDepartment of Medical Biology, University of Melbourne, Parkville, VIC, 3010, Australia.ORCID 0000-0002-2629-4778
Dominic De NardoInflammation Division, The Walter and Eliza Hall Institute of Medical Research, Parkville, VIC, 3052, Australia.ORCID 0000-0002-7045-4695
Laura F DagleyDepartment of Medical Biology, University of Melbourne, Parkville, VIC, 3010, Australia.ORCID 0000-0003-4171-3712
Michael J MlodzianoskiCenter for Dynamic Imaging, The Walter and Eliza Hall Institute of Medical Research, Parkville, VIC, 3052, Australia.ORCID 0000-0002-3510-9167
Kelly L RogersCenter for Dynamic Imaging, The Walter and Eliza Hall Institute of Medical Research, Parkville, VIC, 3052, Australia.ORCID 0000-0002-6755-0221
Thomas ZillingerInstitute of Clinical Chemistry and Clinical Pharmacology, University Hospital Bonn, 53127, Bonn, Germany.ORCID 0000-0002-0866-4835
Gunther HartmannInstitute of Clinical Chemistry and Clinical Pharmacology, University Hospital Bonn, 53127, Bonn, Germany.ORCID 0000-0003-1021-2018
Michael P GantierCentre for Innate Immunity and Infectious Diseases, Hudson Institute of Medical Research, Clayton, VIC, 3168, Australia.
Marco GattornoCentre for Autoinflammatory Diseases and Primary Immunodeficiencies, IRCCS Istituto Giannina Gaslini, 16147, Genoa, Italy.
Matthias GeyerInstitute of Structural Biology, University Hospital Bonn, 53127, Bonn, Germany.ORCID 0000-0002-7718-5002
Stefano VolpiCentre for Autoinflammatory Diseases and Primary Immunodeficiencies, IRCCS Istituto Giannina Gaslini, 16147, Genoa, Italy.ORCID 0000-0002-7129-868X
Sophia Davidson *Inflammation Division, The Walter and Eliza Hall Institute of Medical Research, Parkville, VIC, 3052, Australia.
Seth L Masters *Inflammation Division, The Walter and Eliza Hall Institute of Medical Research, Parkville, VIC, 3052, Australia. masters@wehi.edu.au.ORCID 0000-0003-4763-576X
The University of Melbourne · AUIstituto Giannina Gaslini · ITWalter and Eliza Hall Institute of Medical Research · AUUniversity Hospital Bonn · DEHudson Institute of Medical Research · AUPhilipps University of Marburg · DE

Funding

Howard Hughes Medical InstituteWellcome Trust 208694/Z/17/Z
6 · The paper itself

Abstract

Coatomer complex I (COPI) mediates retrograde vesicular trafficking from Golgi to the endoplasmic reticulum (ER) and within Golgi compartments. Deficiency in subunit alpha causes COPA syndrome and is associated with type I IFN signalling, although the upstream innate immune sensor involved was unknown. Using in vitro models we find aberrant activation of the STING pathway due to deficient retrograde but probably not intra-Golgi transport. Further we find the upstream cytosolic DNA sensor cGAS as essentially required to drive type I IFN signalling. Genetic deletion of COPI subunits COPG1 or COPD similarly induces type I IFN activation in vitro, which suggests that inflammatory diseases associated with mutations in other COPI subunit genes may exist. Finally, we demonstrate that inflammation in COPA syndrome patient peripheral blood mononuclear cells and COPI-deficient cell lines is ameliorated by treatment with the small molecule STING inhibitor H-151, suggesting targeted inhibition of the cGAS/STING pathway as a promising therapeutic approach.

Indexed as

Leukocytes, MononuclearNucleotidyltransferasesCoat Protein Complex ICOP-Coated VesiclesElectron Transport Complex IHumansSignal TransductionCoat Protein Complex IElectron Transport Complex INucleotidyltransferases

Identifiers

PMID35484149
PMCPMC9051092
OpenAlexW4225141722

What Socratic holds

Textmetadata
LicenceCC BY
Read underepoch 390

Registered trials

None linked

Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the Socratic graph.