Evidence map›Paper›PMID 18606876›Full record

ArticleBlood2008

Interferon autoantibodies associated with AIRE deficiency decrease the expression of IFN-stimulated genes.

Kai Kisand, Maire Link, Anette S B Wolff, Anthony Meager, Liina Tserel, Tõnis Org, Astrid Murumägi, Raivo Uibo, Nick Willcox, Katarina Trebusak Podkrajsek and 12 more

Open access · bronzeAbstract read
In one paragraph

Article in Blood, 2008. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 55 papers, 1 of them a synthesis that pooled it.

0numbers the graph read from it
0cells of the map it votes in
55citing papers in PubMed, 1 pooled it
5.0field-weighted citation impact, top 4% 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

55 citing papers in PubMed, 1 synthesis or guideline pooled it, 129 citations in OpenAlex.

  1. Pooled it
  2. Trial
  3. Trial
  4. Article
  5. Article
  6. Article
  7. Long-term follow-up of autoimmune polyendocrine syndrome type 1 in Norway.The Journal of clinical endocrinology and metabolism · 2026
    Article
  8. Article
  9. Article
  10. Article
  11. Article
  12. Review
  13. Article
  14. Aire Mutations and Autoimmune Diseases.Advances in experimental medicine and biology · 2025
    Review
  15. Article
  16. Article
  17. Review
  18. Article
  19. The role of interferon in the thymus.Current opinion in immunology · 2023
    Review
  20. Article
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

22 authors at 13 institutions in 10 countries.

Kai KisandInstitute of General and Molecular Pathology, University of Tartu, Tartu, Estonia.
Maire Link
Anette S B Wolff
Anthony Meager
Liina Tserel
Tõnis Org
Astrid Murumägi
Raivo Uibo
Nick Willcox
Katarina Trebusak Podkrajsek
Tadej Battelino
Anna Lobell
Olle Kämpe
Kari Lima
Antonella Meloni
Berrin Ergun-Longmire
Noel K Maclaren
Jaakko Perheentupa
Kai J E Krohn
Hamish S Scott
Eystein S Husebye
Pärt Peterson
University of Tartu · EEUniversity of Bergen · NOUppsala University · SEAkershus University Hospital · NOJohnson University · USLjubljana University Medical Centre · SIMRC Weatherall Institute of Molecular Medicine · GBNational Institute for Biological Standards and Control · GBOspedale Microcitemico · ITResearch Centre for Medical Genetics · RUTampere University of Applied Sciences · FIUniversity of Adelaide · AUUniversity of Helsinki · FI

Funding

Wellcome Trust 067503
6 · The paper itself

Abstract

Neutralizing autoantibodies to type I, but not type II, interferons (IFNs) are found at high titers in almost every patient with autoimmune polyendocrinopathy candidiasis ectodermal dystrophy (APECED), a disease caused by AIRE gene mutations that lead to defects in thymic T-cell selection. Combining genome-wide expression array with real time RT-PCR assays, we here demonstrate that antibodies against IFN-alpha cause highly significant down-regulation of interferon-stimulated gene expression in cells from APECED patients' blood by blocking their highly dilute endogenous IFNs. This down-regulation was lost progressively as these APECED cells matured in cultures without neutralizing autoantibodies. Most interestingly, a rare APECED patient with autoantibodies to IFN-omega but not IFN-alpha showed a marked increase in expression of the same interferon-stimulated genes. We also report unexpected increases in serum CXCL10 levels in APECED. Our results argue that the breakdown of tolerance to IFNs in AIRE deficiency is associated with impaired responses to them in thymus, and highlight APECED as another autoimmune disease with associated dysregulation of IFN activity.

Indexed as

AdolescentAdultAIRE ProteinAutoantibodiesBlood CellsCase-Control StudiesCell LineChemokine CXCL10Dendritic CellsDown-RegulationFemaleHumansInterferonsInterferon Type IMaleMiddle AgedAIRE ProteinAutoantibodiesChemokine CXCL10InterferonsInterferon Type ISTAT1 Transcription FactorTranscription Factors

Identifiers

PMID18606876
PMCPMC2577576
OpenAlexW2014009872

What OpenQuestion holds

Textmetadata
LicenceCC BY-NC
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 OpenQuestion graph.