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Human intracellular ISG15 prevents interferon-α/β over-amplification and auto-inflammation
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Intracellular ISG15 is an interferon (IFN)-α/β-inducible ubiquitin-like modifier which can covalently bind other proteins in a process called ISGylation; it is an effector of IFN-α/β-dependent antiviral immunity in mice. We previously published a study describing humans with inherited ISG15 deficiency but without unusually severe viral diseases. We showed that these patients were prone to mycobacterial disease and that human ISG15 was non-redundant as an extracellular IFN-γ-inducing molecule. We show here that ISG15-deficient patients also display unanticipated cellular, immunological and clinical signs of enhanced IFN-α/β immunity, reminiscent of the Mendelian autoinflammatory interferonopathies Aicardi-Goutières syndrome and spondyloenchondrodysplasia. We further show that an absence of intracellular ISG15 in the patients' cells prevents the accumulation of USP18, a potent negative regulator of IFN-α/β signalling, resulting in the enhancement and amplification of IFN-α/β responses. Human ISG15, therefore, is not only redundant for antiviral immunity, but is a key negative regulator of IFN-α/β immunity. In humans, intracellular ISG15 is IFN-α/β-inducible not to serve as a substrate for ISGylation-dependent antiviral immunity, but to ensure USP18-dependent regulation of IFN-α/β and prevention of IFN-α/β-dependent autoinflammation.
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“Similarly, ISG15, an interferon-inducible protein necessary for viral protection in mice, does not have the same critical role in humans(48-50).”
From The liver as a central "hub" of the immune system: pathophysiological implications (2025) · cites this paper as [49]
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“Soluble TRAIL activates cell death pathways in hepatocytes in response to ethanol consumption and has been implicated in hepatic steatosis and hepatocyte death in ASH (49).”
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Metadata
- Authors
- Zhang, X., Bogunovic, D., Payelle-Brogard, B., Francois-Newton, V., Speer, S. D., Yuan, C., Volpi, S., Li, Z., Sanal, O., Mansouri, D., Tezcan, I., Rice, G. I., Chen, C., Mansouri, N., Mahdaviani, S. A., Itan, Y., Boisson, B., Okada, S., Zeng, L., Wang, X., Jiang, H., Liu, W., Han, T., Liu, D., Ma, T., Wang, B., Liu, M., Liu, J.-Y., Wang, Q. K., Yalnizoglu, D., Radoshevich, L., Uzé, G., Gros, P., Rozenberg, F., Zhang, S.-Y., Jouanguy, E., Bustamante, J., García-Sastre, A., Abel, L., Lebon, P., Notarangelo, L. D., Crow, Y. J., Boisson-Dupuis, S., Casanova, J.-L., & Pellegrini, S.
- Affiliations
- Huazhong University of Science and Technology (CN)
- Rockefeller University (US)
- Icahn School of Medicine at Mount Sinai (US)
- Boston Children's Hospital (US)
- Centre National de la Recherche Scientifique (FR)
- Masih Daneshvari Hospital (IR)
- Manchester Academic Health Science Centre (GB)
- BGI Group (China) (CN)
- Hubei Provincial Women and Children's Hospital (CN)
- Cleveland Clinic (US)
- Institut Pasteur (FR)
- McGill University (CA)
- Université Paris Descartes (FR)
- Hôpital Necker-Enfants Malades (FR)
- Inserm (FR)
- Corresponding author
- Bogunovic, Dusan
- ORCID
- Zhang, Xianqin
- Bogunovic, Dusan
- Speer, Scott D.
- Yuan, Chao
- Volpi, Stefano
- Li, Zhi
- Mansouri, Davood
- Rice, Gillian I.
- Mansouri, Nahal
- Mahdaviani, Seyed Alireza
- Itan, Yuval
- Boisson, Bertrand
- Okada, Satoshi
- Zeng, Lu
- Wang, Xing
- Jiang, Hui
- Han, Tiantian
- Liu, Delin
- Ma, Tao
- Liu, Mugen
- Wang, Qing K.
- Radoshevich, Lilliana
- Uzé, Gilles
- Gros, Philippe
- Rozenberg, Flore
- Zhang, Shen-Ying
- Jouanguy, Emmanuelle
- Bustamante, Jacinta
- Abel, Laurent
- Notarangelo, Luigi D.
- Crow, Yanick J.
- Casanova, Jean-Laurent
- Pellegrini, Sandra
- Venue
- Nature
- Published
- 2014-10-12
- Volume
- 517
- Issue
- 7532
- Pages
- 89-93
- ISSN
- 0028-0836 (ISSN-L)
- 1476-4687
- PMC
- PMC4303590
- OpenAlex
- W1601709990
- Cited by
- 558
- References
- 31
- Citations by year
- 2026: 45 · 2025: 36 · 2024: 56 · 2023: 57 · 2022: 43 · 2021: 49 · 2020: 57 · 2019: 35 · 2018: 36 · 2017: 58 · 2016: 43 · 2015: 42 · 2014: 1
- FWCI
- 20.31
- Type
- journal article
- Language
- en
- Update notices
- erratum (2015-02-11)
- Open access
- bronze
- doi.org
- Locations
- Nature (publishedVersion)
- PubMed (publishedVersion)
- Research Explorer (The University of Manchester) (publishedVersion)
- PubMed Central (acceptedVersion)
- HAL (submittedVersion)
- RePEc: Research Papers in Economics (submittedVersion)
- CINECA IRIS Institutial Research Information System (University of Genoa) (submittedVersion, other-oa)
- IRIS (submittedVersion)
- Hacettepe University Institutional Repository (hacettepe.edu.tr) (submittedVersion, other-oa)
- SERVAL (Université de Lausanne) (submittedVersion)
- Funders
- Howard Hughes Medical Institute
- European Commission (200732, 241779, 2007–2013, FP7 2007-2013, FP7/2007, 223608)
- Agence Nationale de la Recherche
- Institut National de la Santé et de la Recherche Médicale
- Ligue Contre le Cancer
- Centre National de la Recherche Scientifique
- Greater Manchester Comprehensive Local Research Network
- National Institutes of Health (UL1TR000043, FP7/2007-2013)
- Manchester Biomedical Research Centre
- Japan Society for the Promotion of Science (25713039, 25670477)
- National Institute of Allergy and Infectious Diseases (P01AI076210, R01AI035237, P01AI090935, 1PO1AI076210-01A1, U19AI083025, R00AI106942, R37AI095983)
- National Center for Research Resources
- National Center for Advancing Translational Sciences
- FP7 Ideas: European Research Council (309449)
- CHIST-ERA
- Topics
- Life Sciences › Immunology and Microbiology › Immunology › interferon and immune responses
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Inflammasome and immune disorders
- Life Sciences › Immunology and Microbiology › Immunology › Immunodeficiency and Autoimmune Disorders
- Keywords
- ISG15, USP18, type I interferon, autoinflammation, Aicardi-Goutières syndrome, interferonopathies, ISGylation, ubiquitin-like proteins, mycobacterial infection, interferon-gamma
- MeSH terms
- Adolescent, Alleles, Child, Female, Gene Expression Regulation, Humans, Inflammation, Interferon Type I, Male, Pedigree, Endopeptidases, Ubiquitins, Viruses, Signal Transduction, Cytokines, Intracellular Space, Ubiquitin Thiolesterase, S-Phase Kinase-Associated Proteins, Ubiquitination
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