The DOI matches its Crossref record; 31 of the 31 references it cites were found in a registry or an index.
Human intracellular ISG15 prevents interferon-α/β over-amplification and auto-inflammation
Open accessAbstract
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.
Overview
The claims and statistics of this paper haven’t been checked yet.
Run the check — free, no account needed. It reads the open-access text and checks the sources it cites.
Integrity signals
Registry
Cites
Statistics
Verification
Claims the paper makes
The claims and statistics of this paper haven’t been checked yet.
Run the check — free, no account needed. It reads the open-access text and checks the sources it cites.
Claims other papers make about this one
Each is checked against this paper’s own text: does it say what the citing paper says it does?
The cited source says what the claim attributes to it.
In the citing paper
“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]
The check wasn’t confident either way, so it gives no verdict — never disputed.
In the citing paper
“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).”
From The liver as a central "hub" of the immune system: pathophysiological implications (2025) · cites this paper as [49]
Experimental — accuracy not yet measured
Self-check
Percentages, sums and reported differences re-computed from the paper’s own numbers.
The self-check needs the paper’s full text.
Citations
Cites
Baechler, E. C., Batliwalla, F., Karypis, G., Gaffney, P. M., Ortmann, W. A., Espe, K. J., Shark, K. B., Grande, W. J., Hughes, K. M. H., Kapur, V., et al. (2003) · Proceedings of the National Academy of Sciences · doi:10.1073/pnas.0337679100 · Cited by 2,246
Interferon and Granulopoiesis Signatures in Systemic Lupus Erythematosus Blood
Bennett, L. B., Palucka, K., Arce, E., Cantrell, V., Borvák, J., Banchereau, J., & Pascual, V. (2003) · The Journal of Experimental Medicine · doi:10.1084/jem.20021553 · Cited by 1,990
Genetic Dissection of Immunity to Mycobacteria: The Human Model
Casanova, J., & Abel, L. (2002) · Annual Review of Immunology · doi:10.1146/annurev.immunol.20.081501.125851 · Cited by 1,006
Self-citationDeregulated proteolysis by the F-box proteins SKP2 and β-TrCP: tipping the scales of cancer
Frescas, D., & Pagano, M. (2008) · Nature reviews. Cancer · doi:10.1038/nrc2396 · Cited by 903
Mycobacterial Disease and Impaired IFN-γ Immunity in Humans with Inherited ISG15 Deficiency
Bogunovic, D., Byun, M., Durfee, L. A., Abhyankar, A., Sanal, O., Mansouri, D., Salem, S., Radovanovic, I., Grant, A. V., Adimi, P., et al. (2012) · Science · doi:10.1126/science.1224026 · Cited by 572
Self-citationRice, G., Forte, G. M. A., Szynkiewicz, M., Chase, D. S., Aeby, A., Abdel‐Hamid, M. S., Ackroyd, S., Allcock, R., Bailey, K. M., Balottin, U., et al. (2013) · The Lancet Neurology · doi:10.1016/s1474-4422(13)70258-8 · Cited by 444
Self-citationUBP43 is a novel regulator of interferon signaling independent of its ISG15 isopeptidase activity
Malakhova, O. A., Kim, K. I. I., Luo, J.-K., Zou, W., Kumar, K. G., Fuchs, S. Y., Shuai, K., & Zhang, D. (2006) · The EMBO Journal · doi:10.1038/sj.emboj.7601149 · Cited by 443
Mutations in SLC20A2 link familial idiopathic basal ganglia calcification with phosphate homeostasis
Wang, C., Li, Y., Shi, L., Ren, J., Patti, M., Wang, T., de Oliveira, J. R. M., Sobrido, M. J., Quintáns, B., Baquero, M., et al. (2012) · Nature Genetics · doi:10.1038/ng.1077 · Cited by 417
Self-citationWhat is and what is not ‘Fahr's disease’
Manyam, B. V. (2005) · Parkinsonism & Related Disorders · doi:10.1016/j.parkreldis.2004.12.001 · Cited by 395
Interferon-Stimulated Gene 15 and the Protein ISGylation System
Zhang, D., & Zhang, D. (2010) · Journal of Interferon & Cytokine Research · doi:10.1089/jir.2010.0110 · Cited by 361
Flagged first, then by citations
Cited by
Sorted by citations
Items 401–500 of 563
Chen, F., Yao, C., Feng, Y., Yu, Y., Guo, H., Yan, J., & Chen, J. (2021) · Medicine · doi:10.1097/md.0000000000024669 · Cited by 5
Ubiquitin-Specific Peptidase 18: A Multifaceted Protein Participating in Breast Cancer
Tecalco-Cruz, Á. C., Ramírez‐Jarquín, J. O., Zepeda–Cervantes, J., Solleiro-Villavicencio, H., & Abraham‐Juárez, M. J. (2022) · Exon Publications eBooks · doi:10.36255/exon-publications-breast-cancer-usp18 · Cited by 5
Yu, F., Le, Z.-S., Chen, L., Qian, H., Yu, B., & Chen, W. (2019) · Journal of Computational Biology · doi:10.1089/cmb.2019.0151 · Cited by 5
Monogenetic causes of chilblains, panniculitis and vasculopathy: the Type I interferonopathies
Papa, R., Volpi, S., & Gattorno, M. (2020) · Giornale Italiano di Dermatologia e Venereologia · doi:10.23736/s0392-0488.20.06709-7 · Cited by 5
Self-citationDeveloping Broad-Spectrum Antivirals Using Porcine and Rhesus Macaque Models
Qiu, X., Taft, J. D., & Bogunovic, D. (2019) · The Journal of Infectious Diseases · doi:10.1093/infdis/jiz549 · Cited by 5
Self-citationNucleic acid-mediated autoinflammation and autoimmunity—type I interferonopathies
Lee‐Kirsch, M. A., Günther, C., & Roers, A. (2016) · Journal of Molecular Medicine · doi:10.1007/s00109-016-1467-3 · Cited by 5
The Genetic Architecture of Human Infectious Diseases and Pathogen-Induced Cellular PhenotypesPreprint
Hale, A. T., Zhou, D., Sale, R. L., Bastarache, L. A., Wang, L., Zinkel, S. S., Schiff, S. J., Ko, D. C., & Gamazon, E. R. (2020) · SSRN Electronic Journal · doi:10.2139/ssrn.3707276 · Cited by 5
Zhang, X., Xiong, W., Ma, S., Fan, S., Sun, M., Zhou, Q., Yang, J., Li, J., & Zhang, X. (2025) · Gene · doi:10.1016/j.gene.2025.149537 · Cited by 4
Self-citationThe emerging roles of ubiquitin-like modifications in regulating HIV replication and host defense
Yuan, J., Wang, H., Sun, X., & Huan, C. (2025) · Frontiers in Cellular and Infection Microbiology · doi:10.3389/fcimb.2025.1593445 · Cited by 5
Innate immune sensing of macromolecule homeostasis
Yang, K., Jeltema, D., & Yan, N. (2024) · Advances in immunology · doi:10.1016/bs.ai.2024.03.004 · Cited by 4
Waman, V., Ashford, P., Lam, S. D., Sen, N., Abbasian, M., Woodridge, L., Goldtzvik, Y., Bordin, N., Wu, J., Sillitoe, I., et al. (2024) · Scientific Reports · doi:10.1038/s41598-024-61541-1 · Cited by 7
Su, L.-H., Tsai, W., Shirakawa, H., Tsai, Y., Yeh, S., & Yeh, C. (2025) · Nutrients · doi:10.3390/nu17101700 · Cited by 4
Overview of Mendelian Susceptibility to Mycobacterial Diseases (MSMD)
Raghuraman, K., Rajeswarie, S., Rajkhowa, P., & Kaushik, J. S. (2025) · Cureus · doi:10.7759/cureus.85872 · Cited by 4
ISGylation orchestrates the degradation flux of cellular cargo toward lysosome
Huang, M., Zhang, J., Ouyang, L., & Wang, Y. (2021) · Genes & Diseases · doi:10.1016/j.gendis.2021.09.003 · Cited by 4
Vergneault, H., Pïcard, C., & Georgin‐Lavialle, S. (2022) · Immunology · doi:10.1111/imm.13579 · Cited by 4
Impacts of the SARS-CoV-2 Spike Protein on the Innate Immune System: A Review
Bocquet-Garçon, A. (2024) · Cureus · doi:10.7759/cureus.57008 · Cited by 4
Proteomic Signatures of Monocytes in Hereditary Recurrent Fevers
Penco, F., Petretto, A., Lavarello, C., Papa, R., Bertoni, A., Omenetti, A., Gueli, I., Finetti, M., Caorsi, R., Volpi, S., et al. (2022) · Frontiers in Immunology · doi:10.3389/fimmu.2022.921253 · Cited by 4
Self-citationInterferon-Stimulated Gene 15 Knockout in Mice Impairs IFNα-Mediated Antiviral Activity
Li, C., He, W., Li, L., Chen, J., Yang, G., Chang, H., & Liu, H. (2022) · Viruses · doi:10.3390/v14091862 · Cited by 4
Infection and autoinflammation in inborn errors of immunity: brothers in arms
Delafontaine, S., & Meyts, I. (2021) · Current Opinion in Immunology · doi:10.1016/j.coi.2021.08.004 · Cited by 5
Bonacci, T., & Emanuele, M. J. (2025) · Biochemical Society Transactions · doi:10.1042/bst20240859 · Cited by 3
Gan, J., Dabhade, P., Wijne, C., McKibben, W., Draganov, S. D., Alrawili, H. A. H., Sun, Z.-Y. J., Houghton, J. W., Tate, E. W., Gall, C. M. L., et al. (2025) · Journal of Biological Chemistry · doi:10.1016/j.jbc.2025.110564 · Cited by 3
Yu, J., Guo, W., Gong, X.-Y., Luan, H.-Y., Wu, M., Zhao, Z.-Y., Dan, C., & Zhang, Y.-B. (2025) · Fish & Shellfish Immunology · doi:10.1016/j.fsi.2025.110931 · Cited by 3
The multifaceted roles of ISG15 in cancer: Conjugated and free forms
Liu, X.-R., Ma, Q., Zhao, J., Zou, C., & Huang, K. (2025) · Biochimica et Biophysica Acta (BBA) - Reviews on Cancer · doi:10.1016/j.bbcan.2025.189473 · Cited by 3
Su, Y., Zhang, R., Li, K., Shen, H., Nan, M., Liu, C., Zhang, W., & Chen, S. (2025) · Cell & Bioscience · doi:10.1186/s13578-025-01487-7 · Cited by 3
Mapping the plasma proteomic architecture of systemic lupus erythematosus
Leung, G. H. D., Bottomley, C., Buang, N. B., Maughan, R. T., Whittle, B. J., Zeidaabadi, B., Huang, R. Y., Turner‐Stokes, T., Condon, M. B., Lightstone, L., et al. (2026) · JCI Insight · doi:10.1172/jci.insight.206938 · Cited by 3
Context-Specific Diversity of Antimicrobial Functions of Interferon-Stimulated Genes
Harioudh, M. K., & Sarkar, S. N. (2025) · Viruses · doi:10.3390/v17121635 · Cited by 3
Proteomics and tracer metabolomics link GAPDH ISGylation to glycolytic control
Eggermont, D., Eggermont, L., Aslantas, N., Théry, F., Boucher, K. L., Beling, A., Ghesquière, B., & Impens, F. (2026) · Genome biology · doi:10.1186/s13059-026-04034-w · Cited by 3
Sarkar, R., Patra, U., Mukherjee, A., Mitra, S., Komoto, S., & Chawla‐Sarkar, M. (2023) · Cellular Signalling · doi:10.1016/j.cellsig.2023.110891 · Cited by 3
Abdel–Haq, H. (2024) · Vaccines · doi:10.3390/vaccines12060583 · Cited by 3
Sun, J., Pan, C.-L., Mao, Y., Wang, Z., Sun, J., Zhang, X., Yang, Y., Wei, Z., & Xu, Y. (2024) · International Journal of Radiation Biology · doi:10.1080/09553002.2024.2386982 · Cited by 3
Self-citationUbiquitin-like Proteins in Autoimmune Diseases: Current Evidence and Therapeutic Opportunities
Lee, B., Jeong, H., Kim, Y.-R., Roh, J. S., & Sohn, D. H. (2025) · Immune Network · doi:10.4110/in.2025.25.e21 · Cited by 4
Fernández-Sendin, M., Trani, C. A. D., Bella, Á., Vásquez, M., Ardaiz, N., Gomar, C., Arrizabalaga, L., Ciordia, S., Corrales, F. J., Aranda, F., et al. (2021) · Frontiers in Immunology · doi:10.3389/fimmu.2020.620283 · Cited by 3
Autoinflammation – Unterschiede bei Kindern und Erwachsenen
Krusche, M., & Kallinich, T. (2021) · Zeitschrift für Rheumatologie · doi:10.1007/s00393-021-01115-y · Cited by 3
Mendelian disorders of immunity related to an upregulation of type I interferon
Frémond, M., & Crow, Y. J. (2020) · Elsevier eBooks · doi:10.1016/b978-0-12-816768-7.00032-6 · Cited by 3
Self-citationFamotidine increases cellular phospho-tyrosine levels
Zubiete‐Franco, I., & Tonks, N. K. (2024) · Biochemical and Biophysical Research Communications · doi:10.1016/j.bbrc.2024.150763 · Cited by 3
Helicobacter pylori provokes STING immunosurveillance via trans-kingdom conjugationPreprint
Damke, P. P., Wood, C. R., & Shaffer, C. L. (2022) · bioRxiv (Cold Spring Harbor Laboratory) · doi:10.1101/2022.06.29.498044 · Cited by 3
PLpro Inhibitors as a Potential Treatment for COVID-19
Han, Y., & Xu, F. (2025) · Biomedicines · doi:10.3390/biomedicines13061417 · Cited by 3
Immunogenetics of the Ocular Anterior Segment: Lessons from Inherited Disorders
Serpen, J. Y., Armenti, S. T., & Prasov, L. (2021) · Journal of Ophthalmology · doi:10.1155/2021/6691291 · Cited by 2
Wu, R., Shao, F., Koh, S., Okoli, U. A., Li, D., Wang, J., Tuo, Z., Zhang, R., Wusiman, D., Cheema, U., et al. (2025) · Cellular & Molecular Biology Letters · doi:10.1186/s11658-025-00815-6 · Cited by 2
Stark, D. S., King, M., Ibler, A. E., Srour, S., Brown, F., Sheffield, J. W., Baseer, N., Vernon, E. G., ZHANG, Y., Staples, C. J., et al. (2026) · Molecular Microbiology · doi:10.1111/mmi.70067 · Cited by 2
Self-citationWhen ISG15 is involved in inflammation
Feng, X., Qiao, Y., & Cui, S. (2026) · Frontiers in Immunology · doi:10.3389/fimmu.2026.1775143 · Cited by 2
Type I Interferonopathies: Fifteen Years On, From Concept to Therapeutic Perspectives
Ünal, D., Melki, I., & Frémond, M. (2026) · Current Rheumatology Reports · doi:10.1007/s11926-026-01228-w · Cited by 2
Expression of isgylation related genes in regenerating rat liver
Kuklin, A. V., Poliezhaieva, T. A., Zhyryakova, I. O., Ogryzko, V. V., & Obolenskaya, M. Y. (2015) · Biopolymers and Cell · doi:10.7124/bc.0008f7 · Cited by 2
Hu, J., Zhang, Y., Zheng, S., Chen, G.-R., Lin, Y., Kang, D., Lin, Z., & Yao, P. (2023) · Journal of Psychiatric Research · doi:10.1016/j.jpsychires.2023.05.070 · Cited by 2
Wang, C. S. (2023) · Rheumatic Disease Clinics of North America · doi:10.1016/j.rdc.2023.06.002 · Cited by 2
Liu, S., Zhang, Z., Tang, X., Zhao, X., & An, Y. (2024) · Pediatric Discovery · doi:10.1002/pdi3.67 · Cited by 2
Yao, J.-C., Zhang, J., Wang, X., Wu, Y.-H., Ke, H., Liang, J.-R., Shao, Y., Li, J.-T., Liu, Y., Cai, D., et al. (2025) · Frontiers in Bioengineering and Biotechnology · doi:10.3389/fbioe.2025.1550584 · Cited by 2
Self-citationUSP18 is a key regulator of immune function in mouse midbrain microglia
Lü, L., Jifu, C., Pei, B., Gao, X., Xu, Z., Yin, X., Xia, J., & Wang, J. (2025) · Life Sciences · doi:10.1016/j.lfs.2025.123692 · Cited by 3
Interferons in human inborn errors of disease
Stine, L., Cahill, S. E., & Humphries, F. (2025) · mBio · doi:10.1128/mbio.01570-25 · Cited by 2
ISG15 — human deficiency reveals new proficiency
Shipman, L. (2014) · Nature reviews. Immunology · doi:10.1038/nri3767 · Cited by 2
Liao, X., Wang, Y., Ye, H., Li, S., Chen, L., & Duan, X. (2018) · Future Virology · doi:10.2217/fvl-2017-0160 · Cited by 2
Yamamoto, K., & Mukae, H. (2018) · Respiratory disease series · doi:10.1007/978-981-10-8144-6_10 · Cited by 2
Erratum: Human intracellular ISG15 prevents interferon-α/β over-amplification and auto-inflammation
Zhang, X., Bogunovic, D., Payelle‐Brogard, B., François-Newton, V., Speer, S. D., Yuan, C., Volpi, S., Li, Z., Sanal, Ö., Mansouri, D., et al. (2015) · Nature · doi:10.1038/nature14271 · Cited by 2
Self-citationMacrophages govern antiviral responses in human lung tissues protected from SARS-CoV-2 infectionPreprint
Kenney, D., O’Connell, A. K., Turcinovic, J., Montanaro, P., Hekman, R. M., Tamura, T., Berneshawi, A. R., Cafiero, T. R., Abdullatif, S. A., Blum, B. C., et al. (2021) · bioRxiv (Cold Spring Harbor Laboratory) · doi:10.1101/2021.07.17.452554 · Cited by 2
Jové, V., Wheeler, H., Lee, C. W., Healy, D. R., Levine, K. K., Ralph, E. C., Yang, B., Giddabasappa, A., Loria, P. M., Yamaguchi, M., et al. (2023) · bioRxiv (Cold Spring Harbor Laboratory) · doi:10.1101/2023.03.23.533924 · Cited by 2
Key pathways in primary immune deficiencies
Hanson, E. P., Uzel, G., & Hambleton, S. (2020) · Elsevier eBooks · doi:10.1016/b978-0-12-816768-7.00003-x · Cited by 1
Yang, Z., Wei, J., He, Y., Luo, R., Chen, S., Deng, Y., Zang, N., & Liu, E. (2023) · Archives of Virology · doi:10.1007/s00705-023-05707-8 · Cited by 1
Case Report: ISG15 Deficiency and a Glimpse Into Autoimmunity
Akbar, L., Alawam, B. S., Alhassan, M., Faisal, S. Y., Al-Omari, M. A., & Al-Mayouf, S. M. (2024) · International Journal of Rheumatic Diseases · doi:10.1111/1756-185x.15420 · Cited by 1
Mueller, Y. M., Girard, C., van de Werken, H. J. G., Erkeland, S. J., Grashof, D. G. B., Vaillant, L., den Dekker, R. J. H., van Zuijen, I., Veenbergen, S., Coffin, F., et al. (2026) · Arthritis & Rheumatology · doi:10.1002/art.70102 · Cited by 1
Zhang, Q., Gong, Z., Schnell, R., Zhong, Y., Geurink, P. P., Lauschke, V. M., Xie, J., Gastaldello, S., & Rottenberg, M. (2026) · Frontiers in Immunology · doi:10.3389/fimmu.2026.1739628 · Cited by 1
Ubiquitin-like modifications in adipose tissue development and obesity: New insights
Chakraborty, S., & Chen, W. (2026) · Biochimica et Biophysica Acta (BBA) - Molecular Basis of Disease · doi:10.1016/j.bbadis.2026.168234 · Cited by 1
Lin, X., Chen, G., Tang, R., Wu, M., Zhang, D., Lin, F., Guan, J., Yang, J., Dong, X., Zheng, X., et al. (2026) · Cell Reports Medicine · doi:10.1016/j.xcrm.2026.102754 · Cited by 1
ISG15/ISGylation in central nervous system diseases: molecular mechanisms and therapeutic targeting
Chen, X., Xiao, X., Luo, W., Liu, H., Liu, M., Sima, X., & Gao, L. (2026) · Biochemical Pharmacology · doi:10.1016/j.bcp.2026.118057 · Cited by 1
Self-citationISG15 negatively regulates RIPK3-mediated cell death and viral pathogenesis
Perng, Y.-C., Warsaw, J. M. C., Bais, S. S., Hiller, B. E., Locke, M. C., Morales-Heil, D. J., Li, C., Young, A. R., Monte, K. J., Schmidt, R. E., et al. (2026) · Cell Reports · doi:10.1016/j.celrep.2026.117360 · Cited by 1
Janus Kinase Inhibitors in Treatment of Primary Immune Regulatory Disorders
Alsaati, N., & Satter, L. F. (2026) · The Journal of Allergy and Clinical Immunology In Practice · doi:10.1016/j.jaip.2026.05.019 · Cited by 1
Familial pulmonary alveolar proteinosis and type I interferonopathy by mutation of STAT2 (TIMS2)
Gruber, C. N., Ramba, M., Debnath, B., Cuollo, L., Neehus, A., Lee, A. E., Buta, S., Martín-Fernández, M., Rosain, J., Berteloot, L., et al. (2026) · The Journal of Experimental Medicine · doi:10.1084/jem.20251331 · Cited by 1
Self-citationJuvenal, G. D. A., Meinerz, C., Ayupe, A. C., Campos, H. C., Reis, E. M. R., Longo, B. M., Pillat, M. M., & Ulrich, H. (2024) · Cell & Bioscience · doi:10.1186/s13578-024-01251-3 · Cited by 1
ISG15 depletion enhances oHSV-1 replication and antitumor efficacy in oral squamous cell carcinoma
Qiu, M., Wei, R., Zhang, Q., Zhao, J., Zhang, H., Tan, J., & Qiao, W. (2025) · Virology · doi:10.1016/j.virol.2025.110504 · Cited by 1
Li, Z., Peng, M., Cheng, L., Wang, Z., Wu, Z., Feng, F., Feng, X., Wang, S., Guo, Y., & Li, Y. (2025) · Virology Journal · doi:10.1186/s12985-025-02696-9 · Cited by 1
Self-citationImmunodeficiencies at the Interface of Innate and Adaptive Immunity
Bustamante, J. C., Zhang, S., Boisson, B., Ciancanelli, M. J., Jouanguy, E., Boisson‐Dupuis, S., Puel, A., Pïcard, C., & Casanova, J. (2019) · Elsevier eBooks · doi:10.1016/b978-0-7020-6896-6.00036-3 · Cited by 2
Self-citationType I Interferonopathies: From Pathophysiology to Clinical Expression
Flessa, C., Argiriou, E., & Mavragani, C. P. (2019) · Springer eBooks · doi:10.1007/978-3-319-96929-9_10 · Cited by 1
Eleftheriou, D., Torrelo, A., & Brogan, P. A. (2019) · Springer eBooks · doi:10.1007/978-3-319-98605-0_24 · Cited by 1
Borges-Medeiros, R. L., & de Oliveira, J. R. M. (2017) · Journal of Human Genetics · doi:10.1038/s10038-017-0390-y · Cited by 1
Mogensen, T. H. (2020) · Clinical Infectious Diseases · doi:10.1093/cid/ciaa1798 · Cited by 1
Biological Consequences of Protein Moonlighting
Henderson, B. E., Fares, M. A., & Martin, A. C. R. (2016) · Wiley eBooks · doi:10.1002/9781118952108.ch6 · Cited by 1
Gupta, A. K., Wu, J., Das, P. K., Sharma, R., Das, S., Han, K., Klein, R. J., Huffstutler, R. D., Combs, C. A., Leonard, W. J., et al. (2026) · bioRxiv (Cold Spring Harbor Laboratory) · doi:10.64898/2026.01.19.700051 · Cited by 1
Dissecting the interactions of the ISG15-USP18-STAT2 inhibitory complexPreprint
Rowe, J. C., Ng, Y. M., Kiefer, H., Simmons, M., Paul, M., Sundaramoorthy, R., Hughes, D. J., & Swatek, K. N. (2026) · bioRxiv (Cold Spring Harbor Laboratory) · doi:10.64898/2026.03.26.714284 · Cited by 1
Klinische Symptome und Pathogenese der Typ-1-Interferonopathien
Diesterheft, M., Schuster, M., Rösen‐Wolff, A., Berner, R., Tüngler, V., & Lee‐Kirsch, M. A. (2015) · Monatsschrift Kinderheilkunde · doi:10.1007/s00112-015-3478-5
Autoimmune Diseases Arising out of Single Gene Defects
Peterson, P., & Kisand, K. E. (2016) · Elsevier eBooks · doi:10.1016/b978-0-12-374279-7.15010-6
Mendelian Susceptibility to Infections with Viruses, Mycobacteria, Bacteria, and Candida
Pïcard, C., Bustamante, J. C., Puel, A., & Zhang, S. (2016) · Elsevier eBooks · doi:10.1016/b978-0-12-374279-7.18021-x
Self-citationQuoi de neuf en dermatologie pédiatrique ?
Maruani, A. (2015) · Annales de Dermatologie et de Vénéréologie · doi:10.1016/s0151-9638(16)30004-7
Diseases Caused by Genetic or Congenital Defects in the Immune System or Skin Immune System
Gutierrez, A., Pittelkow, M. R., Xi, L., Huang, X., & Rai, S. (2016) · Springer eBooks · doi:10.1007/978-94-024-0902-4_11
Meeting summary: 2nd Aegean Conference on Cytokine Signaling in Cancer
Ilangumaran, S., Williams, B. R. G., & Kalvakolanu, D. V. (2017) · Cytokine · doi:10.1016/j.cyto.2017.10.025
Schlechter, N. (2017) · SUNScholar (Stellenbosch University)
Characterization Of Epigenetic Plasticity And Chromatin Dynamics In Cancer Cell Models
Gerrard, D. L. (2019) · ScholarWorks -A service of University of Vermont Libraries (University of Vermont)
The impact of sex and species of cells in herpes simplex virus infection
Tseng, Y. (2019) · ANU Open Research (Australian National University) · doi:10.25911/5d77831416351
Defects in Intrinsic and Innate Immunity
Alsina, L., Rodríguez‐Gallego, C., Esteve‐Solé, A., Vlagea, A. D., de Diego, R. P., Martínez‐Barricarte, R., & Deyà‐Martínez, À. (2021) · Rare diseases of the immune system · doi:10.1007/978-3-030-70107-9_8
Functional Testing of the IL-12/IFN-γ Circuit
Alsina, L., Esteve‐Solé, A., Rodríguez‐Gallego, C., & Vlagea, A. D. (2021) · Elsevier eBooks · doi:10.1016/b978-0-12-818731-9.00156-7
Autoinflammation – Unterschiede bei Kindern und Erwachsenen
Krusche, M., & Kallinich, T. (2022) · rheuma plus · doi:10.1007/s12688-022-00534-2
ISG15-modification of the Arp2/3 complex restricts pathogen spreadPreprint
Zhang, Y., Ripley, B. M., Ouyang, W., Sturtz, M., Upton, E. M., Luhmann, E. K., Vessely, M., Coloma, R., Schwery, N., Anthony, S. M., et al. (2022) · bioRxiv (Cold Spring Harbor Laboratory) · doi:10.1101/2022.12.27.522022
Self-citationXu, Q., Li, W., zhao, Q., Lv, G., Sun, G., Gao, Y., Ding, Y., Zhang, Z., Zhou, L., Tang, X., et al. (2022) · Research Square · doi:10.21203/rs.3.rs-2391818/v1
Interféronopathies de type I : d’un nouveau concept aux thérapeutiques ciblées
Melki, I., & Frémond, M. (2023) · Perfectionnement en Pédiatrie · doi:10.1016/j.perped.2023.04.003
Interferone als Schlüsselfaktoren für die Pathogenese, Diagnostik und Therapie
Hinze, C. H., Wittkowski, H., Kessel, C., Varga, G., & Föll, D. (2023) · Monatsschrift Kinderheilkunde · doi:10.1007/s00112-023-01787-7
Enhanced ISGylation reduces respiratory distress following Francisella novicida infectionPreprint
Upton, E. M., Luhmann, E. K., Zhang, Y., Ripley, B. M., Meyerholz, D. K., & Radoshevich, L. (2023) · bioRxiv (Cold Spring Harbor Laboratory) · doi:10.1101/2023.09.19.558558
Self-citationMelki, I., Frémond, M., & Crow, Y. J. (2022) · Elsevier eBooks · doi:10.1016/b978-2-294-77580-2.00024-4
Self-citationMa, Y., Shan, S., Luo, C., Mo, J., Hu, Z., & Jing, R. (2023) · European Journal of Inflammation · doi:10.1177/1721727x231202661
Chen, Y., Li, R., Li, Z., Yang, B., He, J., Li, J., Li, P., Zhou, Z., Wu, Y., Zhao, Y., et al. (2024) · Experimental and Therapeutic Medicine · doi:10.3892/etm.2024.12517
Self-citationQu, T., Zhang, W., Yan, C., Ren, D., Wang, Y., Guo, Y., Guo, Q., Wang, J., Liu, L., Han, L., et al. (2023) · Figshare · doi:10.6084/m9.figshare.23084055
Single gene defects and autoinflammation
Moore, R. E., Hagiwara, A. M., & Jefferies, C. A. (2024) · Elsevier eBooks · doi:10.1016/b978-0-323-93232-5.00015-0
Progress in research of ubiquitination modification of hepatitis B surface antigen
Niu, A.-X., Liu, J., & Zhu, C. (2024) · World Chinese Journal of Digestology · doi:10.11569/wcjd.v32.i5.333
Self-citation
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
About these checks
Registry, retraction, Cites and statistics results are computed, not judged, with one exception: a printed reference that no index lists is read once by a model before it counts as a citation error. Claims are extracted by a model and kept only when their quote appears verbatim in the text; each cited source is then checked, with a model’s help, for whether it says what the claim relies on. Those verdicts can be wrong — read the claim’s quote and the cited source before relying on one.
Statistics and arithmetic are recomputed, not judged.
Confidence bands are shown only for fields calibrated on our annotated test set. None is calibrated yet, so no band is shown.
What counts as a claim ›How we measure confidence ›How we check this ›
Are you an author, or do you see a problem with this page? Request a review
