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GeneE
26 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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ISG15
ISG15 ubiquitin like modifier
Chromosome 1 · 1p36.33
NCBI Gene: 9636Ensembl: ENSG00000187608.11HGNC: HGNC:4053UniProt: P05161
286PubMed Papers
21Diseases
0Drugs
4Pathogenic Variants
FUNCTIONAL ROLE
Hub Gene
RESEARCH IMPACT
Trending
CLINICAL
OMIM Disease Gene
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
protein localization to mitochondrionnegative regulation of type I interferon-mediated signaling pathwaydefense response to virusprotein tag activityCOVID-19bilateral striopallidodentate calcinosisinfluenzaneoplasm
✦AI Summary

ISG15 (Interferon-Stimulated Gene 15) is a ubiquitin-like protein that plays dual roles in antiviral immunity and inflammatory regulation through both conjugated (ISGylation) and unconjugated forms 1. As an interferon-induced gene, ISG15 exhibits potent antiviral activity against DNA and RNA viruses including HIV-1, influenza A, and Ebola virus by disrupting viral budding, inhibiting viral protein function, and preventing virion assembly 1. The protein functions through ISGylation, a post-translational modification process where ISG15 is conjugated to target proteins via enzymatic cascades similar to ubiquitination 2. Key ISGylation targets include viral sensors like IFIH1/MDA5, promoting oligomerization and innate immune activation, and STING, where ISGylation at K289 is crucial for oligomerization and DNA sensing responses 3. However, ISG15 also serves as a negative regulator of interferon signaling by stabilizing USP18, preventing excessive IFN-α/β responses and autoinflammation 4. ISG15 deficiency in humans paradoxically causes enhanced interferon immunity and autoinflammatory symptoms rather than increased viral susceptibility 4. Beyond immunity, ISG15 contributes to pathological processes including cancer stem cell maintenance through mitochondrial regulation 5, adverse cardiac remodeling 6, and kidney injury progression 7, making it both a protective and pathogenic factor depending on context 2.

Sources cited
1
ISG15 exhibits antiviral activity against multiple viruses and functions through both conjugated and unconjugated forms
PMID: 29769653
2
ISG15 is an interferon-induced ubiquitin-like protein that undergoes ISGylation similar to ubiquitination
PMID: 35159348
3
ISGylation of STING at K289 is crucial for oligomerization and DNA sensing responses
PMID: 37864791
4
ISG15 serves as a negative regulator of interferon signaling and prevents autoinflammation through USP18 stabilization
PMID: 25307056
5
ISG15 contributes to cancer stem cell maintenance through mitochondrial regulation
PMID: 32472071
6
ISG15 promotes adverse cardiac remodeling and heart failure development
PMID: 37115698
7
ISG15 accelerates kidney injury progression through TGFβR1 ISGylation
PMID: 39113797
Disease Associationsⓘ21
COVID-19Open Targets
0.47Moderate
bilateral striopallidodentate calcinosisOpen Targets
0.46Moderate
influenzaOpen Targets
0.37Weak
neoplasmOpen Targets
0.12Weak
infectionOpen Targets
0.12Weak
cancerOpen Targets
0.11Weak
hepatocellular carcinomaOpen Targets
0.11Weak
systemic lupus erythematosusOpen Targets
0.11Weak
breast cancerOpen Targets
0.10Weak
nasopharyngeal carcinomaOpen Targets
0.10Suggestive
ovarian cancerOpen Targets
0.10Suggestive
oral squamous cell carcinomaOpen Targets
0.10Suggestive
colorectal carcinomaOpen Targets
0.10Suggestive
pachyonychia congenitaOpen Targets
0.09Suggestive
viral diseaseOpen Targets
0.09Suggestive
posterior cortical atrophyOpen Targets
0.09Suggestive
listeriosisOpen Targets
0.09Suggestive
acute kidney injuryOpen Targets
0.08Suggestive
head and neck squamous cell carcinomaOpen Targets
0.08Suggestive
lung adenocarcinomaOpen Targets
0.08Suggestive
Immunodeficiency 38, with basal ganglia calcificationUniProt
Pathogenic Variants4
NM_005101.4(ISG15):c.4-1G>APathogenic
Mendelian susceptibility to mycobacterial diseases due to complete ISG15 deficiency
★★☆☆2024
NM_005101.4(ISG15):c.379G>T (p.Glu127Ter)Likely pathogenic
Mendelian susceptibility to mycobacterial diseases due to complete ISG15 deficiency
★☆☆☆2023→ Residue 127
NM_005101.4(ISG15):c.339dup (p.Leu114fs)Pathogenic
Mendelian susceptibility to mycobacterial diseases due to complete ISG15 deficiency
☆☆☆☆2015→ Residue 114
NM_005101.4(ISG15):c.163C>T (p.Gln55Ter)Pathogenic
Mendelian susceptibility to mycobacterial diseases due to complete ISG15 deficiency
☆☆☆☆2015→ Residue 55
View on ClinVar ↗
Related Genes
IFI6Protein interaction100%IFI27Protein interaction100%IFIT2Protein interaction100%IFIT1Protein interaction100%IFIT3Protein interaction100%IFNAR1Protein interaction100%
Tissue Expression6 tissues
Bone Marrow
100%
Liver
56%
Lung
47%
Brain
21%
Heart
13%
Ovary
11%
Gene Interaction Network
Click a node to explore
ISG15IFI6IFI27IFIT2IFIT1IFIT3IFNAR1
PROTEIN STRUCTURE
Preparing viewer…
PDB6FFA · 1.50 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
1.80LoF Tolerant
pLIⓘ
0.11Tolerant
Observed/Expected LoF0.77 [0.30–1.80]
RankingsWhere ISG15 stands among ~20K protein-coding genes
  • #1,257of 20,598
    Most Researched286 · top 10%
  • #3,686of 5,498
    Most Pathogenic Variants4
  • #16,582of 17,882
    Most Constrained (LOEUF)1.80
Genes detectedISG15
Sources retrieved26 papers
Response time—
📄 Sources
26▼
1
ISG15 and ISGylation in Human Diseases.
PMID: 35159348
Cells · 2022
1.00
2
ISG15 in antiviral immunity and beyond.
PMID: 29769653
Nat Rev Microbiol · 2018
0.90
3
ISG15 accelerates acute kidney injury and the subsequent AKI-to-CKD transition by promoting TGFβR1 ISGylation.
PMID: 39113797
Theranostics · 2024
0.80
4
Human intracellular ISG15 prevents interferon-α/β over-amplification and auto-inflammation.
PMID: 25307056
Nature · 2015
0.70
5
ISG15 blocks cardiac glycolysis and ensures sufficient mitochondrial energy production during Coxsackievirus B3 infection.
PMID: 38309955
Cardiovasc Res · 2024
0.64