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10 sources retrieved · Most recent: April 2026 · Index updated 14 days ago
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HERC6
HECT and RLD domain containing E3 ubiquitin protein ligase family member 6
Chromosome 4 · 4q22.1
NCBI Gene: 55008Ensembl: ENSG00000138642.17HGNC: HGNC:26072UniProt: Q8IVU3
24PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
cytosolubiquitin protein ligase activityubiquitin-dependent protein catabolic processcytoplasmnephritisNephropathyprotozoa infectious diseasedisease of peritoneum
✦AI Summary

HERC6 is an E3 ubiquitin-protein ligase that functions as a critical regulator of innate antiviral immunity through ISGylation-mediated post-translational modification 1. As an interferon-stimulated gene (ISG) product, HERC6 catalyzes the covalent attachment of ISG15 to multiple pattern recognition receptors and signaling proteins, stabilizing them against proteasomal degradation 1234. Specifically, HERC6 mediates ISGylation of STING at K150, preventing K48-linked ubiquitination and promoting type I interferon responses to viral pathogens like herpes simplex virus 1 1. Additionally, HERC6 enhances NLRP3 inflammasome activation through ISGylation, controlling inflammatory responses during infection 2. HERC6 also facilitates cGAS and MDA5 activation, amplifying antiviral signaling cascades 34. Evolutionary analysis reveals HERC6 has undergone strong adaptive evolution in mammals, suggesting historical pathogen-driven selection 5. Disease relevance includes lupus nephritis, where HERC6 emerges as a hub gene linked to immune activation and inflammation 6. HERC6 deficiency impairs antiviral defenses and increases susceptibility to viral infection, highlighting its clinical significance in host-pathogen interactions 13.

Sources cited
1
HERC6 mediates ISGylation of STING at K150, preventing K48-linked ubiquitination and facilitating type I IFN responses; HERC6 deficiency suppresses antiviral responses to herpes simplex virus 1
PMID: 38652662
2
HERC6 identified as a hub gene in lupus nephritis with high diagnostic accuracy (AUC=0.999), associated with inflammation response and immune activation
PMID: 38130724
3
HERC6 promotes NLRP3 ISGylation, inhibits K48-linked ubiquitination and proteasomal degradation, enhancing inflammasome activation; HERC6 deficiency ameliorates NLRP3-dependent inflammation
PMID: 37651190
4
HERC6 (murine homolog of human HERC5) catalyzes cGAS ISGylation, promoting DNA-induced oligomerization and enhancing cGAS enzymatic activity; HERC6 deficiency increases susceptibility to herpes simplex virus 1
PMID: 38421872
5
HERC6 has undergone strong and recurrent adaptive evolution in mammals, suggesting widespread genetic arms-races with viral pathogens; HERC6 spacer domain may represent a host-pathogen interface
PMID: 33391270
6
HERC6 (human HERC5 homolog) mediates MDA5 ISGylation within CARD domains, promoting MDA5 oligomerization and antiviral signaling; essential for controlling viral replication and pathogenesis
PMID: 40184173
Disease Associationsⓘ20
nephritisOpen Targets
0.33Weak
NephropathyOpen Targets
0.31Weak
protozoa infectious diseaseOpen Targets
0.31Weak
disease of peritoneumOpen Targets
0.22Weak
ovarian neoplasmOpen Targets
0.21Weak
Abnormality of the gastrointestinal tractOpen Targets
0.20Weak
ThrombocytopeniaOpen Targets
0.06Suggestive
immunodeficiency 88Open Targets
0.06Suggestive
linear and whorled nevoid hypermelanosisOpen Targets
0.05Suggestive
autosomal dominant macrothrombocytopeniaOpen Targets
0.05Suggestive
essential thrombocythemiaOpen Targets
0.05Suggestive
eosinophil peroxidase deficiencyOpen Targets
0.05Suggestive
breast carcinomaOpen Targets
0.05Suggestive
chronic myeloproliferative disorderOpen Targets
0.05Suggestive
macrothrombocytopenia, isolated, 2, autosomal dominantOpen Targets
0.05Suggestive
Beta-thalassemia - X-linked thrombocytopeniaOpen Targets
0.05Suggestive
beta-thalassemia-X-linked thrombocytopenia syndromeOpen Targets
0.05Suggestive
thrombocytopenia 4Open Targets
0.05Suggestive
chronic myelogenous leukemiaOpen Targets
0.05Suggestive
X-linked sideroblastic anemia 1Open Targets
0.05Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
HERC3Shared pathway100%ARIH1Shared pathway100%ANKIB1Shared pathway100%RNF148Shared pathway100%RNF149Shared pathway100%RNF215Shared pathway100%
Tissue Expression6 tissues
Heart
100%
Ovary
66%
Bone Marrow
64%
Brain
63%
Lung
37%
Liver
27%
Gene Interaction Network
Click a node to explore
HERC6HERC3ARIH1ANKIB1RNF148RNF149RNF215
PROTEIN STRUCTURE
Preparing viewer…
PDB5W87 · 2.20 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
1.32LoF Tolerant
pLIⓘ
0.00Tolerant
Observed/Expected LoF1.08 [0.89–1.32]
RankingsWhere HERC6 stands among ~20K protein-coding genes
  • #13,203of 20,598
    Most Researched24
  • #13,846of 17,882
    Most Constrained (LOEUF)1.32
Genes detectedHERC6
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
ISGylation by HERCs facilitates STING activation.
PMID: 38652662
Cell Rep · 2024
1.00
2
Identification of driver genes in lupus nephritis based on comprehensive bioinformatics and machine learning.
PMID: 38130724
Front Immunol · 2023
0.90
3
Posttranslational ISGylation of NLRP3 by HERC enzymes facilitates inflammasome activation in models of inflammation.
PMID: 37651190
J Clin Invest · 2023
0.80
4
HERC5-catalyzed ISGylation potentiates cGAS-mediated innate immunity.
PMID: 38421872
Cell Rep · 2024
0.70
5
Rapid Evolution of HERC6 and Duplication of a Chimeric HERC5/6 Gene in Rodents and Bats Suggest an Overlooked Role of HERCs in Mammalian Immunity.
PMID: 33391270
Front Immunol · 2020
0.60