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10 sources retrieved · Most recent: April 2026 · Index updated 15 days ago
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SENP8
SUMO peptidase family member, NEDD8 specific
Chromosome 15 · 15q23
NCBI Gene: 123228Ensembl: ENSG00000166192.16HGNC: HGNC:22992UniProt: A8K8D3
47PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
FUNCTIONAL ROLE
Protease
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
protein bindingdeNEDDylase activityprotein deneddylationpost-translational protein modificationgoutNeurodevelopmental abnormalityretinitis pigmentosaage-related macular degeneration
✦AI Summary

SENP8 (SUMO peptidase family member, NEDD8 specific) is a deNEDDylase enzyme that plays a critical role in regulating the NEDD8 conjugation pathway by removing NEDD8 modifications from target proteins 1. SENP8 demonstrates exquisite specificity for NEDD8 over ubiquitin, with substrate recognition primarily determined by residue differences at positions 51 and 72 2. The enzyme prevents aberrant neddylation of NEDD8 pathway components, including auto-neddylation of the E2 enzyme Ubc12, thereby maintaining proper cullin-RING ligase (CRL) function and proteostasis 1. SENP8 localizes predominantly to the cytoplasm in human cell lines, with some nuclear presence in certain contexts 3. Functionally, SENP8 serves as a proximal regulator of cullin neddylation, controlling inflammatory responses through NF-κB and HIF-1α pathways in endothelial cells 4. In cancer contexts, SENP8 mediates SHP2 deneddylation in response to CD47/SIRPα signaling, promoting tumor immunosuppression 5. Loss of SENP8 function leads to accumulation of CRL substrates, defective cell cycle progression, and can confer resistance to certain therapeutic agents by limiting CRL4 activity 16. The enzyme's activity can be downregulated by viral proteins like adenovirus E4orf6, facilitating viral-mediated degradation of host restriction factors 7.

Sources cited
1
SENP8 prevents aberrant neddylation of NEDD8 pathway components and maintains CRL function
PMID: 28475037
2
SENP8 shows specificity for NEDD8 over ubiquitin based on residue differences at positions 51 and 72
PMID: 22110750
3
SENP8 localizes predominantly to cytoplasm in human ocular cell lines
PMID: 30636609
4
SENP8 regulates cullin neddylation and controls inflammatory responses via NF-κB and HIF-1α pathways
PMID: 23209320
5
SENP8 mediates SHP2 deneddylation in CD47/SIRPα signaling pathway promoting tumor immunosuppression
PMID: 36626230
6
SENP8 inactivation can confer drug resistance by limiting CRL4 activity
PMID: 31300418
7
Viral protein E4orf6 downregulates SENP8 expression to facilitate host protein degradation
PMID: 31301771
Disease Associationsⓘ20
goutOpen Targets
0.17Weak
Neurodevelopmental abnormalityOpen Targets
0.11Weak
retinitis pigmentosaOpen Targets
0.10Weak
age-related macular degenerationOpen Targets
0.09Suggestive
X-linked retinal dysplasiaOpen Targets
0.08Suggestive
acute lymphoblastic leukemiaOpen Targets
0.07Suggestive
Progressive cone dystrophyOpen Targets
0.07Suggestive
hypertensionOpen Targets
0.07Suggestive
response to xenobiotic stimulusOpen Targets
0.07Suggestive
Familial exudative vitreoretinopathyOpen Targets
0.07Suggestive
choroidal dystrophy, central areolar, 1Open Targets
0.06Suggestive
Abruptio PlacentaeOpen Targets
0.06Suggestive
severe early-childhood-onset retinal dystrophyOpen Targets
0.06Suggestive
Stargardt diseaseOpen Targets
0.06Suggestive
reticular dystrophy of the retinal pigment epitheliumOpen Targets
0.06Suggestive
age related macular degeneration 11Open Targets
0.06Suggestive
age related macular degeneration 2Open Targets
0.06Suggestive
age related macular degeneration 4Open Targets
0.06Suggestive
age related macular degeneration 6Open Targets
0.06Suggestive
age related macular degeneration 7Open Targets
0.06Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
NEDD8Protein interaction100%NAE1Protein interaction100%UBE2MProtein interaction100%SENP3Protein interaction100%CUL1Protein interaction100%CUL2Protein interaction100%
Tissue Expression6 tissues
Heart
100%
Brain
63%
Liver
37%
Ovary
36%
Lung
28%
Bone Marrow
6%
Gene Interaction Network
Click a node to explore
SENP8NEDD8NAE1UBE2MSENP3CUL1CUL2
PROTEIN STRUCTURE
Preparing viewer…
PDB2BKR · 1.90 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
1.05LoF Tolerant
pLIⓘ
0.01Tolerant
Observed/Expected LoF0.60 [0.36–1.05]
RankingsWhere SENP8 stands among ~20K protein-coding genes
  • #9,275of 20,598
    Most Researched47
  • #10,535of 17,882
    Most Constrained (LOEUF)1.05
Genes detectedSENP8
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
Neddylation of PTEN regulates its nuclear import and promotes tumor development.
PMID: 33299139
Cell Res · 2021
1.00
2
SHP2 deneddylation mediates tumor immunosuppression in colon cancer via the CD47/SIRPα axis.
PMID: 36626230
J Clin Invest · 2023
0.90
3
Central role for endothelial human deneddylase-1/SENP8 in fine-tuning the vascular inflammatory response.
PMID: 23209320
J Immunol · 2013
0.80
4
Localization Analysis of Seven De-sumoylation Enzymes (SENPs) in Ocular Cell Lines.
PMID: 30636609
Curr Mol Med · 2018
0.70
5
SENP8 limits aberrant neddylation of NEDD8 pathway components to promote cullin-RING ubiquitin ligase function.
PMID: 28475037
Elife · 2017
0.60