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10 sources retrieved · Most recent: April 2026 · Index updated 15 days ago
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SENP6
SUMO specific peptidase 6
Chromosome 6 · 6q14.1
NCBI Gene: 26054Ensembl: ENSG00000112701.18HGNC: HGNC:20944UniProt: B3KMM0
66PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
FUNCTIONAL ROLE
Highly ConstrainedProtease
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
protein bindingSUMO-specific endopeptidase activityprotein desumoylationprotein modification by small protein removalneurodegenerative diseaseatrial fibrillationosteoarthritis, hiptype 2 diabetes mellitus
✦AI Summary

SENP6 (SUMO specific peptidase 6) is a desumoylating enzyme that removes SUMO modifications from target proteins, particularly showing preference for poly-SUMO2 and poly-SUMO3 chains over SUMO1 1. The enzyme plays critical roles in multiple cellular processes through its desumoylation activity. In mitochondrial biology, SENP6 maintains mitochondrial homeostasis by desumoylating TOM40, a component of the mitochondrial protein import machinery, with impaired SENP6 function leading to disrupted protein import and mitochondrial dysfunction 2. SENP6 regulates oxidative stress responses by desumoylating Nrf2, with increased SENP6 activity promoting Nrf2 degradation and exacerbating neuronal damage following ischemic stroke 3. The enzyme also modulates innate immune responses by desumoylating USP8, which affects interferon signaling through regulation of IFNAR2 stability 4. In cancer biology, SENP6 facilitates telomere cluster dissolution in alternative lengthening of telomeres (ALT)-dependent cancers, making it a potential therapeutic target 5. Additionally, SENP6 expression serves as a biomarker for liver transplantation tolerance, showing strong predictive capacity in clinical studies 6. The enzyme's diverse substrate specificity and cellular localization enable it to coordinate multiple stress response pathways.

Sources cited
1
SENP6 shows preference for poly-SUMO2 and poly-SUMO3 chains over SUMO1
PMID: 18799455
2
SENP6 maintains mitochondrial homeostasis by desumoylating TOM40 and regulating protein import
PMID: 40729740
3
SENP6 desumoylates Nrf2, promoting its degradation and exacerbating oxidative stress in ischemic stroke
PMID: 39716997
4
SENP6 desumoylates USP8 and modulates interferon signaling through IFNAR2 regulation
PMID: 38906982
5
SENP6 facilitates telomere cluster dissolution in ALT-dependent cancers
PMID: 36991019
6
SENP6 expression serves as a biomarker for liver transplantation tolerance
PMID: 30720688
Disease Associationsⓘ20
neurodegenerative diseaseOpen Targets
0.51Moderate
atrial fibrillationOpen Targets
0.43Moderate
osteoarthritis, hipOpen Targets
0.37Weak
type 2 diabetes mellitusOpen Targets
0.32Weak
HydrocephalusOpen Targets
0.32Weak
atrial flutterOpen Targets
0.31Weak
autoimmune disorder of musculoskeletal systemOpen Targets
0.31Weak
cutaneous lupus erythematosusOpen Targets
0.31Weak
migraine disorderOpen Targets
0.26Weak
smoking initiationOpen Targets
0.25Weak
facial painOpen Targets
0.21Weak
osteoarthritisOpen Targets
0.08Suggestive
lymphomaOpen Targets
0.08Suggestive
diffuse large B-cell lymphomaOpen Targets
0.07Suggestive
major depressive disorderOpen Targets
0.07Suggestive
smoking behaviorOpen Targets
0.07Suggestive
Ischemic strokeOpen Targets
0.05Suggestive
Cerebral ischemiaOpen Targets
0.05Suggestive
neoplasmOpen Targets
0.04Suggestive
total hip arthroplastyOpen Targets
0.04Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
SENP5Shared pathway100%UBE2IProtein interaction100%NKAIN2Protein interaction91%SUMO4Protein interaction88%ZDBF2Protein interaction79%SENP1Shared pathway75%
Tissue Expression6 tissues
Bone Marrow
100%
Ovary
87%
Heart
59%
Lung
56%
Brain
55%
Liver
45%
Gene Interaction Network
Click a node to explore
SENP6SENP5UBE2INKAIN2SUMO4ZDBF2SENP1
PROTEIN STRUCTURE
Preparing viewer…
AlphaFoldAI-predicted · UniProt Q9GZR1
View on AlphaFold ↗
Constraintⓘ
LOEUFⓘ
0.32Highly Constrained
pLIⓘ
1.00Intolerant
Observed/Expected LoF0.20 [0.13–0.32]
RankingsWhere SENP6 stands among ~20K protein-coding genes
  • #7,119of 20,598
    Most Researched66
  • #1,310of 17,882
    Most Constrained (LOEUF)0.32 · top 10%
Genes detectedSENP6
Sources retrieved10 papers
Response time—
📄 Sources
10▼
1
SENP6-Mediated deSUMOylation of Nrf2 Exacerbates Neuronal Oxidative Stress Following Cerebral Ischemia and Reperfusion Injury.
PMID: 39716997
Adv Sci (Weinh) · 2025
1.00
2
Astragaloside IV combined with ligustrazine ameliorates abnormal mitochondrial dynamics via Drp1 SUMO/deSUMOylation in cerebral ischemia-reperfusion injury.
PMID: 38615367
CNS Neurosci Ther · 2024
0.90
3
SENP6 Maintains Mitochondrial Homeostasis by Regulating Mitochondrial Protein Import Through deSUMOylation of TOM40.
PMID: 40729740
Adv Sci (Weinh) · 2025
0.80
4
SENP6 restricts the IFN-I-induced signaling pathway and antiviral activity by deSUMOylating USP8.
PMID: 38906982
Cell Mol Immunol · 2024
0.70
5
Histone demethylase KDM2A is a selective vulnerability of cancers relying on alternative telomere maintenance.
PMID: 36991019
Nat Commun · 2023
0.60