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25 sources retrieved · Most recent: April 2026 · Index updated 15 days ago
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SENP1
SUMO specific peptidase 1
Chromosome 12 · 12q13.11
NCBI Gene: 29843Ensembl: ENSG00000079387.16HGNC: HGNC:17927UniProt: Q9P0U3
199PubMed Papers
20Diseases
0Drugs
0Pathogenic Variants
FUNCTIONAL ROLE
Protease
RESEARCH IMPACT
Trending
DATA QUALITY
✓ Experimental GO Evidence✓ Swiss-Prot Reviewed
positive regulation of transcription by RNA polymerase IIprotein bindingSUMO-specific endopeptidase activityprotein desumoylationneurodegenerative diseasediabetes mellitustype 2 diabetes mellitusAbruptio Placentae
✦AI Summary

SENP1 is a crucial SUMO-specific protease that catalyzes two essential functions in the SUMO pathway: processing SUMO precursors into mature forms and deconjugating SUMO proteins from target substrates 1. The enzyme plays pivotal roles in multiple cellular processes through its desumoylation activity. In metabolic regulation, SENP1 controls mitochondrial function by desumoylating Sirt3, enhancing its deacetylase activity and promoting fatty acid oxidation during fasting stress 2. SENP1 also regulates cell cycle progression, as lactate directly inhibits SENP1 by binding its zinc-containing active site, leading to APC4 SUMOylation and efficient mitotic exit 3. In cardiovascular disease, SENP1 prevents myocardial fibrosis through HSP90ab1 desumoylation and protects against hypoxic pulmonary hypertension via FIS1 desumoylation, which maintains mitochondrial integrity 45. SENP1 demonstrates therapeutic potential in acute kidney injury by coupling with AMPK activation to reduce mitochondrial oxidative stress 6. However, SENP1 can promote tumorigenesis, as seen in acute myeloid leukemia where it activates AKT signaling through HDAC2 desumoylation 7. These findings establish SENP1 as a critical regulator of cellular homeostasis with both protective and pathological roles depending on the cellular context.

Sources cited
1
SENP1 catalyzes SUMO precursor processing and deconjugation functions
PMID: 10652325
2
SENP1 desumoylates Sirt3 to enhance mitochondrial metabolism during fasting
PMID: 31302001
3
Lactate directly inhibits SENP1 to regulate cell cycle through APC4 SUMOylation
PMID: 36921622
4
SENP1 prevents myocardial fibrosis through HSP90ab1 desumoylation
PMID: 38992961
5
SENP1 protects against hypoxic pulmonary hypertension via FIS1 desumoylation
PMID: 37589160
6
SENP1-Sirt3 axis protects against acute kidney injury by reducing mitochondrial oxidative stress
PMID: 37608549
7
SENP1 promotes acute myeloid leukemia progression through HDAC2 desumoylation and AKT activation
PMID: 38822351
Disease Associationsⓘ20
neurodegenerative diseaseOpen Targets
0.52Moderate
diabetes mellitusOpen Targets
0.28Weak
type 2 diabetes mellitusOpen Targets
0.27Weak
Abruptio PlacentaeOpen Targets
0.15Weak
neoplasmOpen Targets
0.11Weak
hepatocellular carcinomaOpen Targets
0.11Weak
cancerOpen Targets
0.10Weak
smoking initiationOpen Targets
0.10Suggestive
posterior cortical atrophyOpen Targets
0.10Suggestive
lung cancerOpen Targets
0.09Suggestive
nonpapillary renal cell carcinomaOpen Targets
0.09Suggestive
osteosarcomaOpen Targets
0.09Suggestive
triple-negative breast cancerOpen Targets
0.09Suggestive
acute myeloid leukemiaOpen Targets
0.08Suggestive
ovarian cancerOpen Targets
0.08Suggestive
cardiac hypertrophyOpen Targets
0.08Suggestive
renal cell carcinomaOpen Targets
0.08Suggestive
melanomaOpen Targets
0.08Suggestive
type 1 diabetes mellitusOpen Targets
0.08Suggestive
Mantle cell lymphomaOpen Targets
0.07Suggestive
Pathogenic Variants
No pathogenic variants reported on ClinVar for this gene.
View on ClinVar ↗
Related Genes
NEDD8Protein interaction88%SUMO4Protein interaction88%RANGAP1Protein interaction87%SUMO3Protein interaction87%SUMO2Protein interaction87%UBE2IProtein interaction87%
Tissue Expression6 tissues
Bone Marrow
100%
Brain
57%
Ovary
45%
Lung
40%
Heart
35%
Liver
29%
Gene Interaction Network
Click a node to explore
SENP1NEDD8SUMO4RANGAP1SUMO3SUMO2UBE2I
PROTEIN STRUCTURE
Preparing viewer…
PDB2XPH · 2.40 Å · X-ray
View on RCSB ↗
Constraintⓘ
LOEUFⓘ
0.43Moderately Constrained
pLIⓘ
1.00Intolerant
Observed/Expected LoF0.27 [0.18–0.43]
RankingsWhere SENP1 stands among ~20K protein-coding genes
  • #2,131of 20,598
    Most Researched199 · top quartile
  • #2,271of 17,882
    Most Constrained (LOEUF)0.43 · top quartile
Genes detectedSENP1
Sources retrieved25 papers
Response time—
📄 Sources
25▼
1
Lactate regulates cell cycle by remodelling the anaphase promoting complex.
PMID: 36921622
Nature · 2023
1.00
2
SENP1 Decreases RNF168 Phase Separation to Promote DNA Damage Repair and Drug Resistance in Colon Cancer.
PMID: 37350666
Cancer Res · 2023
0.90
3
SENP1-Sirt3 Signaling Controls Mitochondrial Protein Acetylation and Metabolism.
PMID: 31302001
Mol Cell · 2019
0.80
4
Endothelial FIS1 DeSUMOylation Protects Against Hypoxic Pulmonary Hypertension.
PMID: 37589160
Circ Res · 2023
0.70
5
MANF facilitates breast cancer cell survival under glucose-starvation conditions via PRKN-mediated mitophagy regulation.
PMID: 39147386
Autophagy · 2025
0.60