SENP3 (SUMO Specific Peptidase 3)

A key regulator of SUMOylation involved in cellular stress responses and cancer progression.

Gene Information Card

Symbol SENP3
Full Name SUMO specific peptidase 3
Gene Type protein-coding
Chromosomal Location 17p13.1
NCBI Gene ID 26168 ncbi.nlm.nih.gov/gene/26168
Ensembl ID ENSG00000161956
UniProt ID Q9H4L4
OMIM ID 612844
HGNC ID 17827
Aliases SMT3IP1, SENP3, SUMO1/sentrin specific peptidase 3

Description

SENP3 (SUMO specific peptidase 3) encodes a cysteine protease that specifically deconjugates SUMO2 and SUMO3 from target proteins. It plays a critical role in regulating SUMOylation dynamics, particularly under stress conditions such as hypoxia and oxidative stress. SENP3 is involved in cell cycle progression, ribosome biogenesis, and DNA damage repair. Dysregulation of SENP3 has been implicated in various cancers and neurodegenerative disorders.

Disease Associations

Disease category Pathophysiological mechanism Genomic evidence
Cancer (multiple types) Overexpression of SENP3 promotes cell proliferation and invasion by deSUMOylating key oncoproteins (e.g., HIF-1α, p53). COSMIC, PubMed
Neurodegenerative diseases Altered SENP3 activity affects SUMOylation of proteins involved in neuronal survival and aggregation (e.g., tau, α-synuclein). PubMed
Cardiovascular disease SENP3 modulates cardiac hypertrophy and fibrosis through deSUMOylation of transcription factors. PubMed

Expression Profile

Tissue Expression
Tissue nTPM level
Testis 12.5 Medium
Lymph node 10.2 Medium
Spleen 9.8 Medium
Bone marrow 8.5 Medium
Brain 6.3 Low
Liver 5.1 Low
Cell Line Expression
Cell Line nTPM Notes
HeLa 15.3 Cervical cancer cell line
HEK293 12.1 Embryonic kidney cell line
K562 11.4 Leukemia cell line
MCF7 9.2 Breast cancer cell line
HepG2 7.8 Liver cancer cell line
Data source:Human Protein Atlas(proteinatlas.org)

Mutations & Variants

Hotspot Mutations
Variant Type Frequency Functional Description
c.1015C>T (p.Arg339Trp) Missense <0.01% Unknown; predicted damaging by SIFT
c.1246G>A (p.Gly416Arg) Missense <0.01% Unknown; predicted benign
c.1489_1490insA Frameshift <0.01% Loss of function
Mutation functional classification

Loss of Function (LOF)

Frameshift mutations (e.g., c.1489_1490insA) lead to truncated protein and loss of catalytic activity.

Gain of Function (GOF)

Not well characterized; overexpression in tumors suggests potential gain-of-function effects.

Dominant Negative (DN)

Not reported.

Pathways

SUMOylation pathway
Cellular response to hypoxia
p53 signaling pathway
Ribosome biogenesis

Protein Summary

SENP3 is a 574-amino acid cysteine protease localized primarily in the nucleolus. It contains a conserved C-terminal catalytic domain (peptidase C48 family) and an N-terminal regulatory region. The enzyme specifically removes SUMO2/SUMO3 modifications from target proteins, thereby reversing SUMOylation. Under stress conditions, SENP3 relocalizes to the nucleoplasm and deSUMOylates substrates such as HIF-1α, p53, and PML, influencing transcription, apoptosis, and cell cycle control. Its overexpression in tumors suggests a role in cancer progression.

Related Products

Product name Cat.No. Species Gene ID
SENP3 Knockout HEK293 Cell Line EDJ-KQ1000 Human 26168 Details Get a Quote
SENP3 Knockout A-549 Cell Line EDJ-KQ20043 Human 26168 Details Get a Quote
SENP3 Knockout HCT 116 Cell Line EDJ-KQ20044 Human 26168 Details Get a Quote
SENP3 Knockout HeLa Cell Line EDJ-KQ18725 Human 26168 Details Get a Quote
Displaying Records 1 To 4 Of 4 Records
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