| SENP3 | |
|---|---|
| Gene | [SENP3](/genes/senp3) |
| UniProt | Q9H4L4 |
| PDB | 4F5W |
| Mol. Weight | 73 kDa (574 amino acids) |
| Localization | Nucleus, nucleolus |
| Family | SUMO protease (ULP) family |
| Chromosome | 17p13.1 |
| Diseases | [AD](/diseases/ad), [PD](/diseases/pd), [ALS](/diseases/als), [Cancer](/diseases/cancer) |
SENP3 is a SUMO-specific protease that removes SUMO (Small Ubiquitin-like Modifier) conjugates from target proteins. It plays crucial roles in regulating transcription, DNA repair, stress responses, and mitochondrial dynamics. SENP3 dysfunction contributes to neurodegeneration through altered sumoylation of key disease-related proteins.
SENP3 (Sentrin-specific protease 3) is a 73 kDa protein encoded by the SENP3 gene on chromosome 17p13.1 [1]. It belongs to the ULP (Ubiquitin-like protease) family of SUMO proteases [2].
SENP3 contains key structural features:
The enzyme is predominantly localized to the nucleolus, where it regulates ribosomal biogenesis and stress responses [3].
In the healthy nervous system, SENP3 performs essential functions:
SENP3 contributes to AD through:
In PD, SENP3 involvement includes:
SENP3 dysfunction in ALS:
Yeh et al. The SUMO protease SENP3 in health and disease (2020). 2020. ↩︎
Kunz et al. SUMO-specific proteases in neurodegeneration (2019). 2019. ↩︎
Rohira et al. SENP3 in nucleolar stress response (2021). 2021. ↩︎
Zhang et al. SENP3 in Alzheimer's disease (2022). 2022. ↩︎