| USP19 | |
|---|---|
| Gene | [USP19](/genes/usp19) |
| UniProt | Q9Y5E4 |
| PDB | 5W7V |
| Mol. Weight | ~150 kDa (1459 amino acids) |
| Localization | Endoplasmic reticulum, cytoplasm |
| Family | USP (Ubiquitin-specific peptidase) family |
| Chromosome | 3p21.2 |
| Diseases | [AD](/diseases/ad), [PD](/diseases/pd), [Inclusion Body Myositis](/diseases/ibm) |
USP19 is an ER-resident deubiquitinating enzyme involved in ER-associated degradation (ERAD), protein quality control, and cellular stress responses. USP19 plays important roles in neurodegenerative diseases through its functions in protein clearance and survival pathways.
USP19 (Ubiquitin-specific peptidase 19) is a ~150 kDa protein encoded by the USP19 gene on chromosome 3p21.2 [1]. It belongs to the USP family and contains a unique transmembrane domain that anchors it to the endoplasmic reticulum [2].
USP19 contains several distinctive features:
The protein has a unique architecture among USPs, with the catalytic domain facing the cytosol [3].
In the healthy nervous system, USP19 participates in:
USP19 contributes to AD pathogenesis:
In PD, USP19 involvement includes:
USP19 is notably implicated in sporadic inclusion body myositis:
Hassink et al. USP19: an ER-resident DUB (2009). 2009. ↩︎
Lee et al. USP19 structure and function (2016). 2016. ↩︎
Wang et al. USP19 in ERAD and disease (2020). 2020. ↩︎
Chen et al. USP19 in neurodegenerative diseases (2021). 2021. ↩︎