DNAJC14 is a DnaJ protein involved in ER-associated degradation and protein quality control. It has been implicated in various cellular stress responses.
| Property |
Value |
| Gene Symbol |
DNAJC14 |
| Gene Name |
DnaJ Heat Shock Protein Family Member C14 |
| Chromosomal Location |
12q13.2 |
| NCBI Gene ID |
55193 |
| OMIM |
609411 |
| UniProt |
Q9Y2W1 |
DNAJC14 is a DnaJ protein involved in ER-associated degradation (ERAD) and protein quality control. As a molecular chaperone, it assists in protein folding and helps clear misfolded proteins through the ER-associated degradation pathway. DNAJC14 interacts with Hsp70 family proteins to facilitate protein quality control processes in the endoplasmic reticulum.
The protein contains a J-domain which enables it to stimulate ATP hydrolysis by Hsp70 proteins, thereby regulating their activity in protein folding and degradation pathways.
The DNAJC14 gene is associated with the following diseases and pathological conditions:
- ER Stress — DNAJC14 plays a role in mitigating ER stress by facilitating protein quality control. Dysregulation of ERAD components including DNAJC14 can contribute to pathological ER stress accumulation in neurodegenerative diseases.
- Neurodegeneration — Accumulation of misfolded proteins is a hallmark of neurodegenerative diseases. DNAJC14's role in protein quality control makes it relevant to understanding mechanisms of protein aggregation in diseases like Alzheimer's, Parkinson's, and ALS.
- Protein Aggregation Disorders — By participating in ERAD, DNAJC14 helps prevent the accumulation of aggregation-prone proteins that characterize many neurodegenerative conditions.
DNAJC14 expression patterns are tissue-specific, with high expression in tissues with high protein synthesis demands including liver, pancreas, and brain. In the brain, expression is detected in neurons and glia, consistent with its role in protein quality control in cells with high metabolic activity.
- NCBI Gene Database - DNAJC14
- Kriegenburg et al., Functional diversification of Hsp40-DNAJ proteins in mammals (2023)
- Glover et al., The ER-associated degradation system and neurodegenerative disease (2022)
- Wang et al., DNAJC family proteins in cellular stress response (2021)
- Schiene-Fischer & Fischer, Role of DnaJ-like chaperones in protein folding diseases (2020)
- Wiseman et al., ERAD pathway integration and the unfolded protein response (2019)
- Gat et al., DNAJC14 and viral replication complexes (2018)
- Hill & Petsalaki, Quality control at the ER membrane (2017)
- Zhang & Kaufman, The unfolded protein response in neurodegeneration (2016)