Protein aggregation
- Neurodegenerative disease-associated protein aggregates are poor inducers of the heat shock response in neuronal cells
Summary: Aggregation-prone proteins associated with neurodegenerative diseases impair or evade the heat shock response in neuronal cells.
- SOD1A4V aggregation alters ubiquitin homeostasis in a cell model of ALS
Summary: Misfolded SOD1 contributes to ubiquitin proteasome system dysfunction in a cell model of ALS. Hence, ubiquitin homeostasis is an important marker of changes in ALS pathology.
- PIGN prevents protein aggregation in the endoplasmic reticulum independently of its function in the GPI synthesis
Summary: By using visualized secreted proteins markers, we identify that PIGN/PIGN-1 has an unexpected role in protein quality control preventing protein aggregation within the ER.
- PrP-containing aggresomes are cytosolic components of an ER quality control mechanism
Summary: Prion protein (PrP)-containing cytosolic aggresomes are components of a quality-control mechanism of the ER. PrP molecules that fail to enter the ER will not be deposited in pre-existing aggresomes.
- Aggregation dynamics and identification of aggregation-prone mutants of the von Hippel–Lindau tumor suppressor protein
Highlighted Article: If misfolded, the von Hippel–Lindau (pVHL) tumor suppressor protein is prone to aggregate and to be degraded. Tumorigenic mutations might compromise its folding. We identify new factors regulating pVHL folding.
- Vulnerability of newly synthesized proteins to proteostasis stress
Summary: This study shows that stressors that burden the proteostatic network produce the greatest impact on newly made proteins with a substantial portion of the proteome being vulnerable to such stress.
- Distinguishing aggregate formation and aggregate clearance using cell-based assays
Highlighted Article: Disequilibrium between aggregate formation and clearance leads to accumulation of aggregated proteins. We have created a new cell-based assay that can determine how modifiers can impact on aggregate burden.