The proteostasis network and its decline in ageing

MS Hipp, P Kasturi, FU Hartl - Nature reviews Molecular cell biology, 2019 - nature.com
Ageing is a major risk factor for the development of many diseases, prominently including
neurodegenerative disorders such as Alzheimer disease and Parkinson disease. A hallmark …

The logic of the 26S proteasome

GA Collins, AL Goldberg - Cell, 2017 - cell.com
The ubiquitin proteasome pathway is responsible for most of the protein degradation in
mammalian cells. Rates of degradation by this pathway have generally been assumed to be …

[HTML][HTML] In situ structure of neuronal C9orf72 poly-GA aggregates reveals proteasome recruitment

Q Guo, C Lehmer, A Martínez-Sánchez, T Rudack… - Cell, 2018 - cell.com
Protein aggregation and dysfunction of the ubiquitin-proteasome system are hallmarks of
many neurodegenerative diseases. Here, we address the elusive link between these …

A common mechanism of proteasome impairment by neurodegenerative disease-associated oligomers

TA Thibaudeau, RT Anderson, DM Smith - Nature communications, 2018 - nature.com
Protein accumulation and aggregation with a concomitant loss of proteostasis often
contribute to neurodegenerative diseases, and the ubiquitin–proteasome system plays a …

Prions, prionoids and protein misfolding disorders

C Scheckel, A Aguzzi - Nature Reviews Genetics, 2018 - nature.com
Prion diseases are progressive, incurable and fatal neurodegenerative conditions. The term
'prion'was first nominated to express the revolutionary concept that a protein could be …

Degradation of misfolded proteins in neurodegenerative diseases: therapeutic targets and strategies

A Ciechanover, YT Kwon - Experimental & molecular medicine, 2015 - nature.com
Mammalian cells remove misfolded proteins using various proteolytic systems, including the
ubiquitin (Ub)-proteasome system (UPS), chaperone mediated autophagy (CMA) and …

Proteostasis impairment in protein-misfolding and-aggregation diseases

MS Hipp, SH Park, FU Hartl - Trends in cell biology, 2014 - cell.com
Cells possess an extensive network of components to safeguard proteome integrity and
maintain protein homeostasis (proteostasis). When this proteostasis network (PN) declines …

Tau-driven 26S proteasome impairment and cognitive dysfunction can be prevented early in disease by activating cAMP-PKA signaling

N Myeku, CL Clelland, S Emrani, NV Kukushkin… - Nature medicine, 2016 - nature.com
The ubiquitin proteasome system (UPS) degrades misfolded proteins including those
implicated in neurodegenerative diseases. We investigated the effects of tau accumulation …

Proteotoxic stress and the ubiquitin proteasome system

R Kandel, J Jung, S Neal - Seminars in cell & developmental biology, 2024 - Elsevier
The ubiquitin proteasome system maintains protein homeostasis by regulating the
breakdown of misfolded proteins, thereby preventing misfolded protein aggregates. The …

The ubiquitin-proteasome system in neurodegenerative diseases: precipitating factor, yet part of the solution

NP Dantuma, LC Bott - Frontiers in molecular neuroscience, 2014 - frontiersin.org
The ubiquitin-proteasome system (UPS) has been implicated in neurodegenerative
diseases based on the presence of deposits consisting of ubiquitylated proteins in affected …