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 …

[HTML][HTML] Protein misfolding in neurodegenerative diseases: implications and strategies

P Sweeney, H Park, M Baumann, J Dunlop… - Translational …, 2017 - Springer
A hallmark of neurodegenerative proteinopathies is the formation of misfolded protein
aggregates that cause cellular toxicity and contribute to cellular proteostatic collapse …

[PDF][PDF] 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 …

The biology of proteostasis in aging and disease

J Labbadia, RI Morimoto - Annual review of biochemistry, 2015 - annualreviews.org
Loss of protein homeostasis (proteostasis) is a common feature of aging and disease that is
characterized by the appearance of nonnative protein aggregates in various tissues. Protein …

[HTML][HTML] Chaperone-mediated autophagy: roles in disease and aging

AM Cuervo, E Wong - Cell research, 2014 - nature.com
This review focuses on chaperone-mediated autophagy (CMA), one of the proteolytic
systems that contributes to degradation of intracellular proteins in lysosomes. CMA substrate …

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 …

[HTML][HTML] 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 …

[HTML][HTML] In situ architecture and cellular interactions of PolyQ inclusions

FJB Bäuerlein, I Saha, A Mishra, M Kalemanov… - Cell, 2017 - cell.com
Expression of many disease-related aggregation-prone proteins results in cytotoxicity and
the formation of large intracellular inclusion bodies. To gain insight into the role of inclusions …

[HTML][HTML] The life cycle of the 26S proteasome: from birth, through regulation and function, and onto its death

I Livneh, V Cohen-Kaplan, C Cohen-Rosenzweig… - Cell research, 2016 - nature.com
The 26S proteasome is a large,∼ 2.5 MDa, multi-catalytic ATP-dependent protease complex
that serves as the degrading arm of the ubiquitin system, which is the major pathway for …

[HTML][HTML] Crosstalk and interplay between the ubiquitin-proteasome system and autophagy

CH Ji, YT Kwon - Molecules and cells, 2017 - Elsevier
Proteolysis in eukaryotic cells is mainly mediated by the ubiquitin (Ub)-proteasome system
(UPS) and the autophagylysosome system (hereafter autophagy). The UPS is a selective …