Promoting the clearance of neurotoxic proteins in neurodegenerative disorders of ageing

B Boland, WH Yu, O Corti, B Mollereau… - Nature reviews Drug …, 2018 - nature.com
Neurodegenerative disorders of ageing (NDAs) such as Alzheimer disease, Parkinson
disease, frontotemporal dementia, Huntington disease and amyotrophic lateral sclerosis …

Regulation of heat shock transcription factors and their roles in physiology and disease

R Gomez-Pastor, ET Burchfiel, DJ Thiele - Nature reviews Molecular …, 2018 - nature.com
The heat shock transcription factors (HSFs) were discovered over 30 years ago as direct
transcriptional activators of genes regulated by thermal stress, encoding heat shock …

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 …

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 …

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 …

Polyglutamine repeats in neurodegenerative diseases

AP Lieberman, VG Shakkottai… - Annual Review of …, 2019 - annualreviews.org
Among the age-dependent protein aggregation disorders, nine neurodegenerative diseases
are caused by expansions of CAG repeats encoding polyglutamine (polyQ) tracts. We …

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 …

Autophagy modulation as a potential therapeutic target for diverse diseases

DC Rubinsztein, P Codogno, B Levine - Nature reviews Drug discovery, 2012 - nature.com
Autophagy is an essential, conserved lysosomal degradation pathway that controls the
quality of the cytoplasm by eliminating protein aggregates and damaged organelles. It …

Huntington disease

GP Bates, R Dorsey, JF Gusella, MR Hayden… - Nature reviews Disease …, 2015 - nature.com
Huntington disease is devastating to patients and their families—with autosomal dominant
inheritance, onset typically in the prime of adult life, progressive course, and a combination …

Functional modules of the proteostasis network

GG Jayaraj, MS Hipp, FU Hartl - Cold Spring Harbor …, 2020 - cshperspectives.cshlp.org
Cells invest in an extensive network of factors to maintain protein homeostasis (proteostasis)
and prevent the accumulation of potentially toxic protein aggregates. This proteostasis …