Mechanisms and functions of spatial protein quality control

EM Sontag, RS Samant… - Annual review of …, 2017 - annualreviews.org
A healthy proteome is essential for cell survival. Protein misfolding is linked to a rapidly
expanding list of human diseases, ranging from neurodegenerative diseases to aging and …

Alternative systems for misfolded protein clearance: life beyond the proteasome

HE Johnston, RS Samant - The FEBS journal, 2021 - Wiley Online Library
Protein misfolding is a major driver of ageing‐associated frailty and disease pathology.
Although all cells possess multiple, well‐characterised protein quality control systems to …

Differential roles of the ubiquitin proteasome system and autophagy in the clearance of soluble and aggregated TDP-43 species

EL Scotter, C Vance, AL Nishimura… - Journal of cell …, 2014 - journals.biologists.com
ABSTRACT TAR DNA-binding protein (TDP-43, also known as TARDBP) is the major
pathological protein in amyotrophic lateral sclerosis (ALS) and frontotemporal dementia …

[HTML][HTML] Distinct partitioning of ALS associated TDP-43, FUS and SOD1 mutants into cellular inclusions

NE Farrawell, IA Lambert-Smith, ST Warraich, IP Blair… - Scientific reports, 2015 - nature.com
Amyotrophic lateral sclerosis is a rapidly progressing neurodegenerative disease
associated with protein misfolding and aggregation. Most cases are characterized by TDP …

Dynamic JUNQ inclusion bodies are asymmetrically inherited in mammalian cell lines through the asymmetric partitioning of vimentin

M Ogrodnik, H Salmonowicz, R Brown… - Proceedings of the …, 2014 - National Acad Sciences
Aging is associated with the accumulation of several types of damage: in particular, damage
to the proteome. Recent work points to a conserved replicative rejuvenation mechanism that …

Protein quality control in health and disease

T Dubnikov, T Ben-Gedalya… - Cold Spring Harbor …, 2017 - cshperspectives.cshlp.org
Maintaining functional protein homeostasis (proteostasis) is a constant challenge in the face
of limited protein-folding capacity, environmental threats, and aging. Cells have developed …

[HTML][HTML] Aging and the aggregating proteome

DC David - Frontiers in genetics, 2012 - frontiersin.org
For all organisms promoting protein homeostasis is a high priority in order to optimize
cellular functions and resources. However, there is accumulating evidence that aging leads …

[HTML][HTML] Caloric restriction extends yeast chronological lifespan by altering a pattern of age-related changes in trehalose concentration

P Kyryakov, A Beach, VR Richard, MT Burstein… - Frontiers in …, 2012 - frontiersin.org
The non-reducing disaccharide trehalose has been long considered only as a reserve
carbohydrate. However, recent studies in yeast suggested that this osmolyte can protect …

[HTML][HTML] Prion degradation pathways: Potential for therapeutic intervention

R Goold, C McKinnon, SJ Tabrizi - Molecular and Cellular Neuroscience, 2015 - Elsevier
Prion diseases are fatal neurodegenerative disorders. Pathology is closely linked to the
misfolding of native cellular PrP C into the disease-associated form PrP Sc that accumulates …

[HTML][HTML] Lanosterol modulates proteostasis via dissolving cytosolic sequestosomes/aggresome-like induced structures

LD Hu, J Wang, XJ Chen, YB Yan - Biochimica et Biophysica Acta (BBA) …, 2020 - Elsevier
Sequestration of misfolded proteins into distinct cellular compartments plays a pivotal role in
proteostasis and proteopathies. Cytoplasmic ubiquitinated proteins are sequestered by …