Surviving protein quality control catastrophes–from cells to organisms

K Schneider, A Bertolotti - Journal of Cell Science, 2015 - journals.biologists.com
Organisms have evolved mechanisms to cope with and adapt to unexpected challenges and
harsh conditions. Unfolded or misfolded proteins represent a threat for cells and organisms …

Hereditary spastic paraplegia and future therapeutic directions: beneficial effects of small compounds acting on cellular stress

S Gumeni, C Vantaggiato, M Montopoli… - Frontiers in …, 2021 - frontiersin.org
Hereditary spastic paraplegia (HSP) is a group of inherited neurodegenerative conditions
that share a characteristic feature of degeneration of the longest axons within the …

Pharmacological reduction of ER stress protects against TDP-43 neuronal toxicity in vivo

A Vaccaro, SA Patten, D Aggad, C Julien, C Maios… - Neurobiology of …, 2013 - Elsevier
C. elegans and D. rerio expressing mutant TAR DNA Binding Protein 43 (TDP-43) are
powerful in vivo animal models for the genetics and pharmacology of amyotrophic lateral …

Novel mechanisms of eIF2B action and regulation by eIF2α phosphorylation

AM Bogorad, KY Lin, A Marintchev - Nucleic acids research, 2017 - academic.oup.com
Eukaryotic translation initiation factor 2 (eIF2) is a heterotrimeric GTPase, which plays a
critical role in protein synthesis regulation. eIF2-GTP binds Met-tRNAi to form the eIF2-GTP• …

Distinct modulatory role of RNA in the aggregation of the tumor suppressor protein p53 core domain

PS Kovachev, D Banerjee, LP Rangel… - Journal of Biological …, 2017 - ASBMB
Inactivation of the tumor suppressor protein p53 by mutagenesis, chemical modification,
protein-protein interaction, or aggregation has been associated with different human …

A yeast-based assay identifies drugs active against human mitochondrial disorders

E Couplan, RS Aiyar, R Kucharczyk… - Proceedings of the …, 2011 - National Acad Sciences
Due to the lack of relevant animal models, development of effective treatments for human
mitochondrial diseases has been limited. Here we establish a rapid, yeast-based assay to …

Amyloids and yeast prion biology

RB Wickner, HK Edskes, DA Bateman, AC Kelly… - Biochemistry, 2013 - ACS Publications
The prions (infectious proteins) of Saccharomyces cerevisiae are proteins acting as genes,
by templating their conformation from one molecule to another in analogy to DNA templating …

RNA modulates aggregation of the recombinant mammalian prion protein by direct interaction

PS Kovachev, MPB Gomes, Y Cordeiro, NC Ferreira… - Scientific reports, 2019 - nature.com
Recent studies have proposed that nucleic acids act as potential cofactors for protein
aggregation and prionogenesis. By means of sedimentation, transmission electron …

Protein inheritance (prions) based on parallel in‐register β‐sheet amyloid structures

RB Wickner, F Shewmaker, D Kryndushkin… - …, 2008 - Wiley Online Library
Most prions (infectious proteins) are self‐propagating amyloids (filamentous protein
multimers), and have been found in both mammals and fungal species. The prions [URE3] …

Nonspecific prion protein–nucleic acid interactions lead to different aggregates and cytotoxic species

B Macedo, TA Millen, CACA Braga, MPB Gomes… - Biochemistry, 2012 - ACS Publications
A misfolded form of the prion protein (PrP) is the primary culprit in mammalian prion
diseases. It has been shown that nucleic acids catalyze the misfolding of cellular PrP into a …