Molecular mechanisms of TDP-43 misfolding and pathology in amyotrophic lateral sclerosis

A Prasad, V Bharathi, V Sivalingam… - Frontiers in molecular …, 2019 - frontiersin.org
TAR DNA binding protein 43 (TDP-43) is a versatile RNA/DNA binding protein involved in
RNA-related metabolism. Hyper-phosphorylated and ubiquitinated TDP-43 deposits act as …

The role of TDP-43 propagation in neurodegenerative diseases: integrating insights from clinical and experimental studies

M Jo, S Lee, YM Jeon, S Kim, Y Kwon… - Experimental & molecular …, 2020 - nature.com
Abstract TAR DNA-binding protein 43 (TDP-43) is a highly conserved nuclear RNA/DNA-
binding protein involved in the regulation of RNA processing. The accumulation of TDP-43 …

Mechanisms and consequences of macromolecular phase separation

LP Bergeron-Sandoval, N Safaee, SW Michnick - Cell, 2016 - cell.com
Over a century ago, colloidal phase separation of matter into non-membranous bodies was
recognized as a fundamental organizing principal of cell" protoplasm." Recent insights into …

The tip of the iceberg: RNA-binding proteins with prion-like domains in neurodegenerative disease

OD King, AD Gitler, J Shorter - Brain research, 2012 - Elsevier
Prions are self-templating protein conformers that are naturally transmitted between
individuals and promote phenotypic change. In yeast, prion-encoded phenotypes can be …

Prions in yeast

SW Liebman, YO Chernoff - Genetics, 2012 - academic.oup.com
The concept of a prion as an infectious self-propagating protein isoform was initially
proposed to explain certain mammalian diseases. It is now clear that yeast also has …

Disome-seq reveals widespread ribosome collisions that promote cotranslational protein folding

T Zhao, YM Chen, Y Li, J Wang, S Chen, N Gao… - Genome biology, 2021 - Springer
Background The folding of proteins is challenging in the highly crowded and sticky
environment of a cell. Regulation of translation elongation may play a crucial role in …

Protein-based inheritance: epigenetics beyond the chromosome

ZH Harvey, Y Chen, DF Jarosz - Molecular cell, 2018 - cell.com
Epigenetics refers to changes in phenotype that are not rooted in DNA sequence. This
phenomenon has largely been studied in the context of chromatin modification. Yet many …

Prion-like disorders: blurring the divide between transmissibility and infectivity

M Cushman, BS Johnson, OD King… - Journal of cell …, 2010 - journals.biologists.com
Prions are proteins that access self-templating amyloid forms, which confer phenotypic
changes that can spread from individual to individual within or between species. These …

A yeast prion, Mod5, promotes acquired drug resistance and cell survival under environmental stress

G Suzuki, N Shimazu, M Tanaka - Science, 2012 - science.org
Prion conversion from a soluble protein to an aggregated state may be involved in the
cellular adaptation of yeast to the environment. However, it remains unclear whether and …

Essential role of coiled coils for aggregation and activity of Q/N-rich prions and PolyQ proteins

F Fiumara, L Fioriti, ER Kandel, WA Hendrickson - Cell, 2010 - cell.com
The functional switch of glutamine/asparagine (Q/N)-rich prions and the neurotoxicity of
polyQ-expanded proteins involve complex aggregation-prone structural transitions …