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 …

[HTML][HTML] The molecular language of membraneless organelles

E Gomes, J Shorter - Journal of Biological Chemistry, 2019 - Elsevier
Eukaryotic cells organize their intracellular components into organelles that can be
membrane-bound or membraneless. A large number of membraneless organelles, including …

[HTML][HTML] A designer AAV variant permits efficient retrograde access to projection neurons

DGR Tervo, BY Hwang, S Viswanathan, T Gaj… - Neuron, 2016 - cell.com
Efficient retrograde access to projection neurons for the delivery of sensors and effectors
constitutes an important and enabling capability for neural circuit dissection. Such an …

Nuclear-import receptors reverse aberrant phase transitions of RNA-binding proteins with prion-like domains

L Guo, HJ Kim, H Wang, J Monaghan, F Freyermuth… - Cell, 2018 - cell.com
RNA-binding proteins (RBPs) with prion-like domains (PrLDs) phase transition to functional
liquids, which can mature into aberrant hydrogels composed of pathological fibrils that …

Protein phase separation: a new phase in cell biology

S Boeynaems, S Alberti, NL Fawzi, T Mittag… - Trends in cell …, 2018 - cell.com
Cellular compartments and organelles organize biological matter. Most well-known
organelles are separated by a membrane boundary from their surrounding milieu. There are …

FUS and TDP-43 phases in health and disease

B Portz, BL Lee, J Shorter - Trends in biochemical sciences, 2021 - cell.com
The distinct prion-like domains (PrLDs) of FUS and TDP-43, modulate phase transitions that
result in condensates with a range of material states. These assemblies are implicated in …

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 …

Astrocytes: a central element in neurological diseases

M Pekny, M Pekna, A Messing, C Steinhäuser… - Acta …, 2016 - Springer
The neurone-centred view of the past disregarded or downplayed the role of astroglia as a
primary component in the pathogenesis of neurological diseases. As this concept is …

Liquid-liquid phase separation of TDP-43 and FUS in physiology and pathology of neurodegenerative diseases

JL Carey, L Guo - Frontiers in molecular biosciences, 2022 - frontiersin.org
Liquid-liquid phase separation of RNA-binding proteins mediates the formation of numerous
membraneless organelles with essential cellular function. However, aberrant phase …

RNA-binding proteins with prion-like domains in health and disease

AF Harrison, J Shorter - Biochemical Journal, 2017 - portlandpress.com
Approximately 70 human RNA-binding proteins (RBPs) contain a prion-like domain (PrLD).
PrLDs are low-complexity domains that possess a similar amino acid composition to prion …