[HTML][HTML] Phase separation and neurodegenerative diseases: a disturbance in the force
A Zbinden, M Pérez-Berlanga, P De Rossi… - Developmental cell, 2020 - cell.com
Protein aggregation is the main hallmark of neurodegenerative diseases. Many proteins
found in pathological inclusions are known to undergo liquid-liquid phase separation, a …
found in pathological inclusions are known to undergo liquid-liquid phase separation, a …
Liquid–liquid phase separation and its mechanistic role in pathological protein aggregation
WM Babinchak, WK Surewicz - Journal of molecular biology, 2020 - Elsevier
Liquid–liquid phase separation (LLPS) of proteins underlies the formation of membrane-less
organelles. While it has been recognized for some time that these organelles are of key …
organelles. While it has been recognized for some time that these organelles are of key …
The role of liquid–liquid phase separation in aggregation of the TDP-43 low-complexity domain
WM Babinchak, R Haider, BK Dumm, P Sarkar… - Journal of Biological …, 2019 - ASBMB
Pathological aggregation of the transactive response DNA-binding protein of 43 kDa (TDP-
43) is associated with several neurodegenerative disorders, including ALS, frontotemporal …
43) is associated with several neurodegenerative disorders, including ALS, frontotemporal …
The amyotrophic lateral sclerosis exposome: recent advances and future directions
Amyotrophic lateral sclerosis (ALS) is a fatal disease of motor neuron degeneration with
typical survival of only 2–5 years from diagnosis. The causes of ALS are multifactorial …
typical survival of only 2–5 years from diagnosis. The causes of ALS are multifactorial …
Mechanisms and regulation of RNA condensation in RNP granule formation
Ribonucleoprotein (RNP) granules are RNA–protein assemblies that are involved in
multiple aspects of RNA metabolism and are linked to memory, development, and disease …
multiple aspects of RNA metabolism and are linked to memory, development, and disease …
TDP-43 condensates and lipid droplets regulate the reactivity of microglia and regeneration after traumatic brain injury
A Zambusi, KT Novoselc, S Hutten, S Kalpazidou… - Nature …, 2022 - nature.com
Decreasing the activation of pathology-activated microglia is crucial to prevent chronic
inflammation and tissue scarring. In this study, we used a stab wound injury model in …
inflammation and tissue scarring. In this study, we used a stab wound injury model in …
[HTML][HTML] NUP62 localizes to ALS/FTLD pathological assemblies and contributes to TDP-43 insolubility
Abstract A G4C2 hexanucleotide repeat expansion in the C9orf72 gene is the most common
genetic cause of ALS and FTLD (C9-ALS/FTLD) with cytoplasmic TDP-43 inclusions …
genetic cause of ALS and FTLD (C9-ALS/FTLD) with cytoplasmic TDP-43 inclusions …
Modeling Neurodegenerative Disorders in Drosophila melanogaster
H Bolus, K Crocker, G Boekhoff-Falk… - International Journal of …, 2020 - mdpi.com
Drosophila melanogaster provides a powerful genetic model system in which to investigate
the molecular mechanisms underlying neurodegenerative diseases. In this review, we …
the molecular mechanisms underlying neurodegenerative diseases. In this review, we …
FUS pathology in ALS is linked to alterations in multiple ALS-associated proteins and rescued by drugs stimulating autophagy
L Marrone, HCA Drexler, J Wang, P Tripathi… - Acta …, 2019 - Springer
Amyotrophic lateral sclerosis (ALS) is a lethal disease characterized by motor neuron
degeneration and associated with aggregation of nuclear RNA-binding proteins (RBPs) …
degeneration and associated with aggregation of nuclear RNA-binding proteins (RBPs) …
CLIP-Seq analysis enables the design of protective ribosomal RNA bait oligonucleotides against C9ORF72 ALS/FTD poly-GR pathophysiology
Amyotrophic lateral sclerosis and frontotemporal dementia patients with a hexanucleotide
repeat expansion in C9ORF72 (C9-HRE) accumulate poly-GR and poly-PR aggregates. The …
repeat expansion in C9ORF72 (C9-HRE) accumulate poly-GR and poly-PR aggregates. The …