[PDF][PDF] 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 …

[PDF][PDF] ALS genetics: gains, losses, and implications for future therapies

G Kim, O Gautier, E Tassoni-Tsuchida, XR Ma… - Neuron, 2020 - cell.com
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disorder caused by the loss
of motor neurons from the brain and spinal cord. The ALS community has made remarkable …

[PDF][PDF] Phase separation of FUS is suppressed by its nuclear import receptor and arginine methylation

M Hofweber, S Hutten, B Bourgeois, E Spreitzer… - Cell, 2018 - cell.com
Cytoplasmic FUS aggregates are a pathological hallmark in a subset of patients with
frontotemporal dementia (FTD) or amyotrophic lateral sclerosis (ALS). A key step that is …

Phosphorylation of the FUS low‐complexity domain disrupts phase separation, aggregation, and toxicity

Z Monahan, VH Ryan, AM Janke, KA Burke… - The EMBO …, 2017 - embopress.org
Neuronal inclusions of aggregated RNA‐binding protein fused in sarcoma (FUS) are
hallmarks of ALS and frontotemporal dementia subtypes. Intriguingly, FUS's nearly …

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 …

Frontotemporal dementia

NT Olney, S Spina, BL Miller - Neurologic clinics, 2017 - neurologic.theclinics.com
Frontotemporal dementia (FTD) has undergone numerous changes in nomenclature and
categorization schemes since it was first described by Pick in 1892. Presently, FTD …

Imaging tau and amyloid-β proteinopathies in Alzheimer disease and other conditions

VL Villemagne, V Doré, SC Burnham… - Nature Reviews …, 2018 - nature.com
Most neurodegenerative disorders are associated with aggregated protein deposits. In the
case of Alzheimer disease (AD), extracellular amyloid-β (Aβ) aggregates and intracellular …

Frontotemporal lobar degeneration

M Grossman, WW Seeley, AL Boxer, AE Hillis… - Nature Reviews …, 2023 - nature.com
Frontotemporal lobar degeneration (FTLD) is one of the most common causes of early-onset
dementia and presents with early social–emotional–behavioural and/or language changes …

Tau and tauopathies

T Arendt, JT Stieler, M Holzer - Brain research bulletin, 2016 - Elsevier
Most neurodegenerative diseases are characterized by intracellular aggregates of insoluble
proteins. As for the majority of these disorders, aetiology and pathogenesis are only poorly …

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 …