[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 …
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 …
PrLDs are low-complexity domains that possess a similar amino acid composition to prion …
Phosphorylation of the FUS low‐complexity domain disrupts phase separation, aggregation, and toxicity
Neuronal inclusions of aggregated RNA‐binding protein fused in sarcoma (FUS) are
hallmarks of ALS and frontotemporal dementia subtypes. Intriguingly, FUS's nearly …
hallmarks of ALS and frontotemporal dementia subtypes. Intriguingly, FUS's nearly …
The tip of the iceberg: RNA-binding proteins with prion-like domains in neurodegenerative disease
Prions are self-templating protein conformers that are naturally transmitted between
individuals and promote phenotypic change. In yeast, prion-encoded phenotypes can be …
individuals and promote phenotypic change. In yeast, prion-encoded phenotypes can be …
TDP-43 and FUS in amyotrophic lateral sclerosis and frontotemporal dementia
IRA Mackenzie, R Rademakers… - The Lancet Neurology, 2010 - thelancet.com
Abnormal intracellular protein aggregates comprise a key characteristic in most
neurodegenerative diseases, including amyotrophic lateral sclerosis (ALS) and …
neurodegenerative diseases, including amyotrophic lateral sclerosis (ALS) and …
TDP-43 and FUS/TLS: emerging roles in RNA processing and neurodegeneration
C Lagier-Tourenne, M Polymenidou… - Human molecular …, 2010 - academic.oup.com
Amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD) are
neurodegenerative diseases with clinical and pathological overlap. Landmark discoveries of …
neurodegenerative diseases with clinical and pathological overlap. Landmark discoveries of …
Residue-by-residue view of in vitro FUS granules that bind the C-terminal domain of RNA polymerase II
Phase-separated states of proteins underlie ribonucleoprotein (RNP) granules and nuclear
RNA-binding protein assemblies that may nucleate protein inclusions associated with …
RNA-binding protein assemblies that may nucleate protein inclusions associated with …
ALS‐associated fused in sarcoma (FUS) mutations disrupt Transportin‐mediated nuclear import
D Dormann, R Rodde, D Edbauer, E Bentmann… - The EMBO …, 2010 - embopress.org
Mutations in fused in sarcoma (FUS) are a cause of familial amyotrophic lateral sclerosis
(fALS). Patients carrying point mutations in the C‐terminus of FUS show neuronal …
(fALS). Patients carrying point mutations in the C‐terminus of FUS show neuronal …
Motor neuron susceptibility in ALS/FTD
AMG Ragagnin, S Shadfar, M Vidal… - Frontiers in …, 2019 - frontiersin.org
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by the
death of both upper and lower motor neurons (MNs) in the brain, brainstem and spinal cord …
death of both upper and lower motor neurons (MNs) in the brain, brainstem and spinal cord …
The role of FUS gene variants in neurodegenerative diseases
H Deng, K Gao, J Jankovic - Nature Reviews Neurology, 2014 - nature.com
The neurodegenerative diseases are a diverse group of disorders characterized by
progressive loss of specific groups of neurons. These diseases affect different populations …
progressive loss of specific groups of neurons. These diseases affect different populations …