[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 …
[HTML][HTML] RNA binding antagonizes neurotoxic phase transitions of TDP-43
TDP-43 proteinopathy is a pathological hallmark of amyotrophic lateral sclerosis and
frontotemporal dementia where cytoplasmic TDP-43 inclusions are observed within …
frontotemporal dementia where cytoplasmic TDP-43 inclusions are observed within …
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 …
Nuclear-import receptors reverse aberrant phase transitions of RNA-binding proteins with prion-like domains
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 …
liquids, which can mature into aberrant hydrogels composed of pathological fibrils that …
RNA buffers the phase separation behavior of prion-like RNA binding proteins
Prion-like RNA binding proteins (RBPs) such as TDP43 and FUS are largely soluble in the
nucleus but form solid pathological aggregates when mislocalized to the cytoplasm. What …
nucleus but form solid pathological aggregates when mislocalized to the cytoplasm. What …
Antisense proline-arginine RAN dipeptides linked to C9ORF72-ALS/FTD form toxic nuclear aggregates that initiate in vitro and in vivo neuronal death
Summary Expanded GGGGCC (G4C2) nucleotide repeats within the C9ORF72 gene are the
most common genetic mutation associated with both amyotrophic lateral sclerosis (ALS) and …
most common genetic mutation associated with both amyotrophic lateral sclerosis (ALS) and …
RNA transport and local translation in neurodevelopmental and neurodegenerative disease
MS Fernandopulle, J Lippincott-Schwartz… - Nature …, 2021 - nature.com
Neurons decentralize protein synthesis from the cell body to support the active metabolism
of remote dendritic and axonal compartments. The neuronal RNA transport apparatus …
of remote dendritic and axonal compartments. The neuronal RNA transport apparatus …
Stress granules as crucibles of ALS pathogenesis
Amyotrophic lateral sclerosis (ALS) is a fatal human neurodegenerative disease affecting
primarily motor neurons. Two RNA-binding proteins, TDP-43 and FUS, aggregate in the …
primarily motor neurons. Two RNA-binding proteins, TDP-43 and FUS, aggregate in the …
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 …
membraneless organelles with essential cellular function. However, aberrant phase …