ALS genetics, mechanisms, and therapeutics: where are we now?
The scientific landscape surrounding amyotrophic lateral sclerosis (ALS) continues to shift
as the number of genes associated with the disease risk and pathogenesis, and the cellular …
as the number of genes associated with the disease risk and pathogenesis, and the cellular …
The molecular language of membraneless organelles
Eukaryotic cells organize their intracellular components into organelles that can be
membrane-bound or membraneless. A large number of membraneless organelles, including …
membrane-bound or membraneless. A large number of membraneless organelles, including …
Molecular interactions underlying liquid− liquid phase separation of the FUS low-complexity domain
The low-complexity domain of the RNA-binding protein FUS (FUS LC) mediates liquid−
liquid phase separation (LLPS), but the interactions between the repetitive SYGQ-rich …
liquid phase separation (LLPS), but the interactions between the repetitive SYGQ-rich …
[HTML][HTML] Neurodegenerative disease: models, mechanisms, and a new hope
Neurodegeneration is a feature of many debilitating, incurable diseases that are rapidly
rising in prevalence, such as Parkinson's disease. There is an urgent need to develop new …
rising in prevalence, such as Parkinson's disease. There is an urgent need to develop new …
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 …
ALS/FTD mutation-induced phase transition of FUS liquid droplets and reversible hydrogels into irreversible hydrogels impairs RNP granule function
T Murakami, S Qamar, JQ Lin, GSK Schierle, E Rees… - Neuron, 2015 - cell.com
The mechanisms by which mutations in FUS and other RNA binding proteins cause ALS
and FTD remain controversial. We propose a model in which low-complexity (LC) domains …
and FTD remain controversial. We propose a model in which low-complexity (LC) domains …
Paraspeckles: where long noncoding RNA meets phase separation
Long noncoding RNA (lncRNA) molecules are some of the newest and least understood
players in gene regulation. Hence, we need good model systems with well-defined RNA and …
players in gene regulation. Hence, we need good model systems with well-defined RNA and …
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 …
FUS-dependent liquid–liquid phase separation is important for DNA repair initiation
BR Levone, SC Lenzken, M Antonaci, A Maiser… - Journal of Cell …, 2021 - rupress.org
RNA-binding proteins (RBPs) are emerging as important effectors of the cellular DNA
damage response (DDR). The RBP FUS is implicated in RNA metabolism and DNA repair …
damage response (DDR). The RBP FUS is implicated in RNA metabolism and DNA repair …
Converging mechanisms in ALS and FTD: disrupted RNA and protein homeostasis
Breakthrough discoveries identifying common genetic causes for amyotrophic lateral
sclerosis (ALS) and frontotemporal dementia (FTD) have transformed our view of these …
sclerosis (ALS) and frontotemporal dementia (FTD) have transformed our view of these …