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 …
FUS affects circular RNA expression in murine embryonic stem cell-derived motor neurons
L Errichelli, S Dini Modigliani, P Laneve… - Nature …, 2017 - nature.com
The RNA-binding protein FUS participates in several RNA biosynthetic processes and has
been linked to the pathogenesis of amyotrophic lateral sclerosis (ALS) and frontotemporal …
been linked to the pathogenesis of amyotrophic lateral sclerosis (ALS) and frontotemporal …
RGG/RG motif regions in RNA binding and phase separation
PA Chong, RM Vernon, JD Forman-Kay - Journal of molecular biology, 2018 - Elsevier
Abstract RGG/RG motifs are RNA binding segments found in many proteins that can partition
into membraneless organelles. They occur in the context of low-complexity disordered …
into membraneless organelles. They occur in the context of low-complexity disordered …
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 …
Liquid–liquid phase separation in disease
S Alberti, D Dormann - Annual review of genetics, 2019 - annualreviews.org
We have made rapid progress in recent years in identifying the genetic causes of many
human diseases. However, despite this recent progress, our mechanistic understanding of …
human diseases. However, despite this recent progress, our mechanistic understanding of …
[PDF][PDF] Nuclear import receptor inhibits phase separation of FUS through binding to multiple sites
Liquid-liquid phase separation (LLPS) is believed to underlie formation of biomolecular
condensates, cellular compartments that concentrate macromolecules without surrounding …
condensates, cellular compartments that concentrate macromolecules without surrounding …
Modifiers of C9orf72 dipeptide repeat toxicity connect nucleocytoplasmic transport defects to FTD/ALS
C9orf72 mutations are the most common cause of amyotrophic lateral sclerosis (ALS) and
frontotemporal dementia (FTD). Dipeptide repeat proteins (DPRs) produced by …
frontotemporal dementia (FTD). Dipeptide repeat proteins (DPRs) produced by …
[PDF][PDF] 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 …
Physiological functions and pathobiology of TDP‐43 and FUS/TLS proteins
The multiple roles played by RNA binding proteins in neurodegeneration have become
apparent following the discovery of TAR DNA binding protein 43 kDa (TDP‐43) and fused in …
apparent following the discovery of TAR DNA binding protein 43 kDa (TDP‐43) and fused in …
[HTML][HTML] 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 …