Liquid–liquid phase separation in human health and diseases
Emerging evidence suggests that liquid–liquid phase separation (LLPS) represents a vital
and ubiquitous phenomenon underlying the formation of membraneless organelles in …
and ubiquitous phenomenon underlying the formation of membraneless organelles in …
RNA-binding proteins in human genetic disease
RNA-binding proteins (RBPs) are critical effectors of gene expression, and as such their
malfunction underlies the origin of many diseases. RBPs can recognize hundreds of …
malfunction underlies the origin of many diseases. RBPs can recognize hundreds of …
[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 …
found in pathological inclusions are known to undergo liquid-liquid phase separation, a …
[PDF][PDF] ALS genetics: gains, losses, and implications for future therapies
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 …
of motor neurons from the brain and spinal cord. The ALS community has made remarkable …
The role of TDP-43 mislocalization in amyotrophic lateral sclerosis
TR Suk, MWC Rousseaux - Molecular neurodegeneration, 2020 - Springer
Since its discovery as a primary component in cytoplasmic aggregates in post-mortem tissue
of patients with Amyotrophic Lateral Sclerosis (ALS), TAR DNA Binding Protein 43 kDa (TDP …
of patients with Amyotrophic Lateral Sclerosis (ALS), TAR DNA Binding Protein 43 kDa (TDP …
Tau activates microglia via the PQBP1-cGAS-STING pathway to promote brain inflammation
M Jin, H Shiwaku, H Tanaka, T Obita, S Ohuchi… - Nature …, 2021 - nature.com
Brain inflammation generally accompanies and accelerates neurodegeneration. Here we
report a microglial mechanism in which polyglutamine binding protein 1 (PQBP1) senses …
report a microglial mechanism in which polyglutamine binding protein 1 (PQBP1) senses …
[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 …
frontotemporal dementia (FTD) or amyotrophic lateral sclerosis (ALS). A key step that is …
Liquid-liquid phase separation in biology: mechanisms, physiological functions and human diseases
Cells are compartmentalized by numerous membrane-enclosed organelles and
membraneless compartments to ensure that a wide variety of cellular activities occur in a …
membraneless compartments to ensure that a wide variety of cellular activities occur in a …
[PDF][PDF] Cytoplasmic TDP-43 de-mixing independent of stress granules drives inhibition of nuclear import, loss of nuclear TDP-43, and cell death
While cytoplasmic aggregation of TDP-43 is a pathological hallmark of amyotrophic lateral
sclerosis and frontotemporal dementia, how aggregates form and what drives its nuclear …
sclerosis and frontotemporal dementia, how aggregates form and what drives its nuclear …
Distinct stages in stress granule assembly and disassembly
Stress granules are non-membrane bound RNA-protein (RNP) assemblies that form when
translation initiation is limited and contain a biphasic structure with stable core structures …
translation initiation is limited and contain a biphasic structure with stable core structures …