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[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 …
Molecular mechanisms of TDP-43 misfolding and pathology in amyotrophic lateral sclerosis
TAR DNA binding protein 43 (TDP-43) is a versatile RNA/DNA binding protein involved in
RNA-related metabolism. Hyper-phosphorylated and ubiquitinated TDP-43 deposits act as …
RNA-related metabolism. Hyper-phosphorylated and ubiquitinated TDP-43 deposits act as …
TDP-43 loss and ALS-risk SNPs drive mis-splicing and depletion of UNC13A
Variants of UNC13A, a critical gene for synapse function, increase the risk of amyotrophic
lateral sclerosis and frontotemporal dementia,–, two related neurodegenerative diseases …
lateral sclerosis and frontotemporal dementia,–, two related neurodegenerative diseases …
TDP-43 condensation properties specify its RNA-binding and regulatory repertoire
Mutations causing amyotrophic lateral sclerosis (ALS) often affect the condensation
properties of RNA-binding proteins (RBPs). However, the role of RBP condensation in the …
properties of RNA-binding proteins (RBPs). However, the role of RBP condensation in the …
HSP70 chaperones RNA-free TDP-43 into anisotropic intranuclear liquid spherical shells
INTRODUCTION Aggregation of the RNA binding protein TDP-43 (TAR DNA-binding protein
43) is a common pathological hallmark shared by several age-related neurodegenerative …
43) is a common pathological hallmark shared by several age-related neurodegenerative …
Seeding the aggregation of TDP-43 requires post-fibrillization proteolytic cleavage
Despite the strong evidence linking the transactive response DNA-binding protein 43 (TDP-
43) aggregation to the pathogenesis of frontotemporal lobar degeneration with TDP-43 …
43) aggregation to the pathogenesis of frontotemporal lobar degeneration with TDP-43 …
The role of TDP-43 propagation in neurodegenerative diseases: integrating insights from clinical and experimental studies
M Jo, S Lee, YM Jeon, S Kim, Y Kwon… - Experimental & molecular …, 2020 - nature.com
Abstract TAR DNA-binding protein 43 (TDP-43) is a highly conserved nuclear RNA/DNA-
binding protein involved in the regulation of RNA processing. The accumulation of TDP-43 …
binding protein involved in the regulation of RNA processing. The accumulation of TDP-43 …
TDP-43 α-helical structure tunes liquid–liquid phase separation and function
Liquid–liquid phase separation (LLPS) is involved in the formation of membraneless
organelles (MLOs) associated with RNA processing. The RNA-binding protein TDP-43 is …
organelles (MLOs) associated with RNA processing. The RNA-binding protein TDP-43 is …
Calcium permeable-AMPA receptors and excitotoxicity in neurological disorders
C Guo, YY Ma - Frontiers in neural circuits, 2021 - frontiersin.org
Excitotoxicity is one of the primary mechanisms of cell loss in a variety of diseases of the
central and peripheral nervous systems. Other than the previously established signaling …
central and peripheral nervous systems. Other than the previously established signaling …
ALS mutations disrupt phase separation mediated by α-helical structure in the TDP-43 low-complexity C-terminal domain
RNA-binding protein TDP-43 mediates essential RNA processing but forms cytoplasmic
neuronal inclusions via its C-terminal domain (CTD) in amyotrophic lateral sclerosis (ALS). It …
neuronal inclusions via its C-terminal domain (CTD) in amyotrophic lateral sclerosis (ALS). It …