[HTML][HTML] Liquid–liquid phase separation in human health and diseases

B Wang, L Zhang, T Dai, Z Qin, H Lu, L Zhang… - … and Targeted Therapy, 2021 - nature.com
Emerging evidence suggests that liquid–liquid phase separation (LLPS) represents a vital
and ubiquitous phenomenon underlying the formation of membraneless organelles in …

[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 …

Structure of pathological TDP-43 filaments from ALS with FTLD

D Arseni, M Hasegawa, AG Murzin, F Kametani, M Arai… - Nature, 2022 - nature.com
The abnormal aggregation of TAR DNA-binding protein 43 kDa (TDP-43) in neurons and
glia is the defining pathological hallmark of the neurodegenerative disease amyotrophic …

Stress granules and neurodegeneration

B Wolozin, P Ivanov - Nature Reviews Neuroscience, 2019 - nature.com
Recent advances suggest that the response of RNA metabolism to stress has an important
role in the pathophysiology of neurodegenerative diseases, particularly amyotrophic lateral …

[HTML][HTML] TDP-43 forms amyloid filaments with a distinct fold in type A FTLD-TDP

D Arseni, R Chen, AG Murzin, SY Peak-Chew… - Nature, 2023 - nature.com
The abnormal assembly of TAR DNA-binding protein 43 (TDP-43) in neuronal and glial cells
characterizes nearly all cases of amyotrophic lateral sclerosis (ALS) and around half of …

[HTML][HTML] 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 …

HSP70 chaperones RNA-free TDP-43 into anisotropic intranuclear liquid spherical shells

H Yu, S Lu, K Gasior, D Singh, S Vazquez-Sanchez… - Science, 2021 - science.org
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 …

[HTML][HTML] Molecular mechanisms of TDP-43 misfolding and pathology in amyotrophic lateral sclerosis

A Prasad, V Bharathi, V Sivalingam… - Frontiers in molecular …, 2019 - frontiersin.org
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 …

[HTML][HTML] The molecular language of membraneless organelles

E Gomes, J Shorter - Journal of Biological Chemistry, 2019 - ASBMB
Eukaryotic cells organize their intracellular components into organelles that can be
membrane-bound or membraneless. A large number of membraneless organelles, including …

FUS and TDP-43 phases in health and disease

B Portz, BL Lee, J Shorter - Trends in biochemical sciences, 2021 - cell.com
The distinct prion-like domains (PrLDs) of FUS and TDP-43, modulate phase transitions that
result in condensates with a range of material states. These assemblies are implicated in …