[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 …

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 …

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 …

It's not just a phase: function and characteristics of RNA-binding proteins in phase separation

HJ Wiedner, J Giudice - Nature structural & molecular biology, 2021 - nature.com
Biomolecular condensates that form via phase separation are increasingly regarded as
coordinators of cellular reactions that regulate a wide variety of biological phenomena …

The overlapping genetics of amyotrophic lateral sclerosis and frontotemporal dementia

YA Abramzon, P Fratta, BJ Traynor… - Frontiers in neuroscience, 2020 - frontiersin.org
Amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) are two diseases
that form a broad neurodegenerative continuum. Considerable effort has been made to …

Oxidative stress in amyotrophic lateral sclerosis: pathophysiology and opportunities for pharmacological intervention

T Cunha-Oliveira, L Montezinho… - Oxidative medicine …, 2020 - Wiley Online Library
Amyotrophic lateral sclerosis (ALS), also known as Lou Gehrig's disease or Charcot
disease, is a fatal neurodegenerative disease that affects motor neurons (MNs) and leads to …

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 …

ALS monocyte-derived microglia-like cells reveal cytoplasmic TDP-43 accumulation, DNA damage, and cell-specific impairment of phagocytosis associated with …

H Quek, C Cuní-López, R Stewart, T Colletti… - Journal of …, 2022 - Springer
Background Amyotrophic lateral sclerosis (ALS) is a multifactorial neurodegenerative
disease characterised by the loss of upper and lower motor neurons. Increasing evidence …

Transposable elements, inflammation, and neurological disease

A Saleh, A Macia, AR Muotri - Frontiers in neurology, 2019 - frontiersin.org
Transposable Elements (TE) are mobile DNA elements that can replicate and insert
themselves into different locations within the host genome. Their propensity to self …

Do post-translational modifications influence protein aggregation in neurodegenerative diseases: a systematic review

LN Schaffert, WG Carter - Brain sciences, 2020 - mdpi.com
The accumulation of abnormal protein aggregates represents a universal hallmark of
neurodegenerative diseases (NDDs). Post-translational modifications (PTMs) regulate …