Molecular pathology of neurodegenerative diseases by cryo-EM of amyloids
Abnormal assembly of tau, α-synuclein, TDP-43 and amyloid-β proteins into amyloid
filaments defines most human neurodegenerative diseases. Genetics provides a direct link …
filaments defines most human neurodegenerative diseases. Genetics provides a direct link …
Amyotrophic lateral sclerosis: translating genetic discoveries into therapies
Recent advances in sequencing technologies and collaborative efforts have led to
substantial progress in identifying the genetic causes of amyotrophic lateral sclerosis (ALS) …
substantial progress in identifying the genetic causes of amyotrophic lateral sclerosis (ALS) …
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 …
glia is the defining pathological hallmark of the neurodegenerative disease amyotrophic …
TDP-43 triggers mitochondrial DNA release via mPTP to activate cGAS/STING in ALS
Cytoplasmic accumulation of TDP-43 is a disease hallmark for many cases of amyotrophic
lateral sclerosis (ALS), associated with a neuroinflammatory cytokine profile related to …
lateral sclerosis (ALS), associated with a neuroinflammatory cytokine profile related to …
Mitochondria as cellular and organismal signaling hubs
Mitochondria are traditionally known as the powerhouse of the cell, but their functions
extend far beyond energy production. They are vital in cellular and organismal pathways …
extend far beyond energy production. They are vital in cellular and organismal pathways …
Amyotrophic lateral sclerosis: a clinical review
P Masrori, P Van Damme - European journal of neurology, 2020 - Wiley Online Library
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder affecting primarily the
motor system, but in which extra‐motor manifestations are increasingly recognized. The loss …
motor system, but in which extra‐motor manifestations are increasingly recognized. The loss …
[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 …
ALS genetics, mechanisms, and therapeutics: where are we now?
The scientific landscape surrounding amyotrophic lateral sclerosis (ALS) continues to shift
as the number of genes associated with the disease risk and pathogenesis, and the cellular …
as the number of genes associated with the disease risk and pathogenesis, and the cellular …
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 …
characterizes nearly all cases of amyotrophic lateral sclerosis (ALS) and around half of …
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 …