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 …
Non-neuronal cells in amyotrophic lateral sclerosis—from pathogenesis to biomarkers
The prevailing motor neuron-centric view of amyotrophic lateral sclerosis (ALS)
pathogenesis could be an important factor in the failure to identify disease-modifying therapy …
pathogenesis could be an important factor in the failure to identify disease-modifying therapy …
TDP-43 aggregation induced by oxidative stress causes global mitochondrial imbalance in ALS
X Zuo, J Zhou, Y Li, K Wu, Z Chen, Z Luo… - Nature structural & …, 2021 - nature.com
Amyotrophic lateral sclerosis (ALS) was initially thought to be associated with oxidative
stress when it was first linked to mutant superoxide dismutase 1 (SOD1). The subsequent …
stress when it was first linked to mutant superoxide dismutase 1 (SOD1). The subsequent …
Converging mechanisms in ALS and FTD: disrupted RNA and protein homeostasis
Breakthrough discoveries identifying common genetic causes for amyotrophic lateral
sclerosis (ALS) and frontotemporal dementia (FTD) have transformed our view of these …
sclerosis (ALS) and frontotemporal dementia (FTD) have transformed our view of these …
Guidelines for the use and interpretation of assays for monitoring autophagy (4th edition)1
DJ Klionsky, AK Abdel-Aziz, S Abdelfatah, M Abdellatif… - autophagy, 2021 - Taylor & Francis
In 2008, we published the first set of guidelines for standardizing research in autophagy.
Since then, this topic has received increasing attention, and many scientists have entered …
Since then, this topic has received increasing attention, and many scientists have entered …
Autophagy and ALS: mechanistic insights and therapeutic implications
JP Chua, H De Calbiac, E Kabashi, SJ Barmada - Autophagy, 2022 - Taylor & Francis
Mechanisms of protein homeostasis are crucial for overseeing the clearance of misfolded
and toxic proteins over the lifetime of an organism, thereby ensuring the health of neurons …
and toxic proteins over the lifetime of an organism, thereby ensuring the health of neurons …
Amyotrophic lateral sclerosis—a model of corticofugal axonal spread
H Braak, J Brettschneider, AC Ludolph… - Nature Reviews …, 2013 - nature.com
The pathological process underlying amyotrophic lateral sclerosis (ALS) is associated with
the formation of cytoplasmic inclusions consisting mainly of phosphorylated 43-kDa …
the formation of cytoplasmic inclusions consisting mainly of phosphorylated 43-kDa …
TDP-43 repression of nonconserved cryptic exons is compromised in ALS-FTD
Cytoplasmic aggregation of TDP-43, accompanied by its nuclear clearance, is a key
common pathological hallmark of amyotrophic lateral sclerosis and frontotemporal dementia …
common pathological hallmark of amyotrophic lateral sclerosis and frontotemporal dementia …
Gains or losses: molecular mechanisms of TDP43-mediated neurodegeneration
RNA-binding proteins, and in particular TAR DNA-binding protein 43 (TDP43), are central to
the pathogenesis of motor neuron diseases and related neurodegenerative disorders …
the pathogenesis of motor neuron diseases and related neurodegenerative disorders …
ALS-linked TDP-43 mutations produce aberrant RNA splicing and adult-onset motor neuron disease without aggregation or loss of nuclear TDP-43
Transactivating response region DNA binding protein (TDP-43) is the major protein
component of ubiquitinated inclusions found in amyotrophic lateral sclerosis (ALS) and …
component of ubiquitinated inclusions found in amyotrophic lateral sclerosis (ALS) and …