ALS genetics: gains, losses, and implications for future therapies
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disorder caused by the loss
of motor neurons from the brain and spinal cord. The ALS community has made remarkable …
of motor neurons from the brain and spinal cord. The ALS community has made remarkable …
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 …
Mis-spliced transcripts generate de novo proteins in TDP-43–related ALS/FTD
Functional loss of TDP-43, an RNA binding protein genetically and pathologically linked to
amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD), leads to the …
amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD), leads to the …
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 …
RNA-related metabolism. Hyper-phosphorylated and ubiquitinated TDP-43 deposits act as …
ALS-implicated protein TDP-43 sustains levels of STMN2, a mediator of motor neuron growth and repair
JR Klim, LA Williams, F Limone… - Nature …, 2019 - nature.com
The findings that amyotrophic lateral sclerosis (ALS) patients almost universally display
pathological mislocalization of the RNA-binding protein TDP-43 and that mutations in its …
pathological mislocalization of the RNA-binding protein TDP-43 and that mutations in its …
Therapeutic reduction of ataxin-2 extends lifespan and reduces pathology in TDP-43 mice
Amyotrophic lateral sclerosis (ALS) is a rapidly progressing neurodegenerative disease that
is characterized by motor neuron loss and that leads to paralysis and death 2–5 years after …
is characterized by motor neuron loss and that leads to paralysis and death 2–5 years after …
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 …
[HTML][HTML] TDP-43 proteinopathy and ALS: insights into disease mechanisms and therapeutic targets
Therapeutic options for patients with amyotrophic lateral sclerosis (ALS) are currently
limited. However, recent studies show that almost all cases of ALS, as well as tau-negative …
limited. However, recent studies show that almost all cases of ALS, as well as tau-negative …
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 …
The inhibition of TDP-43 mitochondrial localization blocks its neuronal toxicity
Genetic mutations in TAR DNA-binding protein 43 (TARDBP, also known as TDP-43) cause
amyotrophic lateral sclerosis (ALS), and an increase in the presence of TDP-43 (encoded by …
amyotrophic lateral sclerosis (ALS), and an increase in the presence of TDP-43 (encoded by …