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Amyotrophic lateral sclerosis: a neurodegenerative disorder poised for successful therapeutic translation
Amyotrophic lateral sclerosis (ALS) is a devastating disease caused by degeneration of
motor neurons. As with all major neurodegenerative disorders, development of disease …
motor neurons. As with all major neurodegenerative disorders, development of disease …
Animal models of neurodegenerative diseases
Animal models of adult-onset neurodegenerative diseases have enhanced the
understanding of the molecular pathogenesis of Alzheimer's disease, Parkinson's disease …
understanding of the molecular pathogenesis of Alzheimer's disease, Parkinson's disease …
[HTML][HTML] Phase separation of FUS is suppressed by its nuclear import receptor and arginine methylation
M Hofweber, S Hutten, B Bourgeois, E Spreitzer… - Cell, 2018 - cell.com
Cytoplasmic FUS aggregates are a pathological hallmark in a subset of patients with
frontotemporal dementia (FTD) or amyotrophic lateral sclerosis (ALS). A key step that is …
frontotemporal dementia (FTD) or amyotrophic lateral sclerosis (ALS). A key step that is …
Axonal transport and neurological disease
Axonal transport is the process whereby motor proteins actively navigate microtubules to
deliver diverse cargoes, such as organelles, from one end of the axon to the other, and is …
deliver diverse cargoes, such as organelles, from one end of the axon to the other, and is …
The overlap** genetics of amyotrophic lateral sclerosis and frontotemporal dementia
Amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) are two diseases
that form a broad neurodegenerative continuum. Considerable effort has been made to …
that form a broad neurodegenerative continuum. Considerable effort has been made to …
Neuromuscular junction dysfunction in amyotrophic lateral sclerosis
Amyotrophic lateral sclerosis (ALS) is a fatal neurological disorder characterized by
progressive degeneration of motor neurons leading to skeletal muscle denervation. Earlier …
progressive degeneration of motor neurons leading to skeletal muscle denervation. Earlier …
hnRNP A1 dysfunction alters RNA splicing and drives neurodegeneration in multiple sclerosis (MS)
Neurodegeneration is the primary driver of disease progression in multiple sclerosis (MS)
resulting in permanent disability, creating an urgent need to discover its underlying …
resulting in permanent disability, creating an urgent need to discover its underlying …
ALS/FTD-linked mutation in FUS suppresses intra-axonal protein synthesis and drives disease without nuclear loss-of-function of FUS
Through the generation of humanized FUS mice expressing full-length human FUS, we
identify that when expressed at near endogenous murine FUS levels, both wild-type and …
identify that when expressed at near endogenous murine FUS levels, both wild-type and …
Nearly 30 years of animal models to study amyotrophic lateral sclerosis: a historical overview and future perspectives
T Bonifacino, RA Zerbo, M Balbi, C Torazza… - International journal of …, 2021 - mdpi.com
Amyotrophic lateral sclerosis (ALS) is a fatal, multigenic, multifactorial, and non-cell
autonomous neurodegenerative disease characterized by upper and lower motor neuron …
autonomous neurodegenerative disease characterized by upper and lower motor neuron …
Nutritional and metabolic factors in amyotrophic lateral sclerosis
Amyotrophic lateral sclerosis (ALS) is a complex neurodegenerative disease that is
classically thought to impact the motor system. Over the past 20 years, research has started …
classically thought to impact the motor system. Over the past 20 years, research has started …