Riluzole and edaravone: A tale of two amyotrophic lateral sclerosis drugs
MK Jaiswal - Medicinal Research Reviews, 2019 - Wiley Online Library
Over the past decades, a multitude of experimental drugs have been shown to delay
disease progression in preclinical animal models of amyotrophic lateral sclerosis (ALS) but …
disease progression in preclinical animal models of amyotrophic lateral sclerosis (ALS) but …
Motor neuron vulnerability and resistance in amyotrophic lateral sclerosis
J Nijssen, LH Comley, E Hedlund - Acta neuropathologica, 2017 - Springer
In the fatal disease—amyotrophic lateral sclerosis (ALS)—upper (corticospinal) motor
neurons (MNs) and lower somatic MNs, which innervate voluntary muscles, degenerate …
neurons (MNs) and lower somatic MNs, which innervate voluntary muscles, degenerate …
Axonal TDP-43 condensates drive neuromuscular junction disruption through inhibition of local synthesis of nuclear encoded mitochondrial proteins
Mislocalization of the predominantly nuclear RNA/DNA binding protein, TDP-43, occurs in
motor neurons of~ 95% of amyotrophic lateral sclerosis (ALS) patients, but the contribution …
motor neurons of~ 95% of amyotrophic lateral sclerosis (ALS) patients, but the contribution …
Microglia-mediated recovery from ALS-relevant motor neuron degeneration in a mouse model of TDP-43 proteinopathy
Though motor neurons selectively degenerate in amyotrophic lateral sclerosis, other cell
types are likely involved in this disease. We recently generated rNLS8 mice in which human …
types are likely involved in this disease. We recently generated rNLS8 mice in which human …
The debated toxic role of aggregated TDP-43 in amyotrophic lateral sclerosis: a resolution in sight?
RC Hergesheimer, AA Chami, DR De Assis, P Vourc'h… - Brain, 2019 - academic.oup.com
Transactive response DNA-binding protein-43 (TDP-43) is an RNA/DNA binding protein that
forms phosphorylated and ubiquitinated aggregates in the cytoplasm of motor neurons in …
forms phosphorylated and ubiquitinated aggregates in the cytoplasm of motor neurons in …
Motor neuron susceptibility in ALS/FTD
AMG Ragagnin, S Shadfar, M Vidal… - Frontiers in …, 2019 - frontiersin.org
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by the
death of both upper and lower motor neurons (MNs) in the brain, brainstem and spinal cord …
death of both upper and lower motor neurons (MNs) in the brain, brainstem and spinal cord …
Microglia in neurodegenerative diseases
A major feature of neurodegeneration is disruption of central nervous system homeostasis,
during which microglia play diverse roles. In the central nervous system, microglia serve as …
during which microglia play diverse roles. In the central nervous system, microglia serve as …
The emerging role of central and peripheral immune systems in neurodegenerative diseases
X Zang, S Chen, JY Zhu, J Ma, Y Zhai - Frontiers in aging …, 2022 - frontiersin.org
For decades, it has been widely believed that the blood–brain barrier (BBB) provides an
immune privileged environment in the central nervous system (CNS) by blocking peripheral …
immune privileged environment in the central nervous system (CNS) by blocking peripheral …
Amyotrophic lateral sclerosis and autophagy: dysfunction and therapeutic targeting
Over the past 20 years, there has been a drastically increased understanding of the genetic
basis of Amyotrophic Lateral Sclerosis. Despite the identification of more than 40 different …
basis of Amyotrophic Lateral Sclerosis. Despite the identification of more than 40 different …
Locomotor deficits in a mouse model of ALS are paralleled by loss of V1-interneuron connections onto fast motor neurons
ALS is characterized by progressive inability to execute movements. Motor neurons
innervating fast-twitch muscle-fibers preferentially degenerate. The reason for this …
innervating fast-twitch muscle-fibers preferentially degenerate. The reason for this …