Defects in synaptic transmission at the neuromuscular junction precede motor deficits in a TDP‐43Q331K transgenic mouse model of amyotrophic lateral sclerosis
Transactive response DNA‐binding protein‐43 (TDP‐43) is involved in gene regulation via
the control of RNA transcription, splicing, and transport. TDP‐43 is a major protein …
the control of RNA transcription, splicing, and transport. TDP‐43 is a major protein …
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 …
[HTML][HTML] Development of abnormalities at the neuromuscular junction in the SOD1-G93A mouse model of ALS: dysfunction then disruption of postsynaptic structure …
J McIntosh, I Mekrouda, M Dashti… - Frontiers in Molecular …, 2023 - frontiersin.org
The ultimate deficit in ALS is neuromuscular junction (NMJ) loss, producing permanent
paralysis, ultimately in respiratory muscles. However, understanding the functional and …
paralysis, ultimately in respiratory muscles. However, understanding the functional and …
Opposite synaptic alterations at the neuromuscular junction in an ALS mouse model: when motor units matter
E Tremblay, É Martineau, R Robitaille - Journal of Neuroscience, 2017 - Soc Neuroscience
Denervation of the neuromuscular junction (NMJ) precedes the loss of motor neurons (MNs)
in amyotrophic lateral sclerosis (ALS). ALS is characterized by a motor unit (MU)-dependent …
in amyotrophic lateral sclerosis (ALS). ALS is characterized by a motor unit (MU)-dependent …
Analysis of neuromuscular junctions and effects of anabolic steroid administration in the SOD1G93A mouse model of ALS
Several lines of evidence indicate that neuromuscular junction (NMJ) destruction and
disassembly is an early phenomenon in amyotrophic lateral sclerosis (ALS). Here we …
disassembly is an early phenomenon in amyotrophic lateral sclerosis (ALS). Here we …
Neuromuscular junction destruction during amyotrophic lateral sclerosis: insights from transgenic models
L Dupuis, JP Loeffler - Current opinion in pharmacology, 2009 - Elsevier
Amyotrophic lateral sclerosis (ALS) represents the major adult-onset motor neuron disease.
Analyses of ALS animal models have shown that motor neuron death starts with …
Analyses of ALS animal models have shown that motor neuron death starts with …
Loss of TDP-43 causes age-dependent progressive motor neuron degeneration
Y Iguchi, M Katsuno, J Niwa, S Takagi, S Ishigaki… - Brain, 2013 - academic.oup.com
Amyotrophic lateral sclerosis is a devastating, progressive neurodegenerative disease that
affects upper and lower motor neurons. Although several genes are identified as the cause …
affects upper and lower motor neurons. Although several genes are identified as the cause …
Small junction, big problems: Neuromuscular junction pathology in mouse models of amyotrophic lateral sclerosis (ALS)
Amyotrophic lateral sclerosis (ALS) is a motor neuron disease with an extremely
heterogeneous clinical and genetic phenotype. In our efforts to find therapies for ALS, the …
heterogeneous clinical and genetic phenotype. In our efforts to find therapies for ALS, the …
Shortened TDP43 isoforms upregulated by neuronal hyperactivity drive TDP43 pathology in ALS
K Weskamp, EM Tank, R Miguez… - The Journal of …, 2020 - Am Soc Clin Investig
Cortical hyperexcitability and mislocalization of the RNA-binding protein TDP43 are highly
conserved features in amyotrophic lateral sclerosis (ALS). Nevertheless, the relationship …
conserved features in amyotrophic lateral sclerosis (ALS). Nevertheless, the relationship …
Early and persistent abnormal decoding by glial cells at the neuromuscular junction in an ALS model
D Arbour, E Tremblay, É Martineau… - Journal of …, 2015 - Soc Neuroscience
Amyotrophic lateral sclerosis (ALS) is a late-onset neuromuscular disease characterized by
progressive loss of motor neurons (MNs) preceded by neuromuscular junction (NMJ) …
progressive loss of motor neurons (MNs) preceded by neuromuscular junction (NMJ) …