Defects in synaptic transmission at the neuromuscular junction precede motor deficits in a TDP‐43Q331K transgenic mouse model of amyotrophic lateral sclerosis

KK Chand, KM Lee, JD Lee, H Qiu, EF Willis… - The FASEB …, 2018 - Wiley Online Library
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 …

Neuromuscular junction dysfunction in amyotrophic lateral sclerosis

S Verma, S Khurana, A Vats, B Sahu, NK Ganguly… - Molecular …, 2022 - Springer
Amyotrophic lateral sclerosis (ALS) is a fatal neurological disorder characterized by
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 …

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 …

Analysis of neuromuscular junctions and effects of anabolic steroid administration in the SOD1G93A mouse model of ALS

V Cappello, E Vezzoli, M Righi, M Fossati… - Molecular and Cellular …, 2012 - Elsevier
Several lines of evidence indicate that neuromuscular junction (NMJ) destruction and
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 …

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 …

Small junction, big problems: Neuromuscular junction pathology in mouse models of amyotrophic lateral sclerosis (ALS)

A Alhindi, I Boehm, H Chaytow - Journal of Anatomy, 2022 - Wiley Online Library
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 …

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 …

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) …