iPSC-derived functional human neuromuscular junctions model the pathophysiology of neuromuscular diseases [PDF]
JCI Insight, 2019 The control of voluntary skeletal muscle contraction relies on action potentials, which send signals from the motor neuron through the neuromuscular junction (NMJ). Although dysfunction of the NMJ causes various neuromuscular diseases, a reliable in vitro system for disease modeling is currently unavailable. Here, we present a potentially novel 2-step,
Chuang-Yu Lin +9 more
openaire +2 more sources
Idiopathic Pulmonary Fibrosis and Myasthenia Gravis: An Unusual Association [PDF]
Journal of Clinical and Diagnostic Research, 2016 Idiopathic Pulmonary Fibrosis (IPF) is a chronic fibrosing lung condition with high morbidity and mortality, accounting for about 25% of the cases of interstitial lung diseases.
Bharti Chogtu +2 more
doaj +1 more source
Restoration of SMN in Schwann cells reverses myelination defects and improves neuromuscular function in spinal muscular atrophy [PDF]
, 2016 Spinal muscular atrophy (SMA) is a neuromuscular disease caused by low levels of SMN protein, primarily affecting lower motor neurons. Recent evidence from SMA and related conditions suggests that glial cells can influence disease severity.
Brophy, Peter J. +9 more
core +3 more sources
O-GlcNAcase contributes to cognitive function in Drosophila [PDF]
, 2020 Contains fulltext : 219183.pdf (publisher's version ) (Open ...
Catinozzi, Marica +7 more
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eLife, 2019 Overproduction of reactive oxygen species (ROS) is known to mediate glutamate excitotoxicity in neurological diseases. However, how ROS burdens can influence neural circuit integrity remains unclear.
Jhan-Jie Peng +5 more
doaj +1 more source
Adaptive Remodeling of the Neuromuscular Junction with Aging
Cells, 2022 Aging is associated with gradual degeneration, in mass and function, of the neuromuscular system. This process, referred to as “sarcopenia”, is considered a disease by itself, and it has been linked to a number of other serious maladies such as type II ...
Michael R. Deschenes +4 more
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Snake and spider toxins induce a rapid recovery of function of botulinum neurotoxin paralysed neuromuscular junction [PDF]
, 2015 Botulinum neurotoxins (BoNTs) and some animal neurotoxins (-Bungarotoxin, -Btx, from elapid snakes and -Latrotoxin, -Ltx, from black widow spiders) are pre-synaptic neurotoxins that paralyse motor axon terminals with similar clinical outcomes in patients.
Duregotti, Elisa +6 more
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Growth hormone plus resistance exercise attenuate structural changes in rat myotendinous junctions resulting from chronic unloading. [PDF]
, 2013 Myotendinous junctions (MTJs) are specialized sites on the muscle surface where forces generated by myofibrils are transmitted across the sarcolemma to the extracellular matrix.
Burattini, S +8 more
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Neuromuscular Junction as an Entity of Nerve-Muscle Communication
Cells, 2019 One of the crucial systems severely affected in several neuromuscular diseases is the loss of effective connection between muscle and nerve, leading to a pathological non-communication between the two tissues.
Elisa Lepore +3 more
doaj +1 more source
Neuromuscular junction-specific genes screening by deep RNA-seq analysis
Cell & Bioscience, 2021 Background Neuromuscular junctions (NMJs) are chemical synapses formed between motor neurons and skeletal muscle fibers and are essential for controlling muscle contraction.
Tiankun Hui, Hongyang Jing, Xinsheng Lai
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