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Neuromuscular junction

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https://www.readbyqxmd.com/read/28647557/kcc3-loss-of-function-contributes-to-andermann-syndrome-by-inducing-activity-dependent-neuromuscular-junction-defects
#1
Melissa Bowerman, Céline Salsac, Véronique Bernard, Claire Soulard, Annie Dionne, Emmanuelle Coque, Salim Benlefki, Pascale Hince, Patrick A Dion, Gillian Butler-Browne, William Camu, Jean-Pierre Bouchard, Eric Delpire, Guy A Rouleau, Cédric Raoul, Frédérique Scamps
Loss-of-function mutations in the potassium-chloride cotransporter KCC3 lead to Andermann syndrome, a severe sensorimotor neuropathy characterized by areflexia, amyotrophy and locomotor abnormalities. The molecular events responsible for axonal loss remain poorly understood. Here, we establish that global or neuron-specific KCC3 loss-of-function in mice leads to early neuromuscular junction (NMJ) abnormalities and muscular atrophy that are consistent with the pre-synaptic neurotransmission defects observed in patients...
June 21, 2017: Neurobiology of Disease
https://www.readbyqxmd.com/read/28645846/heparan-sulfate-proteoglycans-in-drosophila-neuromuscular-development
#2
REVIEW
Keisuke Kamimura, Nobuaki Maeda
Heparan sulfate proteoglycans (HSPGs) are glycoconjugates bearing heparan sulfate (HS) chains covalently attached to core proteins, which are ubiquitously distributed on the cell surface and in the extracellular matrix. HSPGs interact with a number of molecules mainly through HS chains, which play critical roles in diverse physiological and disease processes. Among these, recent vertebrate studies showed that HSPGs are closely involved in synapse development and function. However, the detailed molecular mechanisms remain elusive...
June 20, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/28645175/perturbation-to-cholesterol-at-the-neuromuscular-junction-confers-botulinum-neurotoxin-a-sensitivity-to-neonatal-mice
#3
Baskaran Thyagarajan, Joseph G Potian, Joseph J McArdle, Padmamalini Baskaran
Botulinum neurotoxin A (BoNT/A) cleaves SNAP25 at the motor nerve terminals (MNT) and inhibits stimulus evoked acetylcholine release (SEAR). This causes skeletal muscle paralysis. However, younger neonatal mice (<P7; less than 7 days old) are resistant to the neuroparalytic effects of BoNT/A. That is, in vivo injection of BoNT/A at the innervations of Extensor digitorum longus (EDL) muscle in the hindlimbs inhibited the toe spread reflex within 24 hours following BoNT/A injection in adult mouse and in older (> P7) neonatal mice younger than 7 days-age remained unaffected by BoNT/A injection...
June 22, 2017: Toxicological Sciences: An Official Journal of the Society of Toxicology
https://www.readbyqxmd.com/read/28642851/sarcopenia-neurological-point-of-view
#4
REVIEW
Young Nam Kwon, Sung Sang Yoon
Sarcopenia is an age-related geriatric syndrome which is characterized by the gradual loss of muscle mass, muscle strength, and muscle quality. There are a lot of neurologic insults on sarcopenia at various levels from the brain to the neuromuscular junctions (NMJs) to generate a volitional task. Dopaminergic downregulation, inadequate motor programming and motor coordination impairment lead to decline of supraspinal drive. Motor unit reorganization and inflammatory changes in motor neuron decrease conduction velocity and amplitude of compound muscle action potential...
May 2017: Journal of Bone Metabolism
https://www.readbyqxmd.com/read/28642541/can-uk-undergraduate-dental-programmes-provide-training-in-non-surgical-facial-aesthetics
#5
T W M Walker, F Gately, S Stagnell, A Kerai, C Mills, S Thomas
Aim Recently, more and more dentists have found themselves engaging in the delivery of non-surgical facial aesthetics (NSFA) as part of their regular practice routine. NSFA is a growing field in aesthetic medicine that is practised by a range of clinicians including doctors, dentists and registered prescriber nurses and is an industry estimated to be worth over £3 billion in the UK alone. In the past few years, several public scandals in aesthetic medicine have prompted reactions by several bodies including the Government and Royal Colleges...
June 23, 2017: British Dental Journal
https://www.readbyqxmd.com/read/28638701/reinnervation-of-denervated-muscle-by-implantation-of-nerve-muscle-endplate-band-graft-to-the-native-motor-zone-of-the-target-muscle
#6
Liancai Mu, Stanislaw Sobotka, Jingming Chen, Themba Nyirenda
INTRODUCTION: Motor endplate reinnervation is critical for restoring motor function of the denervated muscle. We developed a novel surgical technique called nerve-muscle-endplate band grafting (NMEG) for muscle reinnervation. METHODS: Experimentally denervated sternomastoid muscle in the rat was reinnervated by transferring a NMEG from the ipsilateral sternohyoid muscle to the native motor zone (NMZ) of the target muscle. A NMEG pedicle contained a block of muscle (~ 6 × 6 × 3 mm), a nerve branch with axon terminals, and a motor endplate band with numerous neuromuscular junctions...
June 2017: Brain and Behavior
https://www.readbyqxmd.com/read/28637657/seasonal-factors-influence-quantal-transmitter-release-and-calcium-dependence-at-amphibian-neuromuscular-junctions
#7
Dengyun Ge, Nickolas Anastasios Lavidis
Amphibian neuromuscular junctions (NMJs) are composed of hundreds of neurotransmitter release sites which exhibit non-uniform transmitter release probabilities and demonstrated seasonal modulation. We examined whether recruitment of release sites is variable when the extracellular calcium concentration ([Ca(2+)]o) is increased in the wet and dry seasons. The amount of transmitter released from the entire nerve terminal increases by approximately the 4(th)-power as [Ca(2+)]o is increased. Toad (Bufo marinus) NMJs were visualised using DiOC2(5)-fluorescence and focal loose patch extracellular recordings were used to record the end-plate currents (EPCs) from small groups of release sites...
June 21, 2017: American Journal of Physiology. Regulatory, Integrative and Comparative Physiology
https://www.readbyqxmd.com/read/28635376/advances-in-understanding-the-role-of-disease-associated-proteins-in-spinal-muscular-atrophy
#8
Seyyedmohsen Hosseinibarkooie, Svenja Schneider, Brunhilde Wirth
Spinal muscular atrophy (SMA) is a neurodegenerative disorder characterized by alpha motor neuron loss in the spinal cord due to reduced survival of motor neuron (SMN) protein level. While the genetic basis of SMA is well described, the specific molecular pathway underlying SMA is still not fully understood. Areas covered: This review discusses the recent advancements in understanding the molecular pathways in SMA using different omics approaches and genetic modifiers identified in both vertebrate and invertebrate systems...
June 21, 2017: Expert Review of Proteomics
https://www.readbyqxmd.com/read/28630297/correction-for-khan-et-al-sympathetic-innervation-controls-homeostasis-of-neuromuscular-junctions-in-health-and-disease
#9
(no author information available yet)
No abstract text is available yet for this article.
June 19, 2017: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/28624507/effect-of-pyridostigmine-on-in-vivo-and-in-vitro-respiratory-muscle-of-mdx-mice
#10
Gabriela de Cássia Sousa Amancio, Andrea Grabe-Guimarães, Dridi Haikel, Johan Moreau, Neila Marcia Silva Barcellos, Alain Lacampagne, Stefan Matecki, Olivier Cazorla
The current work was conducted to verify the contribution of neuromuscular transmission defects at the neuromuscular junction to Duchenne Muscular Dystrophy disease progression and respiratory dysfunction. We tested pyridostigmine and pyridostigmine encapsulated in liposomes (liposomal PYR), an acetylcholinesterase inhibitor to improve muscular contraction on respiratory muscle function in mdx mice at different ages. We evaluated in vivo with the whole-body plethysmography, the ventilatory response to hypercapnia, and measured in vitro diaphragm strength in each group...
June 15, 2017: Respiratory Physiology & Neurobiology
https://www.readbyqxmd.com/read/28620838/modelling-fus-mislocalisation-in-an-in-vitro-model-of-innervated-human-muscle
#11
Sonja Prpar Mihevc, Mojca Pavlin, Simona Darovic, Marko Živin, Matej Podbregar, Boris Rogelj, Tomaz Mars
Degeneration of distal axons and neuromuscular junctions is an early feature in the pathology of amyotrophic lateral sclerosis (ALS), which culminates in motor neuron loss due to axon retraction and muscle atrophy. The complex interactions in the pathogenesis of ALS between motor neurons, muscle cells and accompanying glia require an appropriate experimental model. Here, we have defined a co-culture model based on human myotubes innervated by neurons from embryonic rat spinal cord explants to investigate the pathology and treatment of ALS...
June 15, 2017: Journal of Molecular Neuroscience: MN
https://www.readbyqxmd.com/read/28615969/a-behavioral-survey-of-the-effects-of-kavalactones-on-caenorhabditis-elegans-neuromuscular-transmission
#12
Bwarenaba B Kautu, Juliana Phillips, Kellie Steele, M Shawn Mengarelli, Eric A Nord
Kava is a plant root extract that is widely consumed by Pacific Islanders. Kava contains a class of lactone compounds called kavalactones. The sedative and anxiolytic effects of kava are likely attributed to the efficacies of kavalactones on the nervous system. Although some studies have implicated the potencies of certain kavalactone species on γ-aminobutyric acid transmission, evidence supporting the action of kavalactones on the eukaryotic neuromuscular junction (NMJ) and acetylcholine (ACh) transmission is scant...
2017: Journal of Experimental Neuroscience
https://www.readbyqxmd.com/read/28612182/invaginating-presynaptic-terminals-in-neuromuscular-junctions-photoreceptor-terminals-and-other-synapses-of-animals
#13
REVIEW
Ronald S Petralia, Ya-Xian Wang, Mark P Mattson, Pamela J Yao
Typically, presynaptic terminals form a synapse directly on the surface of postsynaptic processes such as dendrite shafts and spines. However, some presynaptic terminals invaginate-entirely or partially-into postsynaptic processes. We survey these invaginating presynaptic terminals in all animals and describe several examples from the central nervous system, including giant fiber systems in invertebrates, and cup-shaped spines, electroreceptor synapses, and some specialized auditory and vestibular nerve terminals in vertebrates...
June 13, 2017: Neuromolecular Medicine
https://www.readbyqxmd.com/read/28608304/lambert-eaton-myasthenic-syndrome-lems-a-rare-autoimmune-presynaptic-disorder-often-associated-with-cancer
#14
REVIEW
Benedikt Schoser, Bruno Eymard, Joe Datt, Renato Mantegazza
Lambert-Eaton myasthenic syndrome (LEMS) is a rare autoimmune neuromuscular junction disorder that is related to the loss of functional P/Q-type voltage-gated calcium channels (VGCCs) on presynaptic nerve terminals. Up to 60% of cases occur as a paraneoplastic disorder (SCLC-LEMS), most commonly in association with small cell lung cancer. The remaining cases have an idiopathic non-tumor etiology but are associated with underlying autoimmune disease (NT-LEMS). Patients with LEMS invariably experience progressive proximal muscle weakness, often accompanied by general fatigue and autonomic symptoms...
June 12, 2017: Journal of Neurology
https://www.readbyqxmd.com/read/28607408/differences-in-pharmacodynamic-responses-to-rocuronium-in-normal-or-injured-orbicularis-oris-are-associated-with-expression-of-acetylcholine-receptor-subunits
#15
Yong Huang, Yian Xing, Hong Wang, Lianhua Chen, Shitong Li
Previous research has indicated that differences in sensitivities to muscle relaxants exist between facial nerve- and somatic nerve-innervated muscles. Here, we report that the 50% inhibitory concentration (IC50) values for rocuronium were significantly larger in the normal orbicularis oris than those in the gastrocnemius. Increased IC50 values and reduced twitch tension were observed after facial nerve injury. The normal orbicularis oris had a smaller muscle fiber cross-sectional area (CSA) and a larger ratio of endplate surface area (ESA) to muscle fiber CSA (ESA/CSA), but no difference was found in the density of nicotinic acetylcholine receptor (nAChR) subunits on endplates between normal orbicularis oris and gastrocnemius...
June 12, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28606041/mechanisms-of-action-resistance-and-toxicity-of-insecticides-targeting-gaba-receptors
#16
Steven Buckingham, Makoto Ihara, David B Sattelle, Kazuhiko Matsuda
Ionotropic γ-aminobutyric acid (GABA)-gated chloride channels belong to the cys-loop superfamily of ligand-gated ion channels (cys-loop LGICs) and mediate fast inhibitory synaptic transmission in the nervous system and at neuromuscular junctions of insects. Insect GABA-gated cation channels have also been described. The sequencing of insect genomes has enhanced our understanding of the subunits that make up GABA receptor gene families of many species, including a genetic model organism (Drosophila melanogaster) as well as pest, vector and beneficial (pollinator) species...
June 12, 2017: Current Medicinal Chemistry
https://www.readbyqxmd.com/read/28603484/evolutionary-divergence-of-the-c-terminal-domain-of-complexin-accounts-for-functional-disparities-between-vertebrate-and-invertebrate-complexins
#17
Rachel T Wragg, Daniel A Parisotto, Zhenlong Li, Mayu S Terakawa, David Snead, Ishani Basu, Harel Weinstein, David Eliezer, Jeremy S Dittman
Complexin is a critical presynaptic protein that regulates both spontaneous and calcium-triggered neurotransmitter release in all synapses. Although the SNARE-binding central helix of complexin is highly conserved and required for all known complexin functions, the remainder of the protein has profoundly diverged across the animal kingdom. Striking disparities in complexin inhibitory activity are observed between vertebrate and invertebrate complexins but little is known about the source of these differences or their relevance to the underlying mechanism of complexin regulation...
2017: Frontiers in Molecular Neuroscience
https://www.readbyqxmd.com/read/28599652/regulatory-t-cells-in-multiple-sclerosis-and-myasthenia-gravis
#18
REVIEW
K M Danikowski, S Jayaraman, B S Prabhakar
Multiple sclerosis (MS) is a chronic debilitating disease of the central nervous system primarily mediated by T lymphocytes with specificity to neuronal antigens in genetically susceptible individuals. On the other hand, myasthenia gravis (MG) primarily involves destruction of the neuromuscular junction by antibodies specific to the acetylcholine receptor. Both autoimmune diseases are thought to result from loss of self-tolerance, which allows for the development and function of autoreactive lymphocytes. Although the mechanisms underlying compromised self-tolerance in these and other autoimmune diseases have not been fully elucidated, one possibility is numerical, functional, and/or migratory deficits in T regulatory cells (Tregs)...
June 9, 2017: Journal of Neuroinflammation
https://www.readbyqxmd.com/read/28599246/ablation-of-il-17-expression-moderates-experimental-autoimmune-myasthenia-gravis-disease-severity
#19
Gabriela Aguilo-Seara, Yanchen Xie, Jarrod Sheehan, Linda L Kusner, Henry J Kaminski
An array of cytokines influences the pathogenesis of early onset myasthenia gravis (MG) and its animal model, experimental autoimmune myasthenia gravis (EAMG). Patients with MG, in particular those with more severe weakness, have elevations of the pro-inflammatory cytokine IL-17 in the blood. We assessed the role of IL-17A in autoimmunity by inducing EAMG in mice with knockout of IL-17 and found a reduction of EAMG severity, but not a complete ablation of disease. The IL-17(ko) mice had no evidence of weakness, low levels of acetylcholine receptor antibodies, and retention of acetylcholine receptor at the neuromuscular junction...
June 6, 2017: Cytokine
https://www.readbyqxmd.com/read/28589042/pyridostigmine-induced-prolonged-asystole-in-a-patient-with-myasthenia-gravis-successfully-treated-with-hyoscyamine
#20
Mohammad Saud Khan, Abhinav Tiwari, Zubair Khan, Himani Sharma, Mohammad Taleb, Jeffrey Hammersley
Reversible acetylcholinesterase inhibitors are used as first-line treatment for myasthenia gravis. They improve symptoms by increasing concentration of acetylcholine at the neuromuscular junction and stimulating nicotinic receptors. Serious bradyarrhythmias can occur from muscarinic stimulation in heart, which in rare cases may progress to asystole. These patients can initially be managed with hyoscyamine, a muscarinic antagonist. Persistence of bradyarrhythmias even after hyoscyamine treatment may warrant pacemaker placement...
2017: Case Reports in Cardiology
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