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Neuromuscular junction

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https://www.readbyqxmd.com/read/29674962/invaginating-structures-in-mammalian-synapses
#1
REVIEW
Ronald S Petralia, Ya-Xian Wang, Mark P Mattson, Pamela J Yao
Invaginating structures at chemical synapses in the mammalian nervous system exist in presynaptic axon terminals, postsynaptic spines or dendrites, and glial processes. These invaginating structures can be divided into three categories. The first category includes slender protrusions invaginating into axonal terminals, postsynaptic spines, or glial processes. Best known examples of this category are spinules extending from postsynaptic spines into presynaptic terminals in forebrain synapses. Another example of this category are protrusions from inhibitory presynaptic terminals invaginating into postsynaptic neuronal somas...
2018: Frontiers in Synaptic Neuroscience
https://www.readbyqxmd.com/read/29666602/myasthenia-gravis-induced-by-ipilimumab-in-a-patient-with-metastatic-melanoma
#2
Vera Montes, Sandra Sousa, Fernando Pita, Rui Guerreiro, Cátia Carmona
In daily clinical practice, there is a growing number of patients receiving new biological agents used in the treatment of malignancies. Ipilimumab is a fully humanized monoclonal antibody approved for patients with melanoma. It acts as an immune checkpoint inhibitor, binding and blocking cytotoxic T-lymphocyte antigen-4 in order to increase the antitumor immune response. There are several reports of autoimmune responses after its use. A 74-year-old man developed a mild rash and pruritus a few hours after the second infusion of ipilimumab and 24 h after the third dose of ipilimumab, he presented with shortness of breath, proximal limb muscle weakness, and diplopia...
2018: Frontiers in Neurology
https://www.readbyqxmd.com/read/29666144/sporadic-amyotrophic-lateral-sclerosis-sals-skeletal-muscle-response-to-cerebrospinal-fluid-from-sals-patients-in-a-rat-model
#3
Shruthi Shanmukha, Gayathri Narayanappa, Atchayaram Nalini, Phalguni Anand Alladi, Trichur R Raju
Skeletal muscle atrophy is the most prominent feature of amyotrophic lateral sclerosis (ALS), an adult-onset neurodegenerative disease of motor neurons. However, the contribution of skeletal muscle to disease progression remains elusive. Our previous studies have shown that intrathecal injection of cerebrospinal fluid from sporadic ALS patients (ALS-CSF) induces several degenerative changes in motor neurons and glia of neonatal rats. Here, we describe various pathologic events in the rat extensor digitorum longus muscle following intrathecal injection of ALS-CSF...
April 16, 2018: Disease Models & Mechanisms
https://www.readbyqxmd.com/read/29663639/a-novel-ecel1-mutation-expands-the-phenotype-of-distal-arthrogryposis-multiplex-congenita-type-5d-to-include-pretibial-vertical-skin-creases
#4
Eva-Lena Stattin, Josefin Johansson, Sanna Gudmundsson, Adam Ameur, Staffan Lundberg, Marie-Louise Bondeson, Maria Wilbe
Arthrogryposis multiplex congenita (AMC) is a heterogeneous disorder characterized by multiple joint contractures often in association with other congenital abnormalities. Pretibial linear vertical creases are a rare finding associated with arthrogryposis, and the etiology of the specific condition is unknown. We aimed to genetically and clinically characterize a boy from a consanguineous family, presenting with AMC and pretibial vertical linear creases on the shins. Whole exome sequencing and variant analysis revealed homozygous novel missense variants of ECEL1 (c...
April 16, 2018: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/29663403/smoke-induced-neuromuscular-junction-degeneration-precedes-the-fibre-type-shift-and-atrophy-in-copd
#5
Sophia Kapchinsky, Madhusudanarao Vuda, Kayla Miguez, Daren Elkrief, Angela R de Souza, Carolyn J Baglole, Sudhakar Aare, Norah J MacMillan, Jacinthe Baril, Paul Rozakis, Vita Sonjak, Charlotte Pion, Mylène Aubertin-Leheudre, Jose A Morais, R Thomas Jagoe, Jean Bourbeau, Tanja Taivassalo, Russell T Hepple
A neurological basis for the fast fibre shift and atrophy seen in limb muscle of patients with chronic obstructive pulmonary disease (COPD) has not been considered previously. The objective of our study was: (i) to determine if denervation contributes to fast fibre shift and muscle atrophy in COPD; and (ii) to assess using a preclinical smoking mouse model whether chronic tobacco smoke (TS) exposure could initiate denervation by causing neuromuscular junction (NMJ) degeneration. Vastus lateralis muscle biopsies were obtained from severe COPD patients (n = 10 with low fat free mass index [FFMI], 65 y; n = 15 normal FFMI, 65 y) and healthy age- and activity-matched non-smoker control subjects (CON; n = 11, 67 y), to evaluate morphological and transcriptional markers of denervation...
April 16, 2018: Journal of Physiology
https://www.readbyqxmd.com/read/29662761/inflammation-and-age-associated-skeletal-muscle-deterioration-sarcopaenia
#6
REVIEW
Jinyu Wang, Kwok-Sui Leung, Simon Kwoon-Ho Chow, Wing-Hoi Cheung
Ageing is accompanied by chronic inflammatory responses due to elevated circulatory inflammatory cytokine production. Several inflammatory cytokines have been shown to be responsible for a decrease in muscle mass. However, little is known about the possible relationship between inflammation and sarcopaenia. This review aims to summarise the existing evidence about inflammation and sarcopaenia. Sarcopaenia is defined as an age-related decrease of muscle mass and/or muscle strength; it is caused by multiple factors, such as skeletal muscle atrophy, neuromuscular junction degeneration, hormone imbalance, cytokine imbalance, protein synthesis and proteolysis...
July 2017: Journal of Orthopaedic Translation
https://www.readbyqxmd.com/read/29659046/polyethylene-glycol-treated-allografts-not-tissue-matched-nor-immunosuppressed-rapidly-repair-sciatic-nerve-gaps-maintain-neuromuscular-functions-and-restore-voluntary-behaviors-in-female-rats
#7
Michelle Mikesh, Cameron L Ghergherehchi, Sina Rahesh, Karthik Jagannath, Amir Ali, Dale R Sengelaub, Richard C Trevino, David M Jackson, Haley O Tucker, George D Bittner
Many publications report that ablations of segments of peripheral nerves produce the following unfortunate results: (1) Immediate loss of sensory signaling and motor control; (2) rapid Wallerian degeneration of severed distal axons within days; (3) muscle atrophy within weeks; (4) poor behavioral (functional) recovery after many months, if ever, by slowly-regenerating (∼1mm/d) axon outgrowths from surviving proximal nerve stumps; and (5) Nerve allografts to repair gap injuries are rejected, often even if tissue matched and immunosuppressed...
April 16, 2018: Journal of Neuroscience Research
https://www.readbyqxmd.com/read/29658266/enzyme-based-electrochemical-biosensor-for-therapeutic-drug-monitoring-of-anticancer-drug-irinotecan
#8
Maria Domenica Alvau, Stefano Tartaggia, Anna Meneghello, Bruno Casetta, Giammario Calia, Pier Andrea Serra, Federico Polo, Giuseppe Toffoli
Therapeutic drug monitoring (TDM) is the clinical practice of measuring pharmaceutical drug concentrations in patients' biofluids at designated intervals, thus allowing a close and timely control of their dosage. To date, TDM in oncology can only be performed by trained personnel in centralized laboratories and core facilities employing conventional analytical techniques (e.g. MS). CPT-11 is an antineoplastic drug that inhibits topoisomerase type I, causing cell death, and is widely used in the treatment of colorectal cancer...
April 16, 2018: Analytical Chemistry
https://www.readbyqxmd.com/read/29656576/repurposing-carbamazepine-for-the-treatment-of-amyotrophic-lateral-sclerosis-in-sod1-g93a-mouse-model
#9
Jing-Jing Zhang, Qin-Ming Zhou, Sheng Chen, Wei-Dong Le
AIMS: To investigate the effect and mechanisms of carbamazepine (CBZ) on the onset and progression of amyotrophic lateral sclerosis (ALS) in SOD1-G93A mouse model. METHODS: Starting from 64 days of age, SOD1-G93A mice were orally administered with CBZ at 200 mg/kg once daily until death. The disease onset and life span of SOD1-G93A mice were recorded. Motor neurons (MNs) in anterior horn of spinal cord were quantified by Nissl staining and SMI-32 immunostaining...
April 14, 2018: CNS Neuroscience & Therapeutics
https://www.readbyqxmd.com/read/29655456/lambert-eaton-myasthenic-syndrome
#10
REVIEW
Vita G Kesner, Shin J Oh, Mazen M Dimachkie, Richard J Barohn
Lambert-Eaton myasthenic syndrome is a paraneoplastic or primary autoimmune neuromuscular junction disorder characterized by proximal weakness, autonomic dysfunction and ariflexia. The characteristic symptoms are thought to be caused by antibodies generated against the P/Q-type voltage-gated calcium channels present on presynaptic nerve terminals and by diminished release of acetylcholine. More than half of Lambert-Eaton myasthenic syndrome cases are associated with small cell lung carcinoma. Diagnosis is confirmed by serologic testing and electrophysiologic studies...
May 2018: Neurologic Clinics
https://www.readbyqxmd.com/read/29655450/nature-and-action-of-antibodies-in-myasthenia-gravis
#11
REVIEW
Robert L Ruff, Robert P Lisak
This article discusses antibodies associated with immune-mediated myasthenia gravis and the pathologic action of these antibodies at the neuromuscular junctions of skeletal muscle. To explain how these antibodies act, we consider the physiology of neuromuscular transmission with emphasis on 4 features: the structure of the neuromuscular junction; the roles of postsynaptic acetylcholine receptors and voltage-gated Na+ channels and in converting the chemical signal from the nerve terminal into a propagated action potential on the muscle fiber that triggers muscle contraction; the safety factor for neuromuscular transmission; and how the safety factor is reduced in different forms of autoimmune myasthenia gravis...
May 2018: Neurologic Clinics
https://www.readbyqxmd.com/read/29655448/generalized-myasthenia-gravis-classification-clinical-presentation-natural-history-and-epidemiology
#12
REVIEW
Michael K Hehir, Nicholas J Silvestri
Myasthenia gravis (MG) is a rare disease, but the most common disorder of the neuromuscular junction. It is the prototypic autoimmune disease most commonly caused by antibodies to the acetylcholine receptor (AChR) leading to characteristic fatigable weakness of the ocular, bulbar, respiratory, axial, and limb muscles. The majority of patients with MG first present with ocular symptoms. Most patients with MG will experience at least 1 exacerbation of symptoms throughout the course of their illness. This article will cover the epidemiology, clinical presentation, classification, and natural history of MG...
May 2018: Neurologic Clinics
https://www.readbyqxmd.com/read/29655446/practical-anatomy-of-the-neuromuscular-junction-in-health-and-disease
#13
REVIEW
Hiroshi Nishimune, Kazuhiro Shigemoto
Neuromuscular junctions (NMJs) form between nerve terminals of spinal cord motor neurons and skeletal muscles, and perisynaptic Schwann cells and kranocytes cap NMJs. One muscle fiber has one NMJ, which is innervated by one motor nerve terminal. NMJs are excitatory synapses that use P/Q-type voltage-gated calcium channels to release the neurotransmitter acetylcholine. Acetylcholine receptors accumulate at the postsynaptic specialization called the end plate on the muscle fiber membrane, the sarcolemma. Proteins essential for the organization of end plates include agrin secreted from nerve terminals, Lrp4 and MuSK receptors for agrin, and Dok-7 and rapsyn cytosolic proteins in the muscle...
May 2018: Neurologic Clinics
https://www.readbyqxmd.com/read/29652962/-myasthenia-gravis-and-its-association-with-lymphoproliferative-disorders-a-case-series
#14
Gabriel Cea, Andrés Gallardo V, María Elena Cabrera C
Myasthenia gravis (MG) is a rare autoimmune disease of the neuromuscular junction. It is characterized by variable weakness and excessive fatigability of skeletal muscles. In the last few years, numerous reports have been published showing the association between autoimmune diseases, such as systemic erythematous lupus or rheumatoid arthritis, with lymphoid neoplasias. The association between MG and lymphoid neoplasia seems to be less frequent. To analyze this association we reviewed the MG patients in the Department of Neurology, Hospital Salvador of Santiago, Chile...
December 2017: Revista Médica de Chile
https://www.readbyqxmd.com/read/29652933/rhogapp190-a-potential-player-in-tbph-mediated-neurodegeneration-in-drosophila
#15
Simona Langellotti, Giulia Romano, Fabian Feiguin, Francisco Ernesto Baralle, Maurizio Romano
TDP-43 is an ubiquitous and highly conserved ribonucleoprotein involved in several cellular processes including pre-mRNA splicing, transcription, mRNA stability and transport. Notwithstanding the evidence of TDP-43 involvement in the pathogenesis of different neurodegenerative disorders (i.e. ALS and FTLD), the underlying mechanisms are still unclear. Given the high degree of functional similarity between the human and fly orthologs of TDP-43, Drosophila melanogaster is a simple and useful model to study the pathophysiological role of this protein in vivo...
2018: PloS One
https://www.readbyqxmd.com/read/29652477/targeted-nanoparticles-for-selective-marking-of-neuromuscular-junctions-and-ex-vivo-monitoring-of-endogenous-acetylcholine-hydrolysis
#16
Alsu R Mukhametshina, Svetlana V Fedorenko, Alexey M Petrov, Guzel F Zakyrjanova, Konstantin A Petrov, Leniz F Nurullin, Irek R Nizameev, Asiya R Mustafina, Oleg G Sinyashin
The present work for the first time introduces nanosensors for luminescent monitoring of acetylcholinesterase (AChE)-catalyzed hydrolysis of endogenous acetylcholine (AСh) released in neuromuscular junctions of isolated muscles. The sensing function results from the quenching of Tb(III)-centered luminescence due to proton-induced degradation of luminescent Tb(III) complexes doped into silica nanoparticles (SNs, 23 nm), when acetic acid is produced from the enzymatic hydrolysis of ACh. The targeting of the silica nanoparticles by α-bungarotoxin was used for selective staining of the synaptic space in the isolated muscles by the nanosensors...
April 13, 2018: ACS Applied Materials & Interfaces
https://www.readbyqxmd.com/read/29649746/degradation-of-alpha-synuclein-by-dendritic-cell-factor-1-delays-neurodegeneration-and-extends-lifespan-in-drosophila
#17
Shiqing Zhang, Ruili Feng, Yanhui Li, Linhua Gan, Fangfang Zhou, Shiquan Meng, Qian Li, Tieqiao Wen
Parkinson's disease (PD) is a common neurodegenerative disease associated with the progressive loss of dopaminergic neurons in the substantia nigra. Proteinaceous depositions of alpha-synuclein (α-syn) and its mutations, A30P and A53T, are one important characteristic of PD. However, little is known about their aggregation and degradation mechanisms. Dendritic cell factor 1 (DCF1) is a membrane protein that plays important roles in nerve development in mouse. In this study, we aimed to show that DCF1 overexpression in a PD Drosophila model significantly ameliorates impaired locomotor behavior in third instar larvae and normalizes neuromuscular junction growth...
March 13, 2018: Neurobiology of Aging
https://www.readbyqxmd.com/read/29645070/-isolated-girdle-weakness-expansion-of-the-phenotypic-spectrum-of-the-merrf-8344a-g-mutation-of-mitochondrial-dna
#18
A Erdocia-Goni, A Alonso-Jimenez, C Ramon-Carbajo, E Garcia-Arumi, P Casquero, E Gallardo, J Diaz-Manera
INTRODUCTION: The differential diagnosis of diseases that are accompanied by adult-onset girdle weakness is broad and includes motor neurone, neuromuscular junction or muscular diseases. The 8344A>G mutation of the MTTK gene of mitochondrial DNA usually presents with involvement of multiple organs associated (or not) with girdle weakness. To date no cases of isolated girdle weakness have been reported as the presenting symptom of this mutation. CASE REPORT: A 57-year-old male, with a four-year history of isolated clinical signs of progressive girdle weakness...
April 16, 2018: Revista de Neurologia
https://www.readbyqxmd.com/read/29643068/hydrogen-peroxide-is-a-neuronal-alarmin-that-triggers-specific-rnas-local-translation-of-annexin-a2-and-cytoskeletal-remodeling-in-schwann-cells
#19
Samuele Negro, Marco Stazi, Marta Marchioretto, Toma Tebaldi, Umberto Rodella, Elisa Duregotti, Volker Gerke, Alessandro Quattrone, Cesare Montecucco, Michela Rigoni, Gabriella Viero
Schwann cells are key players in neuro-regeneration: they sense alarm signals released by degenerating nerve terminals and differentiate toward a pro-regenerative phenotype, with phagocytosis of nerve debris and nerve guidance. At the murine neuromuscular junction, hydrogen peroxide (H2 O2 ) is a key signal of Schwann cells activation in response to a variety of nerve injuries. Here we report that Schwann cells exposed to low doses of H2 O2 rewire the expression of several RNAs at both transcriptional and translational levels...
April 11, 2018: RNA
https://www.readbyqxmd.com/read/29626993/jack-bean-urease-modulates-neurotransmitter-release-at-insect-neuromuscular-junctions
#20
Thiago Carrazoni, Christine Nguyen, Lucas F Maciel, Andres Delgado-Cañedo, Bryan A Stewart, Angela B Lange, Chariston A Dal Belo, Celia R Carlini, Ian Orchard
BACKGROUND: Plants have developed a vast range of mechanisms to compete with phytophagous insects, including entomotoxic proteins such as ureases. The legume Canavalia ensiformis produces several urease isoforms, of which the more abundant is called Jack Bean Urease (JBU). Previews work has demonstrated the potential insecticidal effects of JBU, by mechanisms so far not entirely elucidated. In this work, we investigated the mechanisms involved in the JBU-induced activity upon neurotransmitter release on insect neuromuscular junctions...
April 2018: Pesticide Biochemistry and Physiology
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