keyword
MENU ▼
Read by QxMD icon Read
search

Neuromuscular junction

keyword
https://www.readbyqxmd.com/read/28731862/muscle-neuromuscular-junction-and-peripheral-nerve-diseases
#1
Jean-Marc Léger
No abstract text is available yet for this article.
July 20, 2017: Current Opinion in Neurology
https://www.readbyqxmd.com/read/28729700/sphingomimetic-multiple-sclerosis-drug-fty720-activates-vesicular-synaptobrevin-and-augments-neuroendocrine-secretion
#2
Frederic D Darios, Jernej Jorgacevski, Ajda Flašker, Robert Zorec, Virginia García-Martinez, José Villanueva, Luis M Gutiérrez, Charlotte Leese, Manjot Bal, Elena Nosyreva, Ege T Kavalali, Bazbek Davletov
Neurotransmission and secretion of hormones involve a sequence of protein/lipid interactions with lipid turnover impacting on vesicle trafficking and ultimately fusion of secretory vesicles with the plasma membrane. We previously demonstrated that sphingosine, a sphingolipid metabolite, promotes formation of the SNARE complex required for membrane fusion and also increases the rate of exocytosis in isolated nerve terminals, neuromuscular junctions, neuroendocrine cells and in hippocampal neurons. Recently a fungi-derived sphingosine homologue, FTY720, has been approved for treatment of multiple sclerosis...
July 20, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28729214/milk-fat-globule-membrane-supplementation-with-voluntary-running-exercise-attenuates-age-related-motor-dysfunction-by-suppressing-neuromuscular-junction-abnormalities-in-mice
#3
Michiko Yano, Yoshihiko Minegishi, Satoshi Sugita, Noriyasu Ota
Age-related loss of skeletal muscle mass and function attenuates physical performance, and maintaining fine muscle innervation is known to play an important role in its prevention. We had previously shown that consumption of milk fat globule membrane (MFGM) with habitual exercise improves the muscle mass and motor function in humans and mice. Improvement of neuromuscular junction (NMJ) was suggested as one of the mechanisms underlying these effects. In this study, we evaluated the effect of MFGM intake combined with voluntary running (MFGM-VR) on morphological changes of NMJ and motor function in aging mice...
July 17, 2017: Experimental Gerontology
https://www.readbyqxmd.com/read/28724748/depressed-synaptic-transmission-and-reduced-vesicle-release-sites-in-huntington-s-disease-neuromuscular-junctions
#4
Ahmad Khedraki, Eric J Reed, Shannon H Romer, Qingbo Wang, William Romine, Mark M Rich, Robert J Talmadge, Andrew A Voss
Huntington's disease (HD) is a progressive and fatal degenerative disorder that results in debilitating cognitive and motor dysfunction. Most HD studies have focused on degeneration of the central nervous system. We previously discovered that skeletal muscle from transgenic R6/2 HD mice is hyperexcitable due to decreased chloride and potassium conductances. The progressive and early onset of these defects suggest a primary myopathy in HD. In this study, we examined the relationship between neuromuscular transmission and skeletal muscle hyperexcitability...
July 19, 2017: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/28721012/comparison-of-neurotoxic-potency-between-a-novel-chinbotulinumtoxina-with-onabotulinumtoxina-incobotulinumtoxina-and-lanbotulinumtoxina-in-rats
#5
Ya Feng, Wuchao Liu, Lizhen Pan, Cong Jiang, Chengxi Zhang, Yuxuan Lu, Zhiyu Nie, Lingjing Jin
Four botulinumtoxin type A (BoNT/A) products, onabotulinumtoxinA (A/Ona), incobotulinumtoxinA (A/Inco), lanbotulinumtoxinA (A/Lan) and chinbotulinumtoxinA (A/Chin), are applied in the present study, among which A/Chin is newly produced. We aimed to compare the neurotoxic potency of these toxins by the gauge of muscle strength reduction. Furthermore, potential molecular and cellular mechanisms were also explored. According to our data, muscle strengths in the four toxin groups were all significantly decreased after injection for 1 week...
2017: Drug Design, Development and Therapy
https://www.readbyqxmd.com/read/28718435/agrin-to-yap-in-cancer-and-neuromuscular-junctions
#6
Wen-Cheng Xiong, Lin Mei
Agrin is utilized by motor neurons to stimulate the LRP4-MuSK receptor in muscles for neuromuscular junction (NMJ) formation. Recent studies of cancer have identified novel functions of the low-density lipoprotein receptor-related protein 4-muscle-specific kinase (LRP4-MuSK) pathway. Agrin may act as a mechanotransduction signal in the extracellular matrix (ECM) to coordinate the cross-talk between the LRP4-MuSK pathway and integrin-focal adhesion pathway. Ensuing Yes-associated protein (YAP) activation promotes hepatocellular carcinoma (HCC)...
April 2017: Trends in Cancer
https://www.readbyqxmd.com/read/28715368/loading-a-calcium-dye-into-frog-nerve-endings-through-the-nerve-stump-calcium-transient-registration-in-the-frog-neuromuscular-junction
#7
Dmitry V Samigullin, Eduard F Khaziev, Nikita V Zhilyakov, Ellya A Bukharaeva, Eugeny E Nikolsky
One of the most feasible methods of measuring presynaptic calcium levels in presynaptic nerve terminals is optical recording. It is based on using calcium-sensitive fluorescent dyes that change their emission intensity or wavelength depending on the concentration of free calcium in the cell. There are several methods used to stain cells with calcium dyes. Most common are the processes of loading the dyes through a micropipette or pre-incubating with the acetoxymethyl ester forms of the dyes. However, these methods are not quite applicable to neuromuscular junctions (NMJs) due to methodological issues that arise...
July 8, 2017: Journal of Visualized Experiments: JoVE
https://www.readbyqxmd.com/read/28712002/mutations-in-gfpt1-related-congenital-myasthenic-syndromes-are-associated-with-synaptic-morphological-defects-and-underlie-a-tubular-aggregate-myopathy-with-synaptopathy
#8
Stéphanie Bauché, Geoffroy Vellieux, Damien Sternberg, Marie-Joséphine Fontenille, Elodie De Bruyckere, Claire-Sophie Davoine, Guy Brochier, Julien Messéant, Lucie Wolf, Michel Fardeau, Emmanuelle Lacène, Norma Romero, Jeanine Koenig, Emmanuel Fournier, Daniel Hantaï, Nathalie Streichenberger, Veronique Manel, Arnaud Lacour, Aleksandra Nadaj-Pakleza, Sylvie Sukno, Françoise Bouhour, Pascal Laforêt, Bertrand Fontaine, Laure Strochlic, Bruno Eymard, Frédéric Chevessier, Tanya Stojkovic, Sophie Nicole
Mutations in GFPT1 (glutamine-fructose-6-phosphate transaminase 1), a gene encoding an enzyme involved in glycosylation of ubiquitous proteins, cause a limb-girdle congenital myasthenic syndrome (LG-CMS) with tubular aggregates (TAs) characterized predominantly by affection of the proximal skeletal muscles and presence of highly organized and remodeled sarcoplasmic tubules in patients' muscle biopsies. We report here the first long-term clinical follow-up of 11 French individuals suffering from LG-CMS with TAs due to GFPT1 mutations, of which nine are new...
July 15, 2017: Journal of Neurology
https://www.readbyqxmd.com/read/28710284/the-neuronal-protein-neurexin-directly-interacts-with-the-scribble-pix-complex-to-stimulate-f-actin-assembly-for-synaptic-vesicle-clustering
#9
Menglong Rui, Jinjun Qian, Lijuan Liu, Yihan Cai, Huihui Lv, Junhai Han, Zhengping Jia, Wei Xie
Synaptic vesicles (SVs) form distinct pools at synaptic terminals, and this well-regulated separation is necessary for normal neuro-transmission. However, how SV cluster in particular synaptic compartments to maintain normal neurotransmitter release remains a mystery. The presynaptic protein Neurexin (NRX) plays a significant role in synaptic architecture and function, and some evidences suggest that NRX is associated with neurological disorders, including autism spectrum disorders. However, the role of NRX in SV clustering is unclear...
July 14, 2017: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/28701774/inhibition-of-%C3%AE-glucocerebrosidase-activity-preserves-motor-unit-integrity-in-a-mouse-model-of-amyotrophic-lateral-sclerosis
#10
Alexandre Henriques, Mylene Huebecker, Hélène Blasco, Céline Keime, Christian R Andres, Philippe Corcia, David A Priestman, Frances M Platt, Michael Spedding, Jean-Philippe Loeffler
Recent metabolomic reports connect dysregulation of glycosphingolipids, particularly ceramide and glucosylceramide, to neurodegeneration and to motor unit dismantling in amyotrophic lateral sclerosis at late disease stage. We report here altered levels of gangliosides in the cerebrospinal fluid of amyotrophic lateral sclerosis patients in early disease stage. Conduritol B epoxide is an inhibitor of acid beta-glucosidase, and lowers glucosylceramide degradation. Glucosylceramide is the precursor for all of the more complex glycosphingolipids...
July 12, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28691974/peroral-endoscopic-myotomy-in-children-with-achalasia-a-relatively-long-term-single-center-study
#11
Miao Shijian, Wu Jie, Lu Junping, Wang Yuhuan, Tang Zifei, Zhou Ying, Huang Zhiheng, Ying Huang, Zhou Pinghong
BACKGROUND: Achalasia is a disease caused by neuromuscular dysfunction in the esophagogastric junction, with a relatively low incidence among children. Peroral endoscopic myotomy (POEM) is a new endoscopic procedure for the treatment of achalasia, however it is rarely applied in children. OBJECTIVES: We aim to study the feasibility, safety, and effectiveness of POEM as a treatment for pediatric achalasia. METHODS: A total of 21 pediatric patients (aged from 11 months to 18 years) diagnosed with achalasia and treated with POEM from October of 2014 to October of 2016 in our hospital were included in our study...
July 6, 2017: Journal of Pediatric Gastroenterology and Nutrition
https://www.readbyqxmd.com/read/28690392/congenital-myasthenic-syndrome-phenotypic-variability-in-patients-harbouring-p-t159p-mutation-in-chrne-gene
#12
Anna Ardissone, Isabella Moroni, Pia Bernasconi, Raffaella Brugnoni
Congenital myasthenic syndromes (CMS) are rare and heterogeneous genetic diseases characterized by compromised neuromuscular transmission and clinical features of fatigable weakness; age at onset, presenting symptoms, distribution of weakness, and response to treatment differ depending on the underlying molecular defect. Mutations in one of the multiple genes, encoding proteins expressed at the neuromuscular junction, are currently known to be associated with subtypes of CMS. The most common CMS syndrome identified is associated with mutation in the CHRNE gene, causing principally muscle nicotinic acetylcholine receptor deficiency, that results in reduced receptor density on the postsynaptic membrane...
March 2017: Acta Myologica: Myopathies and Cardiomyopathies: Official Journal of the Mediterranean Society of Myology
https://www.readbyqxmd.com/read/28687442/increased-bace1-activity-inhibits-peripheral-nerve-regeneration-after-injury
#13
Carolyn Tallon, Edward Rockenstein, Eliezer Masliah, Mohamed H Farah
Axons of the peripheral nervous system possess the capacity to regenerate following injury. Previously, we showed that genetically knocking out Beta-Site APP-Cleaving Enzyme 1 (BACE1) leads to increased nerve regeneration. Two cellular components, macrophages and neurons, contribute to enhanced nerve regeneration in BACE1 knockout mice. Here, we utilized a transgenic mouse model that overexpresses BACE1 in its neurons to investigate whether neuronal BACE1 has an inverse effect on regeneration following nerve injury...
July 5, 2017: Neurobiology of Disease
https://www.readbyqxmd.com/read/28680299/the-scaffolding-protein-grb2-associated-binder-1-in-skeletal-muscles-and-terminal-schwann-cells-regulates-postnatal-neuromuscular-synapse-maturation
#14
So Young Park, So Young Jang, Yoon Kyoung Shin, Dong Keun Jung, Byeol A Yoon, Jong Kook Kim, Young Rae Jo, Hye Jeong Lee, Hwan Tae Park
The vertebrate neuromuscular junction (NMJ) is considered as a "tripartite synapse" consisting of a motor axon terminal, a muscle endplate, and terminal Schwann cells that envelope the motor axon terminal. The neuregulin 1 (NRG1)-ErbB2 signaling pathway plays an important role in the development of the NMJ. We previously showed that Grb2-associated binder 1 (Gab1), a scaffolding mediator of receptor tyrosine kinase signaling, is required for NRG1-induced peripheral nerve myelination. Here, we determined the role of Gab1 in the development of the NMJ using muscle-specific conditional Gab1 knockout mice...
June 2017: Experimental Neurobiology
https://www.readbyqxmd.com/read/28669545/retrograde-synaptic-inhibition-is-mediated-by-%C3%AE-neurexin-binding-to-the-%C3%AE-2%C3%AE-subunits-of-n-type-calcium-channels
#15
Xia-Jing Tong, Eduardo Javier López-Soto, Lei Li, Haowen Liu, Daniel Nedelcu, Diane Lipscombe, Zhitao Hu, Joshua M Kaplan
The synaptic adhesion molecules Neurexin and Neuroligin alter the development and function of synapses and are linked to autism in humans. In C. elegans, post-synaptic Neurexin (NRX-1) and pre-synaptic Neuroligin (NLG-1) mediate a retrograde synaptic signal that inhibits acetylcholine (ACh) release at neuromuscular junctions. Here, we show that the retrograde signal decreases ACh release by inhibiting the function of pre-synaptic UNC-2/CaV2 calcium channels. Post-synaptic NRX-1 binds to an auxiliary subunit of pre-synaptic UNC-2/CaV2 channels (UNC-36/α2δ), decreasing UNC-36 abundance at pre-synaptic elements...
July 19, 2017: Neuron
https://www.readbyqxmd.com/read/28666511/unilateral-external-ophthalmoplegia-arare-presentation-of-myasthenia-gravis
#16
Muhammad Saim Khan, Asad Habib, Imran Basit
Myasthenia gravis (MG) is a disease of autoimmunity with variable and diverse clinical presentations. The target tissue is neuromuscular junction of skeletal muscles, where efficient nerve impulse transmission is hampered leading to less effective muscle contraction. Patients of MG usually present with bilateral ptosis, diplopia and fatigability, which may or may not coexist with generalised weakness, dysphagia and dysarthria. A46-year male presented with unilateral ptosis and diplopia. Except for unilateral moderate ptosis and restriction of extraocular movements, ocular and systemic examination was normal...
November 2016: Journal of the College of Physicians and Surgeons—Pakistan: JCPSP
https://www.readbyqxmd.com/read/28662779/ocular-vestibular-evoked-myogenic-potential-in-patients-with-myasthenia-gravis-a-prospective-clinical-study
#17
Mohammad El-Sayed Mojahed, Elsaeid Mohamed Thabet, Mohamed Galal El-Khateeb, Ashraf Elsayed Morgan
OBJECTIVE: Myasthenia gravis (MG) is an archetypic disorder of neuromuscular junctions (NMJs) and autoantibody-mediated disease causing fatigable weakness of skeletal muscles with an ocular onset in up to 85%. The aim of this study was to detect extra ocular muscles (EOMs) abnormalities in MG patients using ocular vestibular evoked myogenic potential (oVEMP) n10 response. METHODS: The oVEMP was performed on 40 myasthenia gravis patients that were divided into three groups: newly diagnosed (10 patients), uncontrolled on treatment (15 patients) and controlled on treatment (15 patients) groups in addition to a control group of 10 subjects...
June 26, 2017: Auris, Nasus, Larynx
https://www.readbyqxmd.com/read/28654072/subcutaneous-neurotrophin-4-infusion-using-osmotic-pumps-or-direct-muscular-injection-enhances-aging-rat-laryngeal-muscles
#18
Richard D Andreatta, Joseph C Stemple, Tanya S Seward, Colleen A McMullen
Laryngeal dysfunction in the elderly is a major cause of disability, from voice disorders to dysphagia and loss of airway protective reflexes. Few, if any, therapies exist that target age-related laryngeal muscle dysfunction. Neurotrophins are involved in muscle innervation and differentiation of neuromuscular junctions (NMJs). It is thought that neurotrophins enhance neuromuscular transmission by increasing neurotransmitter release. The neuromuscular junctions (NMJs) become smaller and less abundant in aging rat laryngeal muscles, with evidence of functional denervation...
June 13, 2017: Journal of Visualized Experiments: JoVE
https://www.readbyqxmd.com/read/28650411/recording-fewer-than-20-potential-pairs-with-sfemg-may-suffice-for-the-diagnosis-of-myasthenia-gravis
#19
Alon Abraham, Ari Breiner, Carolina Barnett, Hans D Katzberg, Vera Bril
PURPOSE: Our aim in the current study was to determine the minimum number of SFEMG potential pairs required to confirm neuromuscular junction impairment and relate this number to disease severity. METHODS: Ninety-four patients with myasthenia gravis (MG) attending the neuromuscular clinic from February 2013 to November 2015 were included. The SFEMG sensitivity was determined for each number of recorded pairs up to 20. In addition, we compared clinical and electrophysiologic characteristics between patients with abnormality within the first 3, 4 to 7, and ≥8 recorded pairs to determine whether this number is associated with disease severity...
June 20, 2017: Journal of Clinical Neurophysiology: Official Publication of the American Electroencephalographic Society
https://www.readbyqxmd.com/read/28647557/kcc3-loss-of-function-contributes-to-andermann-syndrome-by-inducing-activity-dependent-neuromuscular-junction-defects
#20
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
keyword
keyword
13617
1
2
Fetch more papers »
Fetching more papers... Fetching...
Read by QxMD. Sign in or create an account to discover new knowledge that matter to you.
Remove bar
Read by QxMD icon Read
×

Search Tips

Use Boolean operators: AND/OR

diabetic AND foot
diabetes OR diabetic

Exclude a word using the 'minus' sign

Virchow -triad

Use Parentheses

water AND (cup OR glass)

Add an asterisk (*) at end of a word to include word stems

Neuro* will search for Neurology, Neuroscientist, Neurological, and so on

Use quotes to search for an exact phrase

"primary prevention of cancer"
(heart or cardiac or cardio*) AND arrest -"American Heart Association"