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Neuromuscular junction proteines

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https://www.readbyqxmd.com/read/29158500/use-dependent-potentiation-of-voltage-gated-calcium-channels-rescues-neurotransmission-in-nerve-terminals-intoxicated-by-botulinum-neurotoxin-serotype-a
#1
Phillip H Beske, Katie M Hoffman, James B Machamer, Margaret R Eisen, Patrick M McNutt
Botulinum neurotoxins (BoNTs) are highly potent toxins that cleave neuronal SNARE proteins required for neurotransmission, causing flaccid paralysis and death by asphyxiation. Currently, there are no clinical treatments to delay or reverse BoNT-induced blockade of neuromuscular transmission. While aminopyridines have demonstrated varying efficacy in transiently reducing paralysis following BoNT poisoning, the precise mechanisms by which aminopyridines symptomatically treat botulism are not understood. Here we found that activity-dependent potentiation of presynaptic voltage-gated calcium channels (VGCCs) underlies 3,4-diaminopyridine (3,4-DAP)-mediated rescue of neurotransmission in central nervous system synapses and mouse diaphragm neuromuscular junctions fully intoxicated by BoNT serotype A...
November 20, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29130196/super-resolution-single-molecule-fish-at-the-drosophila-neuromuscular-junction
#2
Joshua S Titlow, Lu Yang, Richard M Parton, Ana Palanca, Ilan Davis
The lack of an effective, simple, and highly sensitive protocol for fluorescent in situ hybridization (FISH) at the Drosophila larval neuromuscular junction (NMJ) has hampered the study of mRNA biology. Here, we describe our modified single molecule FISH (smFISH) methods that work well in whole mount Drosophila NMJ preparations to quantify primary transcription and count individual cytoplasmic mRNA molecules in specimens while maintaining ultrastructural preservation. The smFISH method is suitable for high-throughput sample processing and 3D image acquisition using any conventional microscopy imaging modality and is compatible with the use of antibody colabeling and transgenic fluorescent protein tags in axons, glia, synapses, and muscle cells...
2018: Methods in Molecular Biology
https://www.readbyqxmd.com/read/29125188/the-mouse-passive-transfer-model-of-musk-myasthenia-gravis-disrupted-musk-signaling-causes-synapse-failure
#3
REVIEW
Nazanin Ghazanfari, Sofie Trajanovska, Marco Morsch, Simon X Liang, Stephen W Reddel, William D Phillips
While the majority of myasthenia gravis patients express antibodies targeting the acetylcholine receptor, the second most common cohort instead displays autoantibodies against muscle-specific kinase (MuSK). MuSK is a transmembrane tyrosine kinase found in the postsynaptic membrane of the neuromuscular junction. During development, MuSK serves as a signaling hub, coordinating the alignment of the pre- and postsynaptic components of the synapse. Adult mice that received repeated daily injections of IgG from anti-MuSK(+) myasthenia gravis patients developed muscle weakness, associated with neuromuscular transmission failure...
November 10, 2017: Annals of the New York Academy of Sciences
https://www.readbyqxmd.com/read/29118959/congenital-myasthenic-syndrome-due-to-dok7-mutations-in-a-family-from-chile
#4
Jorge A Bevilacqua, Marian Lara, Jorge Díaz, Mario Campero, Jessica Vázquez, Ricardo A Maselli
Congenital myasthenic syndromes (CMS) are neuromuscular transmission disorders caused by mutations in genes encoding neuromuscular junction proteins. A 61-year-old female and her older sister showed bilateral ptosis, facial and proximal limb weakness, and scoliosis since childhood. Another female sibling had milder signs, while other family members were asymptomatic. Facial nerve repetitive stimulation in the proband showed decrement of muscle responses. Single fiber EMG revealed increased jitter and blocking...
June 27, 2017: European Journal of Translational Myology
https://www.readbyqxmd.com/read/29118957/morphological-peculiarities-of-neuromuscular-junctions-among-different-fiber-types-effect-of-exercise
#5
Teet Seene, Maria Umnova, Priit Kaasik
The aim of our research was to examine whether there are differences in the morphology of neuromuscular junctions of different types of muscle fibers in rodents, and after their adaptation to six weeks endurance exercise training. After 5-day acclimation, Wistar rats were subjected to run with the speed 35 m/min during 6 week, 5 days per week and the training volume reached 60 min per day. Muscle samples for ultrastructural studies were fixed, dehydrated and embedded in Epon-812. Ultra-thin sections were cut from longitudinally and transversely oriented blocs, using 4 blocks from each animal...
June 27, 2017: European Journal of Translational Myology
https://www.readbyqxmd.com/read/29114039/neuronal-activity-drives-fmrp-and-hspg-dependent-matrix-metalloproteinase-function-required-for-rapid-synaptogenesis
#6
Mary L Dear, Jarrod Shilts, Kendal Broadie
Matrix metalloproteinase (MMP) functions modulate synapse formation and activity-dependent plasticity. Aberrant MMP activity is implicated in fragile X syndrome (FXS), a disease caused by the loss of the RNA-binding protein FMRP and characterized by neurological dysfunction and intellectual disability. Gene expression studies in Drosophila suggest that Mmps cooperate with the heparan sulfate proteoglycan (HSPG) glypican co-receptor Dally-like protein (Dlp) to restrict trans-synaptic Wnt signaling and that synaptogenic defects in the fly model of FXS are alleviated by either inhibition of Mmp or genetic reduction of Dlp...
November 7, 2017: Science Signaling
https://www.readbyqxmd.com/read/29107812/proteolytic-maturation-of-drosophila-neuroligin-3-by-tumor-necrosis-factor-%C3%AE-converting-enzyme-in-the-nervous-system
#7
Jun Wu, Nana Tao, Yao Tian, Guanglin Xing, Huihui Lv, Junhai Han, Chengqi Lin, Wei Xie
BACKGROUND: The functions of autism-associated Neuroligins (Nlgs) are modulated by their post-translational modifications, such as proteolytic cleavage. A previous study has shown that there are different endogenous forms of DNlg3 in Drosophila, indicating it may undergo proteolytic processing. However, the molecular mechanism underlying DNlg3 proteolytic processing is unknown. Here, we report a novel proteolytic mechanism that is essential for DNlg3 maturation and function in the nervous system...
October 28, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/29105522/orb2-as-modulator-of-brat-and-their-role-at-the-neuromuscular-junction
#8
Elena Santana, Sergio Casas-Tintó
How synapses are built and dismantled is a central question in neurobiology. A wide range of proteins and processes from gene transcription to protein degradation are involved. Orb2 regulates mRNA translation depending on its monomeric or oligomeric state to modulate nervous system development and memory. Orb2 is expressed in Drosophila larval brain and neuromuscular junction (NMJ), Orb2 knockdown causes a reduction of synapse number and defects in neuronal morphology. Brain tumor (Brat) is an Orb2 target; it is expressed in larval brain related with cell growth and proliferation...
November 6, 2017: Journal of Neurogenetics
https://www.readbyqxmd.com/read/29104532/specific-physical-exercise-improves-energetic-metabolism-in-the-skeletal-muscle-of-amyotrophic-lateral-sclerosis-mice
#9
Céline Desseille, Séverine Deforges, Olivier Biondi, Léo Houdebine, Domenico D'amico, Antonin Lamazière, Cédric Caradeuc, Gildas Bertho, Gaëlle Bruneteau, Laure Weill, Jean Bastin, Fatima Djouadi, François Salachas, Philippe Lopes, Christophe Chanoine, Charbel Massaad, Frédéric Charbonnier
Amyotrophic Lateral Sclerosis is an adult-onset neurodegenerative disease characterized by the specific loss of motor neurons, leading to muscle paralysis and death. Although the cellular mechanisms underlying amyotrophic lateral sclerosis (ALS)-induced toxicity for motor neurons remain poorly understood, growing evidence suggest a defective energetic metabolism in skeletal muscles participating in ALS-induced motor neuron death ultimately destabilizing neuromuscular junctions. In the present study, we report that a specific exercise paradigm, based on a high intensity and amplitude swimming exercise, significantly improves glucose metabolism in ALS mice...
2017: Frontiers in Molecular Neuroscience
https://www.readbyqxmd.com/read/29104116/putative-roles-of-soluble-trophic-factors-in-facial-nerve-regeneration-target-reinnervation-and-recovery-of-vibrissal-whisking
#10
REVIEW
Habib Bendella, Svenja Rink, Maria Grosheva, Levent Sarikcioglu, Tessa Gordon, Doychin N Angelov
It is well-known that, after nerve transection and surgical repair, misdirected regrowth of regenerating motor axons may occur in three ways. The first way is that the axons enter into endoneurial tubes that they did not previously occupy, regenerate through incorrect fascicles and reinnervate muscles that they did not formerly supply. Consequently the activation of these muscles results in inappropriate movements. The second way is that, in contrast with the precise target-directed pathfinding by elongating motor nerves during embryonic development, several axons rather than a single axon grow out from each transected nerve fiber...
November 8, 2017: Experimental Neurology
https://www.readbyqxmd.com/read/29079805/genetic-interaction-of-disc1-and-neurexin-in-the-development-of-fruit-fly-glutamatergic-synapses
#11
Himani Pandey, Katia Bourahmoune, Takato Honda, Ken Honjo, Kazuki Kurita, Tomohito Sato, Akira Sawa, Katsuo Furukubo-Tokunaga
Originally identified at the breakpoint of a (1;11)(q42.1; q14.3) chromosomal translocation in a Scottish family with a wide range of mental disorders, the DISC1 gene has been a focus of intensive investigations as an entry point to study the molecular mechanisms of diverse mental dysfunctions. Perturbations of the DISC1 functions lead to behavioral changes in animal models, which are relevant to psychiatric conditions in patients. In this work, we have expressed the human DISC1 gene in the fruit fly (Drosophila melanogaster) and performed a genetic screening for the mutations of psychiatric risk genes that cause modifications of DISC1 synaptic phenotypes at the neuromuscular junction...
October 27, 2017: NPJ Schizophrenia
https://www.readbyqxmd.com/read/29078798/proteomic-profiling-of-neuronal-mitochondria-reveals-modulators-of-synaptic-architecture
#12
Laura C Graham, Samantha L Eaton, Paula J Brunton, Abdelmadjid Atrih, Colin Smith, Douglas J Lamont, Thomas H Gillingwater, Giuseppa Pennetta, Paul Skehel, Thomas M Wishart
BACKGROUND: Neurons are highly polarized cells consisting of three distinct functional domains: the cell body (and associated dendrites), the axon and the synapse. Previously, it was believed that the clinical phenotypes of neurodegenerative diseases were caused by the loss of entire neurons, however it has recently become apparent that these neuronal sub-compartments can degenerate independently, with synapses being particularly vulnerable to a broad range of stimuli. Whilst the properties governing the differential degenerative mechanisms remain unknown, mitochondria consistently appear in the literature, suggesting these somewhat promiscuous organelles may play a role in affecting synaptic stability...
October 27, 2017: Molecular Neurodegeneration
https://www.readbyqxmd.com/read/29074576/a-pre-synaptic-function-of-shank-protein-in-drosophila
#13
Song Wu, Guangming Gan, Zhiping Zhang, Jie Sun, Qifu Wang, Zhongbao Gao, Meixiang Li, Shan Jin, Juan Huang, Ulrich Thomas, Yong-Hui Jiang, Yan Li, Rui Tian, Yong Q Zhang
Human genetic studies support that loss of function mutations in the [highlight]SH[/highlight]3 domain and [highlight]ank[/highlight]yrin repeat containing family proteins (SHANK1-3), the large synaptic scaffolding proteins enriched at the postsynaptic density of excitatory synapses, are causative for autism spectrum disorder (ASD) and other neuropsychiatric disorders in humans. To better understand the in vivo functions of Shank and facilitate dissection of neuropathology associated with SHANK mutations in human, we generated multiple mutations in the Shank gene, the only member of the SHANK family in Drosophila melanogaster Both male and female Shank null mutants were fully viable and fertile with no apparent morphological or developmental defects...
October 26, 2017: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/29074396/translocation-and-dissemination-of-botulinum-neurotoxin-from-the-intestinal-tract
#14
Yukako Fujinaga, Michel R Popoff
Botulinum neurotoxins (BoNTs) are potent toxins which induce flaccid paralysis by inhibiting the release of acetylcholine at the neuromuscular junctions. They associate with non-toxic proteins (ANTPs or NAPs) to form complexes of various sizes which are resistant to acidic pH and protease degradation. BoNT trafficking from the digestive tract to the target neurons is still a matter of debate. BoNTs use different strategies to pass through the intestinal barrier including passage of BoNT complexes containing hemagglutinins (HAs) via M cells, HA-dependent perturbation of E-cadherin intercellular junctions between enterocytes and paracellular passage of BoNT complexes, and transcytosis of BoNT free of NAPs through certain intestinal epithelial cells...
October 23, 2017: Toxicon: Official Journal of the International Society on Toxinology
https://www.readbyqxmd.com/read/29068559/neuromuscular-synapse-electrophysiology-in-myasthenia-gravis-animal-models
#15
REVIEW
Jaap J Plomp, Maartje G M Huijbers, Jan J G M Verschuuren
The neuromuscular junction (NMJ) forms the synaptic connection between a motor neuron and a skeletal muscle fiber. In order to achieve a sustained muscle contraction, this synapse has to reliably transmit motor neuronal action potentials onto the muscle fiber. To guarantee successful transmission even during intense activation of the NMJ, a safety factor of neuromuscular transmission exists. In the neuromuscular disorder myasthenia gravis (MG), autoantibodies are directed against acetylcholine receptors or, in the rarer variants, against other postsynaptic NMJ proteins...
October 25, 2017: Annals of the New York Academy of Sciences
https://www.readbyqxmd.com/read/29068540/the-molecular-cross-talk-of-the-dystrophin-glycoprotein-complex
#16
REVIEW
Marta Gawor, Tomasz J Prószyński
The proper function of skeletal muscles relies on their ability to process signals derived from motor neurons, transmit stimuli along the muscle fibers, contract, and regenerate efficiently after injury. The dystrophin-glycoprotein complex (DGC; also called the dystrophin-associated protein complex) plays a central role in all of these processes. It acts as a transmembrane platform that anchors the extracellular matrix (ECM) to the intracellular cytoskeleton and makes muscle fibers more resistant to injury...
October 25, 2017: Annals of the New York Academy of Sciences
https://www.readbyqxmd.com/read/29067656/imaging-analysis-of-the-neuromuscular-junction-in-dystrophic-muscle
#17
Stephen J P Pratt, Shama R Iyer, Sameer B Shah, Richard M Lovering
Duchenne muscular dystrophy (DMD), caused by the absence of the protein dystrophin, is characterized as a neuromuscular disease in which muscle weakness, increased susceptibility to muscle injury, and inadequate repair appear to underlie the pathology. Considerable attention has been dedicated to studying muscle fiber damage, but there is little information to determine if damage from contraction-induced injury also occurs at or near the nerve terminal axon. Interestingly, both human patients and the mouse model for DMD (the mdx mouse) present fragmented neuromuscular junction (NMJ) morphology...
2018: Methods in Molecular Biology
https://www.readbyqxmd.com/read/29066856/modelling-acrylamide-acute-neurotoxicity-in-zebrafish-larvae
#18
Eva Prats, Cristian Gómez-Canela, Shani Ben-Lulu, Tamar Ziv, Francesc Padrós, Daniel Tornero, Natàlia Garcia-Reyero, Romà Tauler, Arie Admon, Demetrio Raldúa
Acrylamide (ACR), a type-2 alkene, may lead to a synaptopathy characterized by ataxia, skeletal muscles weakness and numbness of the extremities in exposed human and laboratory animals. Currently, only the mildly affected patients undergo complete recovery, and identification of new molecules with therapeutic bioactivity against ACR acute neurotoxicity is urgently needed. Here, we have generated a zebrafish model for ACR neurotoxicity by exposing 5 days post-fertilization zebrafish larvae to 1 mM ACR for 3 days...
October 24, 2017: Scientific Reports
https://www.readbyqxmd.com/read/29065712/comparison-of-whole-body-sod1-knockout-with-muscle-specific-sod1-knockout-mice-reveals-a-role-for-nerve-redox-signaling-in-regulation-of-degenerative-pathways-in-skeletal-muscle
#19
Giorgos Sakellariou, Brian McDonagh, Helen Porter, Ifigeneia Giakoumaki, Kate Earl, Gareth Nye, Aphrodite Vasilaki, Susan Brooks, Arlan Richardson, Holly Van Remmen, Anne McArdle, Malcolm Joseph Jackson
AIMS: Lack of CuZnSOD in homozygous knockout mice (Sod1-/-) leads to accelerated age-related muscle loss and weakness, but specific deletion of CuZnSOD in skeletal muscle(mSod1KO mice) or neurons (nSod1KO mice) resulted in only mild muscle functional deficits and failed to recapitulate the loss of mass and function observed in Sod1-/- mice. To dissect any underlying cross-talk between motor neurons and skeletal muscle in the degeneration in Sod1-/- mice, we characterized neuromuscular changes in the Sod1-/- model compared with mSod1KO mice and examined degenerative molecular mechanisms and pathways in peripheral nerve and skeletal muscle...
October 25, 2017: Antioxidants & Redox Signaling
https://www.readbyqxmd.com/read/29061639/neuronal-lrp4-regulates-synapse-formation-in-the-developing-cns
#20
Andromachi Karakatsani, Nicolas Marichal, Severino Urban, Georgios Kalamakis, Alexander Ghanem, Anna Schick, Yina Zhang, Karl-Klaus Conzelmann, Markus A Rüegg, Benedikt Berninger, Carmen Ruiz de Almodovar, Sergio Gascón, Stephan Kröger
The low-density lipoprotein receptor-related protein 4 (LRP4) is critical in muscle fibers for the establishment of the neuromuscular junction. Here, we show that LRP4 is also expressed by embryonic cortical and hippocampal neurons, and that downregulation of LRP4 in these neurons reduced density of synapses and number of primary dendrites. Accordingly, overexpression of LRP4 in cultured neurons had the opposite effect inducing more but shorter primary dendrites with an increased number of spines. Transsynaptic tracing mediated by rabies virus revealed a reduced number of neurons presynaptic to cortical neurons in which LRP4 was knocked down...
October 23, 2017: Development
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