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Motor neuron

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https://www.readbyqxmd.com/read/28214917/mtss1-promotes-maturation-and-maintenance-of-cerebellar-neurons-via-splice-variant-specific-effects
#1
Thorsten Sistig, Fanziska Lang, Sebastian Wrobel, Stephan L Baader, Karl Schilling, Britta Eiberger
Efficient coupling of the actin cytoskeleton to the cell membrane is crucial for histogenesis and maintenance of the nervous system. At this critical interface, BAR (Bin-Amphiphysin-Rvs) proteins regulate membrane bending, shown to be instrumental for mobility and morphogenesis of individual cells. Yet, the systemic significance of these proteins remains largely unexplored. Here, we probe the role of a prominent member of this protein family, the inverse-BAR protein Mtss1, for the development and function of a paradigmatic neuronal circuit, the cerebellar cortex...
February 18, 2017: Brain Structure & Function
https://www.readbyqxmd.com/read/28214579/motor-training-and-the-combination-of-action-observation-and-peripheral-nerve-stimulation-reciprocally-interfere-with-the-plastic-changes-induced-in-primary-motor-cortex-excitability
#2
Ambra Bisio, Laura Avanzino, Monica Biggio, Piero Ruggeri, Marco Bove
AO-PNS is a stimulation protocol combining action observation (AO) and peripheral nerve stimulation (PNS) to induce plasticity in the primary motor cortex (M1) (increased excitability). Another method to increase M1 excitability is motor training. The combination of two protocols, which individually induce long-term potentiation (LTP)-like plasticity in overlapping neural circuits, results in a transitory occlusion or reverse of this phenomenon. This study aimed to understand the neurophysiological mechanisms underlying AO-PNS by testing whether AO-PNS and motor training induced LTP-like plasticity in, at least partially, overlapping neural networks...
February 15, 2017: Neuroscience
https://www.readbyqxmd.com/read/28214532/smn1-functions-as-a-novel-inhibitor-for-traf6-mediated-nf-%C3%AE%C2%BAb-signaling
#3
Eun Kyung Kim, Eui-Ju Choi
Survival motor neuron (SMN) is a 38-kDa protein, whose deficiency in humans develops spinal muscular atrophy (SMA), an autosomal recessive neurodegenerative disease with muscular atrophy due to motor neuron death in the spinal cord. We now report that SMN prevents the activation of TRAF6 and IκB kinase (IKK) and thereby negatively regulates the NF-κB signaling processes. SMN physically interacted with TRAF6 and with each component of the IKK complex, IKK-α, IKK-β, and IKK-γ in BV2 microglia cells. Moreover, SMN1 inhibited the E3 ubiquitin ligase activity of TRAF6 as well as the kinase activity of IKK...
February 15, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/28213922/expression-of-kv3-1b-potassium-channel-is-widespread-in-macaque-motor-cortex-pyramidal-cells-a-histological-comparison-between-rat-and-macaque
#4
David Soares, Isabelle Goldrick, Roger N Lemon, Alexander Kraskov, Linda Greensmith, Bernadett Kalmar
There are substantial differences across species in the organisation and function of the motor pathways. These differences extend to basic electrophysiological properties. Thus, in rat motor cortex, pyramidal cells have long duration action potentials, while in the macaque, some pyramidal neurons exhibit short duration 'thin' spikes. These differences may be related to the expression of the fast potassium channel Kv3.1b, which in rat interneurons is associated with generation of thin spikes. Rat pyramidal cells typically lack these channels, while there are reports that they are present in macaque pyramids...
February 18, 2017: Journal of Comparative Neurology
https://www.readbyqxmd.com/read/28213645/transcranial-direct-current-stimulation-modulates-neuronal-networks-in-attention-deficit-hyperactivity-disorder
#5
Anna Sotnikova, Cornelia Soff, Enzo Tagliazucchi, Katja Becker, Michael Siniatchkin
Anodal transcranial direct current stimulation (tDCS) of the prefrontal cortex has been repeatedly shown to improve working memory (WM). Since patients with attention deficit hyperactivity disorder (ADHD) are characterized by both underactivation of the prefrontal cortex and deficits in WM, the modulation of prefrontal activity with tDCS in ADHD patients may increase their WM performance as well as improve the activation and connectivity of the WM network. In the present study, this hypothesis was tested using a double-blind sham-controlled experimental design...
February 17, 2017: Brain Topography
https://www.readbyqxmd.com/read/28213588/a-differential-autophagy-dependent-response-to-dna-double-strand-brakes-in-bone-marrow-mesenchymal-stem-cells-from-sporadic-als-patients
#6
Shane Wald-Altman, Edward Pichinuk, Or Kakhlon, Miguel Weil
Amyotrophic Lateral Sclerosis (ALS) is an incurable motor neurodegenerative disease caused by a diversity of genetic and environmental factors leading to neuromuscular degeneration and pathophysiological implications in non-neural systems. Our previous work showed abnormal transcriptional expression levels of biomarker genes in non-neuronal cell samples from ALS patients. The same genes proved to be differentially expressed in brain, spinal cord and muscle of the SOD1(G93A) ALS mouse model. These observations support the pathophysiological relevance of the ALS biomarkers discovered in human mesenchymal stem cells (hMSC) isolated from bone marrow samples of ALS patients (ALS-hMSC)...
February 16, 2017: Disease Models & Mechanisms
https://www.readbyqxmd.com/read/28213152/central-neuronal-motor-behaviour-in-skilled-and-less-skilled-novices-approaching-sports-specific-movement-techniques
#7
Tobias Vogt, Kouki Kato, Stefan Schneider, Stefan Türk, Kazuyuki Kanosue
Research on motor behavioural processes preceding voluntary movements often refers to analysing the readiness potential (RP). For this, decades of studies used laboratory setups with controlled sports-related actions. Further, recent applied approaches focus on athlete-non-athlete comparisons, omitting possible effects of training history on RP. However, RP preceding real sport-specific movements in accordance to skill acquisition remains to be elucidated. Therefore, after familiarization 16 right-handed males with no experience in archery volunteered to perform repeated sports-specific movements, i...
February 14, 2017: Human Movement Science
https://www.readbyqxmd.com/read/28212563/progression-of-motor-deficits-in-glioma-bearing-mice-impact-of-cnf1-therapy-at-symptomatic-stages
#8
Eleonora Vannini, Federica Maltese, Francesco Olimpico, Alessia Fabbri, Mario Costa, Matteo Caleo, Laura Baroncelli
Glioblastoma (GBM) is the most aggressive type of brain tumor. In this context, animal models represent excellent tools for the early detection and longitudinal mapping of neuronal dysfunction, that are critical in the preclinical validation of new therapeutic strategies. In a mouse glioma model, we developed sensitive behavioral readouts that allow early recognizing and following neurological symptoms. We injected GL261 cells into the primary motor cortex of syngenic mice and we used a battery of behavioral tests to longitudinally monitor the dysfunction induced by tumor growth...
February 15, 2017: Oncotarget
https://www.readbyqxmd.com/read/28212525/ablation-of-ferroptosis-regulator-glutathione-peroxidase-4-in-forebrain-neurons-promotes-cognitive-impairment-and-neurodegeneration
#9
William Sealy Hambright, Rene Solano Fonseca, Liuji Chen, Ren Na, Qitao Ran
Synaptic loss and neuron death are the underlying cause of neurodegenerative diseases such as Alzheimer's disease (AD); however, the modalities of cell death in those diseases remain unclear. Ferroptosis, a newly identified oxidative cell death mechanism triggered by massive lipid peroxidation, is implicated in the degeneration of neurons populations such as spinal motor neurons and midbrain neurons. Here, we investigated whether neurons in forebrain regions (cerebral cortex and hippocampus) that are severely afflicted in AD patients might be vulnerable to ferroptosis...
February 1, 2017: Redox Biology
https://www.readbyqxmd.com/read/28212264/correlated-emg-oscillations-between-antagonists-during-cocontraction-in-men
#10
Yasuhide Yoshitake, Hiroaki Kanehisa, Minoru Shinohara
PURPOSE: The purpose of this study was to determine the modulation of common low-frequency oscillations in pools of motor units across antagonistic muscles because of the difference in the activation level of pools of spinal motor neurons and the presence of neuromuscular fatigue during intended cocontraction. METHODS: Ten healthy young men (21.8 ± 1.5 yr) performed intended steady cocontractions of elbow flexors and extensors at maximal and a submaximal (10% of maximal EMG) effort...
March 2017: Medicine and Science in Sports and Exercise
https://www.readbyqxmd.com/read/28211874/datf4-regulation-of-mitochondrial-folate-mediated-one-carbon-metabolism-is-neuroprotective
#11
Ivana Celardo, Susann Lehmann, Ana C Costa, Samantha Hy Loh, L Miguel Martins
Neurons rely on mitochondria as their preferred source of energy. Mutations in PINK1 and PARKIN cause neuronal death in early-onset Parkinson's disease (PD), thought to be due to mitochondrial dysfunction. In Drosophila pink1 and parkin mutants, mitochondrial defects lead to the compensatory upregulation of the mitochondrial one-carbon cycle metabolism genes by an unknown mechanism. Here we uncover that this branch is triggered by the activating transcription factor 4 (ATF4). We show that ATF4 regulates the expression of one-carbon metabolism genes SHMT2 and NMDMC as a protective response to mitochondrial toxicity...
February 17, 2017: Cell Death and Differentiation
https://www.readbyqxmd.com/read/28211850/subthalamic-neurons-encode-both-single-and-multi-limb-movements-in-parkinson-s-disease-patients
#12
Ariel Tankus, Ido Strauss, Tanya Gurevich, Anat Mirelman, Nir Giladi, Itzhak Fried, Jeffrey M Hausdorff
The subthalamic nucleus (STN) is the main target for neurosurgical treatment of motor signs of Parkinson's disease (PD). Despite the therapeutic effect on both upper and lower extremities, its role in motor control and coordination and its changes in Parkinson's disease are not fully clear. We intraoperatively recorded single unit activity in ten patients with PD who performed repetitive feet or hand movements while undergoing implantation of a deep brain stimulator. We found both distinct and overlapping representations of upper and lower extremity movement kinematics in subthalamic units and observed evidence for re-routing to a multi-limb representation that participates in limb coordination...
February 13, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28211451/motor-neuron-disease-communication-for-completely-locked-in-patients
#13
Ian Fyfe
No abstract text is available yet for this article.
February 17, 2017: Nature Reviews. Neurology
https://www.readbyqxmd.com/read/28210902/deletion-of-mammalian-sterile-20-like-kinase-1-attenuates-neuronal-loss-and-improves-locomotor-function-in-a-mouse-model-of-spinal-cord-trauma
#14
Pan-Feng Wang, Da-Yuan Xu, Yuntong Zhang, Xiao-Bin Liu, Yan Xia, Pan-Yu Zhou, Qing-Ge Fu, Shuo-Gui Xu
Neuronal cell death following spinal cord injury (SCI) is an important contributor to neurological deficits. The purpose of our work was to delineate the function of mammalian sterile 20-like kinase 1 (Mst1), a pro-apoptotic kinase and key mediator of apoptotic signaling, in the pathogenesis of an experimental mouse model of SCI. Male mice received a mid-thoracic spinal contusion injury, and it was found that phosphorylation of Mst1 at the injured site was enhanced significantly following SCI. Furthermore, when compared to the wild-type controls, Mst1-deficient mice displayed improved locomotor function by increased Basso mouse scale score...
February 16, 2017: Molecular and Cellular Biochemistry
https://www.readbyqxmd.com/read/28210850/the-laminar-organization-of-the-motor-cortex-in-monodactylous-mammals-a-comparative-assessment-based-on-horse-chimpanzee-and-macaque
#15
Bruno Cozzi, Alberto De Giorgio, A Peruffo, S Montelli, M Panin, C Bombardi, A Grandis, A Pirone, P Zambenedetti, L Corain, Alberto Granato
The architecture of the neocortex classically consists of six layers, based on cytological criteria and on the layout of intra/interlaminar connections. Yet, the comparison of cortical cytoarchitectonic features across different species proves overwhelmingly difficult, due to the lack of a reliable model to analyze the connection patterns of neuronal ensembles forming the different layers. We first defined a set of suitable morphometric cell features, obtained in digitized Nissl-stained sections of the motor cortex of the horse, chimpanzee, and crab-eating macaque...
February 16, 2017: Brain Structure & Function
https://www.readbyqxmd.com/read/28209802/doublet-stimulation-increases-ca-2-binding-to-troponin-c-to-ensure-rapid-force-development-in-skeletal-muscle
#16
Anthony J Bakker, Tanya R Cully, Catherine D Wingate, Christopher J Barclay, Bradley S Launikonis
Fast-twitch skeletal muscle fibers are often exposed to motor neuron double discharges (≥200 Hz), which markedly increase both the rate of contraction and the magnitude of the resulting force responses. However, the mechanism responsible for these effects is poorly understood, likely because of technical limitations in previous studies. In this study, we measured cytosolic Ca(2+) during doublet activation using the low-affinity indicator Mag-Fluo-4 at high temporal resolution and modeled the effects of doublet stimulation on sarcoplasmic reticulum (SR) Ca(2+) release, binding of Ca(2+) to cytosolic buffers, and force enhancement in fast-twitch fibers...
February 16, 2017: Journal of General Physiology
https://www.readbyqxmd.com/read/28208729/altered-intracellular-milieu-of-adar2-deficient-motor-neurons-in-amyotrophic-lateral-sclerosis
#17
REVIEW
Takenari Yamashita, Megumi Akamatsu, Shin Kwak
Transactive response DNA-binding protein (TDP-43) pathology, and failure of A-to-I conversion (RNA editing) at the glutamine/arginine (Q/R) site of α-amino-3-hydroxy-5-methyl-4-isoxazole propionic acid (AMPA) receptor subunit GluA2, are etiology-linked molecular abnormalities that concomitantly occur in the motor neurons of most patients with amyotrophic lateral sclerosis (ALS). Adenosine deaminase acting on RNA 2 (ADAR2) specifically catalyzes GluA2 Q/R site-RNA editing. Furthermore, conditional ADAR2 knockout mice (AR2) exhibit a progressive ALS phenotype with TDP-43 pathology in the motor neurons, which is the most reliable pathological marker of ALS...
February 8, 2017: Genes
https://www.readbyqxmd.com/read/28208679/vanillin-protects-dopaminergic-neurons-against-inflammation-mediated-cell-death-by-inhibiting-erk1-2-p38-and-the-nf-%C3%AE%C2%BAb-signaling-pathway
#18
Xuan Yan, Dian-Feng Liu, Xiang-Yang Zhang, Dong Liu, Shi-Yao Xu, Guang-Xin Chen, Bing-Xu Huang, Wen-Zhi Ren, Wei Wang, Shou-Peng Fu, Ju-Xiong Liu
Neuroinflammation plays a very important role in the pathogenesis of Parkinson's disease (PD). After activation, microglia produce pro-inflammatory mediators that damage surrounding neurons. Consequently, the inhibition of microglial activation might represent a new therapeutic approach of PD. Vanillin has been shown to protect dopaminergic neurons, but the mechanism is still unclear. Herein, we further study the underlying mechanisms in lipopolysaccharide (LPS)-induced PD models. In vivo, we firstly established rat models of PD by unilateral injection of LPS into substantia nigra (SN), and then examined the role of vanillin in motor dysfunction, microglial activation and degeneration of dopaminergic neurons...
February 12, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/28208320/dynamic-model-for-kinesin-mediated-long-range-transport-and-its-local-traffic-jam-caused-by-tau-proteins
#19
Woochul Nam, Bogdan I Epureanu
In neurons, several intracellular cargoes are transported by motor proteins (kinesins) which walk on microtubules (MTs). However, kinesins can possibly unbind from the MTs before they reach their destinations. The unbound kinesins randomly diffuse in neurons until they bind to MTs. Then, they walk again along the MTs to continue their tasks. Kinesins repeat this cycle of motion until they transport their cargoes to the destinations. However, most previous models mainly focused on the motion of kinesins when they walk on MTs...
January 2017: Physical Review. E
https://www.readbyqxmd.com/read/28205668/-spinal-nephroblastoma-at-an-uncommon-localization-in-a-dog
#20
Norbert Langen, Kerstin von Pückler, Carola Tillmanns, Valérie Bornand, Martin Kramer, Martin J Schmidt
A 7-month-old intact male Otterhound was presented for diagnosis of progressive hindlimb paresis. The neurological examination revealed a severe ambulatory paraparesis localised to the left-sided region of the lower motor neuron (L4-S1). Magnetic resonance imaging was suggestive for an intradural-extramedullary neoplasia at the fifth lumbar vertebra. Surgical exploration demonstrated an intradural-extramedullary mass, which partially invaded the spinal cord. The mass was removed with the exception of the intramedullar part, and the diagnosis of a nephroblastoma was made histopathologically...
February 16, 2017: Tierärztliche Praxis. Ausgabe K, Kleintiere/Heimtiere
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