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Motor neuron development

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https://www.readbyqxmd.com/read/28522961/energy-homeostasis-and-abnormal-rna-metabolism-in-amyotrophic-lateral-sclerosis
#1
REVIEW
Yu-Ju Liu, Po-Yi Tsai, Yijuang Chern
Amyotrophic lateral sclerosis (ALS) is a fatal motor neuron disease that is clinically characterized by progressive muscle weakness and impaired voluntary movement due to the loss of motor neurons in the brain, brain stem and spinal cord. To date, no effective treatment is available. Ample evidence suggests that impaired RNA homeostasis and abnormal energy status are two major pathogenesis pathways in ALS. In the present review article, we focus on recent studies that report molecular insights of both pathways, and discuss the possibility that energy dysfunction might negatively regulate RNA homeostasis via the impairment of cytoplasmic-nuclear shuttling in motor neurons and subsequently contribute to the development of ALS...
2017: Frontiers in Cellular Neuroscience
https://www.readbyqxmd.com/read/28522960/motomirs-mirnas-in-motor-neuron-function-and-disease
#2
REVIEW
Zachary C E Hawley, Danae Campos-Melo, Cristian A Droppelmann, Michael J Strong
MiRNAs are key regulators of the mammalian transcriptome that have been increasingly linked to degenerative diseases of the motor neurons. Although many of the miRNAs currently incriminated as participants in the pathogenesis of these diseases are also important to the normal development and function of motor neurons, at present there is no knowledge of the complete miRNA profile of motor neurons. In this review, we examine the current understanding with respect to miRNAs that are specifically required for motor neuron development, function and viability, and provide evidence that these should be considered as a functional network of miRNAs which we have collectively termed MotomiRs...
2017: Frontiers in Molecular Neuroscience
https://www.readbyqxmd.com/read/28521132/linking-neurons-to-network-function-and-behavior-by-two-photon-holographic-optogenetics-and-volumetric-imaging
#3
Marco Dal Maschio, Joseph C Donovan, Thomas O Helmbrecht, Herwig Baier
We introduce a flexible method for high-resolution interrogation of circuit function, which combines simultaneous 3D two-photon stimulation of multiple targeted neurons, volumetric functional imaging, and quantitative behavioral tracking. This integrated approach was applied to dissect how an ensemble of premotor neurons in the larval zebrafish brain drives a basic motor program, the bending of the tail. We developed an iterative photostimulation strategy to identify minimal subsets of channelrhodopsin (ChR2)-expressing neurons that are sufficient to initiate tail movements...
May 17, 2017: Neuron
https://www.readbyqxmd.com/read/28520647/altered-brain-structure-and-function-associated-with-sensory-and-affective-components-of-classic-trigeminal-neuralgia
#4
Yuan Wang, Dong-Yuan Cao, Bethany Remeniuk, Samuel Krimmel, David A Seminowicz, Ming Zhang
Classic trigeminal neuralgia (CTN) is a chronic neuropathic pain state characterized by intense, piercing spasms of the orofacial region, and may be attributable to abnormal pain processing in the central nervous system. Our study investigated neuronal alterations using voxel-based morphometry (VBM), diffuse tensor imaging (DTI), and resting state functional connectivity in 38 CTN patients and 38 matched healthy controls. For VBM analyses, CTN patients displayed gray matter volume (GMV) reductions in the anterior and mid cingulate cortex (ACC and MCC), insula, secondary somatosensory cortex (S2), primary motor cortex (M1), premotor area (PMA), and several regions in the temporal lobe...
May 16, 2017: Pain
https://www.readbyqxmd.com/read/28518055/a-positive-feedback-loop-linking-enhanced-mglur-function-and-basal-calcium-in-spinocerebellar-ataxia-type-2
#5
Pratap Meera, Stefan Pulst, Thomas Otis
Metabotropic glutamate receptor 1 (mGluR1) function in Purkinje neurons (PNs) is essential for cerebellar development and for motor learning and altered mGluR1 signaling causes ataxia. Downstream of mGluR1, dysregulation of calcium homeostasis has been hypothesized as a key pathological event in genetic forms of ataxia but the underlying mechanisms remain unclear. We find in a spinocerebellar ataxia type 2 (SCA2) mouse model that calcium homeostasis in PNs is disturbed across a broad range of physiological conditions...
May 18, 2017: ELife
https://www.readbyqxmd.com/read/28513234/the-development-of-the-uk-national-institute-of-health-and-care-excellence-evidence-based-clinical-guidelines-on-motor-neurone-disease
#6
David Oliver, Aleksandar Radunovic, Alexander Allen, Christopher McDermott
The care of people with motor neuron disease/amyotrophic lateral sclerosis is often complex and involves a wide multidisciplinary team approach. The National Institute for Health and Care Excellence (NICE) in the UK has produced an evidence based guideline for the management of patients. This has made recommendations, based on clear evidence or consensus discussion. The evidence is often limited and areas for further research are suggested.
May 17, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/28512401/increased-gamma-connectivity-in-the-human-prefrontal-cortex-during-the-bereitschaftspotential
#7
Kisun Kim, June Sic Kim, Chun Kee Chung
The Bereitschaftspotential (BP) is a slow negative cortical potential preceding voluntary movement. Since movement preparation is dependent upon the synchronous activity of a variety of neurons, BP may develop through the exchange of information among motor-related neurons. However, the relationship between BP and information flow is not yet well-known. In the present study, we aimed to investigate how the connectivity in the prefrontal cortex (PFC) changes during the occurrence of BP. Electrocorticography (ECoG) was recorded in five patients with epilepsy...
2017: Frontiers in Human Neuroscience
https://www.readbyqxmd.com/read/28511137/effects-of-ibuprofen-diclofenac-and-paracetamol-on-hatch-and-motor-behavior-in-developing-zebrafish-danio-rerio
#8
Liang Xia, Liang Zheng, Jun Liang Zhou
Non-steroidal anti-inflammatory drugs (NSAIDs) which are widely used as pain relief medicines are causing increasing environmental concern due to their incomplete removal in wastewater treatment plant and potential toxicity on endocrine, kidney and reproduction in teleost fish. This study focused on the effects of widely used ibuprofen, diclofenac and paracetamol on the hatch and motor ability of early-stage zebrafish, by exposing embryos to the target chemicals at 5, 50 and 500 μg/L starting from 6 h postfertilization (hpf)...
May 10, 2017: Chemosphere
https://www.readbyqxmd.com/read/28510727/alternative-splicing-in-the-c-terminal-tail-of-cav2-1-is-essential-for-preventing-a-neurological-disease-in-mice
#9
Tomonori Aikawa, Takaki Watanabe, Taisuke Miyazaki, Takayasu Mikuni, Minoru Wakamori, Miyano Sakurai, Hidenori Aizawa, Nobutaka Ishizu, Masahiko Watanabe, Masanobu Kano, Hidehiro Mizusawa, Kei Watase
Alternative splicing (AS) that occurs at the final coding exon (exon 47) of the Cav2.1 voltage-gated calcium channel (VGCC) gene produces two major isoforms in the brain, MPI and MPc. These isoforms differ in their splice acceptor sites; human MPI is translated into a polyglutamine tract associated with spinocerebellar ataxia type 6 (SCA6), whereas MPc splices to an immediate stop codon, resulting in a shorter cytoplasmic tail.To gain insight into the functional role of the AS in vivo and whether modulating the splice patterns at this locus can be a potential therapeutic strategy for SCA6, here we created knockin mice that exclusively express MPc by inserting the splice-site mutation...
May 16, 2017: Human Molecular Genetics
https://www.readbyqxmd.com/read/28506879/a-new-platform-for-long-term-tracking-and-recording-of-neural-activity-and-simultaneous-optogenetic-control-in-freely-behaving-caenorhabditis-elegans
#10
Keiko Gengyo-Ando, Yuko Kagawa-Nagamura, Masamichi Ohkura, Xianfeng Fei, Min Chen, Koichi Hashimoto, Junichi Nakai
BACKGROUND: Real-time recording and manipulation of neural activity in freely behaving animals can greatly advance our understanding of how neural circuits regulate behavior. Ca(2+) imaging and optogenetic manipulation with optical probes are key technologies for this purpose. However, integrating the two optical approaches with behavioral analysis has been technically challenging. NEW METHOD: Here, we developed a new imaging system, ICaST (Integrated platform for Ca(2+) imaging, Stimulation, and Tracking), which combines an automatic worm tracking system and a fast-scanning laser confocal microscope, to image neurons of interest in freely behaving C...
May 12, 2017: Journal of Neuroscience Methods
https://www.readbyqxmd.com/read/28506502/autonomic-dysreflexia-after-spinal-cord-injury-systemic-pathophysiology-and-methods-of-management
#11
REVIEW
Khalid C Eldahan, Alexander G Rabchevsky
Traumatic spinal cord injury (SCI) has widespread physiological effects beyond the disruption of sensory and motor function, notably the loss of normal autonomic and cardiovascular control. Injury at or above the sixth thoracic spinal cord segment segregates critical spinal sympathetic neurons from supraspinal modulation which can result in a syndrome known as autonomic dysreflexia (AD). AD is defined as episodic hypertension and concomitant baroreflex-mediated bradycardia initiated by unmodulated sympathetic reflexes in the decentralized cord...
May 8, 2017: Autonomic Neuroscience: Basic & Clinical
https://www.readbyqxmd.com/read/28504671/reduced-sensory-synaptic-excitation-impairs-motor-neuron-function-via-kv2-1-in-spinal-muscular-atrophy
#12
Emily V Fletcher, Christian M Simon, John G Pagiazitis, Joshua I Chalif, Aleksandra Vukojicic, Estelle Drobac, Xiaojian Wang, George Z Mentis
Behavioral deficits in neurodegenerative diseases are often attributed to the selective dysfunction of vulnerable neurons via cell-autonomous mechanisms. Although vulnerable neurons are embedded in neuronal circuits, the contributions of their synaptic partners to disease process are largely unknown. Here we show that, in a mouse model of spinal muscular atrophy (SMA), a reduction in proprioceptive synaptic drive leads to motor neuron dysfunction and motor behavior impairments. In SMA mice or after the blockade of proprioceptive synaptic transmission, we observed a decrease in the motor neuron firing that could be explained by the reduction in the expression of the potassium channel Kv2...
May 15, 2017: Nature Neuroscience
https://www.readbyqxmd.com/read/28503316/recovery-of-supraspinal-control-of-leg-movement-in-a-chronic-complete-flaccid-paraplegic-man-after-continuous-low-frequency-pelvic-nerve-stimulation-and-fes-assisted-training
#13
Marc Possover, Axel Forman
INTRODUCTION: More than 30 years ago, functional electrical stimulation (FES) was developed as an orthotic system to be used for rehabilitation for SCI patients. In the present case report, FES-assisted training was combined with continuous low-frequency stimulation of the pelvic somatic nerves in a SCI patient. CASE PRESENTATION: We report on unexpected findings in a 41-year-old man with chronic complete flaccid paraplegia, since he was 18 years old, who underwent spinal stem cell therapy and a laparoscopic implantation of neuroprosthesis (LION procedure) in the pelvic lumbosacral nerves...
2017: Spinal Cord Series and Cases
https://www.readbyqxmd.com/read/28500298/zbp1-phosphorylation-at-serine-181-regulates-its-dendritic-transport-and-the-development-of-dendritic-trees-of-hippocampal-neurons
#14
Anna S Urbanska, Aleksandra Janusz-Kaminska, Katarzyna Switon, Alicia L Hawthorne, Malgorzata Perycz, Malgorzata Urbanska, Gary J Bassell, Jacek Jaworski
Local protein synthesis occurs in axons and dendrites of neurons, enabling fast and spatially restricted responses to a dynamically changing extracellular environment. Prior to local translation, mRNA that is to be translated is packed into ribonucleoprotein particles (RNPs) where RNA binding proteins ensure mRNA silencing and provide a link to molecular motors. ZBP1 is a component of RNP transport particles and is known for its role in the local translation of β-actin mRNA. Its binding to mRNA is regulated by tyrosine 396 phosphorylation, and this particular modification was shown to be vital for axonal growth and dendritic branching...
May 12, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28500220/environmental-enrichment-rescues-binocular-matching-of-orientation-preference-in-the-mouse-visual-cortex
#15
Jared Levine, Hui Chen, Yu Gu, Jianhua Cang
Neural circuits are shaped by experience during critical periods of development. Sensory deprivation during these periods permanently compromises an organism's ability to perceive the outside world. In the mouse visual system, normal visual experience during a critical period in early life drives the matching of individual cortical neurons' orientation preferences through the two eyes, likely a key step in the development of binocular vision. Here, in mice of both sexes, we show that the binocular matching process is completely blocked by monocular deprivation spanning the entire critical period...
May 12, 2017: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/28500219/mechanisms-of-enhanced-phrenic-long-term-facilitation-in-sod1-g93a-rats
#16
Nicole L Nichols, Irawan Satriotomo, Latoya L Allen, Ashley M Grebe, Gordon S Mitchell
Amyotrophic lateral sclerosis (ALS) is a degenerative motor neuron disease, causing muscle paralysis and death from respiratory failure. Effective means to preserve/restore ventilation are necessary to increase the quality and duration of life in ALS patients. At disease end-stage in a rat ALS model (SOD1(G93A)), acute intermittent hypoxia (AIH) restores phrenic nerve activity to normal levels via enhanced phrenic long-term facilitation (pLTF). Mechanisms enhancing pLTF in end-stage SOD1(G93A) rats are not known...
May 12, 2017: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/28496512/takayasu-s-arteritis-presenting-with-headache-and-peripheral-facial-palsy-a-case-report
#17
Maryam Sotoudeh Anvari, Farzad Masoudkabir, Kyomars Abbasi, Mohammad Ali Boroumand, Manijeh Zarghampour, Hamidreza Goodarzynejad
Takayasu's arteritis (TA) is a rare case of granulomatous arteritis which mainly involves the aorta and its large branches. Although arterial hypertension is the most common feature of the disease in both adults and children, patients with TA may present with numerous clinical manifestations. Our patient was a 45-year-old woman, known to have hypertension from 3 years earlier following assessments made for severe headache. One year after the diagnosis of hypertension, she developed a left-sided lower motor neuron facial palsy, which was treated with oral corticosteroids (Prednisolone)...
October 3, 2016: Journal of Tehran Heart Center
https://www.readbyqxmd.com/read/28495800/mechanically-patterned-neuromuscular-junctions-in-a-dish-have-improved-functional-maturation
#18
Cassandra L Happe, Kevin P Tenerelli, Anastasia K Gromova, Frederic Kolb, Adam J Engler
Motor neuron (MN) diseases are progressive disorders resulting from degeneration of neuromuscular junctions (NMJs), which form the connection between MNs and muscle fibers. NMJ-in-a-dish models have been developed to examine human MN-associated dysfunction with disease; however such co-culture models have randomly oriented myotubes with immature synapses that contract asynchronously. Mechanically patterned (MP) extracellular matrix with alternating soft and stiff stripes improve current NMJ-in-a-dish models by inducing both mouse and human myoblast durotaxis to stripes where they aligned, differentiated, and fused into patterned myotubes...
May 11, 2017: Molecular Biology of the Cell
https://www.readbyqxmd.com/read/28494967/visualizing-calcium-flux-in-freely-moving-nematode-embryos
#19
Evan L Ardiel, Abhishek Kumar, Joseph Marbach, Ryan Christensen, Rishi Gupta, William Duncan, Jonathan S Daniels, Nico Stuurman, Daniel Colón-Ramos, Hari Shroff
The lack of physiological recordings from Caenorhabditis elegans embryos stands in stark contrast to the comprehensive anatomical and gene expression datasets already available. Using light-sheet fluorescence microscopy to address the challenges associated with functional imaging at this developmental stage, we recorded calcium dynamics in muscles and neurons and developed analysis strategies to relate activity and movement. In muscles, we found that the initiation of twitching was associated with a spreading calcium wave in a dorsal muscle bundle...
May 9, 2017: Biophysical Journal
https://www.readbyqxmd.com/read/28490756/expressing-acetylcholine-receptors-after-innervation-suppresses-spontaneous-vesicle-release-and-causes-muscle-fatigue
#20
Meghan Mott, Victor M Luna, Jee-Young Park, Gerald B Downes, Kimberly Epley, Fumihito Ono
The formation and function of synapses are tightly orchestrated by the precise timing of expression of specific molecules during development. In this study, we determined how manipulating the timing of expression of postsynaptic acetylcholine receptors (AChRs) impacts presynaptic release by establishing a genetically engineered zebrafish line in which we can freely control the timing of AChR expression in an AChR-less fish background. With the delayed induction of AChR expression after an extensive period of AChR-less development, paralyzed fish displayed a remarkable level of recovery, exhibiting a robust escape response following developmental delay...
May 10, 2017: Scientific Reports
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