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alpha motor neuron

A D Surowka, M Töpperwien, M Bernhardt, J D Nicolas, M Osterhoff, T Salditt, D Adamek, M Szczerbowska-Boruchowska
Human dopaminergic system in general, and substantia nigra (SN) neurons, in particular, are implicated in the pathologies underlying the human brain aging. The interplay between aberrations in the structural organization and elemental composition of SN neuron bodies has recently gained in importance as selected metals: Fe, Cu, Zn, Ca were found to trigger oxidative-stress-mediated aberration in their molecular assembly due to concomitant protein (alpha-synuclein, tau-protein) aggregation, gliosis and finally oxidative stress...
December 1, 2016: Talanta
Paulo Victor Sgobbi de Souza, Wladimir Bocca Vieira de Rezende Pinto, Flávio Moura Rezende, Acary Souza Bulle Oliveira
Motor neuron disease is one of the major groups of neurodegenerative diseases, mainly represented by amyotrophic lateral sclerosis. Despite wide genetic and biochemical data regarding its pathophysiological mechanisms, motor neuron disease develops under a complex network of mechanisms not restricted to the unique functions of the alpha motor neurons but which actually involve diverse functions of glial cell interaction. This review aims to expose some of the leading roles of glial cells in the physiological mechanisms of neuron-glial cell interactions and the mechanisms related to motor neuron survival linked to glial cell functions...
October 2016: Arquivos de Neuro-psiquiatria
Cinzia Nasuti, Gloria Brunori, Piera Eusepi, Lisa Marinelli, Roberto Ciccocioppo, Rosita Gabbianelli
INTRODUCTION: Oxidative stress, alpha-synuclein changes, mitochondrial complex I defects and dopamine loss, observed in the striatum of rats exposed to the pesticide permethrin in early life, could represent neuropathological hallmarks of Parkinson's disease (PD). Nevertheless, an animal model of PD should also fulfill criteria of face and predictive validities. This study was designed to: 1) verify dopaminergic status in the striatum and substantia nigra pars compacta; 2) recognize non-motor symptoms; 3) investigate the time-course development of motor disabilities; 4) assess L-Dopa effectiveness on motor symptoms in rats previously exposed to permethrin in early life...
October 15, 2016: Journal of Pharmacological and Toxicological Methods
Dan Denis, Richard Rowe, A Mark Williams, Elizabeth Milne
The human mirror neuron system is believed to play an important role in facilitating the ability of athletes to anticipate the actions of an opponent. This system is often assessed with EEG by measuring event-related changes in mu (8-13Hz) sensorimotor oscillations. However, traditional channel-based analyses of this measure are flawed in that due to volume conduction effects mu and non-mu alpha activity can become mixed. This flaw means it is unclear the extent to which mu activity indexes the mirror system, as opposed to other processes such as attentional demand...
October 13, 2016: NeuroImage
Lucie Chochina, Florian Naudet, Isabelle Bonan, Clément Chehensse, Mireille Damphousse, Andrea Manunta, François Giuliano
OBJECTIVE: To review the efficacy of intracavernous injections (ICI) in spinal cord injured (SCI) men and to identify prognostic factors affecting the efficacy of ICI in this population. MATERIAL/PATIENTS AND METHODS: Systematic review of the literature using 5 databases. Articles published up to november 2014 using the keywords: alprostadil; papaverine; moxisylyte; alpha-blocking agent; phentolamine; intracavernous injection; spinal cord injuries; paraplegia; quadriplegia; erectile dysfunction; were included...
September 2016: Annals of Physical and Rehabilitation Medicine
D James Surmeier, Paul T Schumacker, Jaime D Guzman, Ema Ilijic, Ben Yang, Enrico Zampese
Parkinson's disease (PD) is the second most common neurodegenerative disease in the world. Its causes are poorly understood and there is no proven therapeutic strategy for slowing disease progression. The core motor symptoms of PD are caused by the death of dopaminergic neurons in the substantia nigra pars compacta (SNc). In these neurons, Ca(2+)entry through plasma membrane Cav1 channels drives a sustained feed-forward stimulation of mitochondrial oxidative phosphorylation. Although this design helps prevent bioenergetic failure when activity needs to be sustained, it leads to basal mitochondrial oxidant stress...
August 30, 2016: Biochemical and Biophysical Research Communications
Julian Scherer, Zachary A Yaffe, Michael Vershinin, Lynn W Enquist
: Alpha herpesviruses, such as herpes simplex virus and pseudorabies virus (PRV), are neuroinvasive dsDNA viruses that establish life-long latency in peripheral nervous system (PNS) neurons of their native hosts. Following reactivation, the infection can spread back to the initial mucosal site of infection or, in rare cases, to the central nervous system with usually serious outcomes. During entry and egress, viral capsids depend on microtubule-based molecular motors for efficient and fast transport...
August 31, 2016: Journal of Virology
Maria Xilouri, Oeystein Roed Brekk, Alexia Polissidis, Margarita Chrysanthou-Piterou, Ismini Kloukina, Leonidas Stefanis
Chaperone-mediated autophagy (CMA) involves the selective lysosomal degradation of cytosolic proteins such as SNCA (synuclein α), a protein strongly implicated in Parkinson disease (PD) pathogenesis. However, the physiological role of CMA and the consequences of CMA failure in the living brain remain elusive. Here we show that CMA inhibition in the adult rat substantia nigra via adeno-associated virus-mediated delivery of short hairpin RNAs targeting the LAMP2A receptor, involved in CMA's rate limiting step, was accompanied by intracellular accumulation of SNCA-positive puncta, which were also positive for UBIQUITIN, and in accumulation of autophagic vacuoles within LAMP2A-deficient nigral neurons...
August 19, 2016: Autophagy
Loek Brinkman, Arjen Stolk, Tom R Marshall, Sophie Esterer, Poppy Sharp, H Chris Dijkerman, Floris P de Lange, Ivan Toni
UNLABELLED: To select a movement, specific neuronal populations controlling particular features of that movement need to be activated, whereas other populations are downregulated. The selective (dis)inhibition of cortical sensorimotor populations is governed by rhythmic neural activity in the alpha (8-12 Hz) and beta (15-25 Hz) frequency range. However, it is unclear whether and how these rhythms contribute independently to motor behavior. Building on a recent dissociation of the sensorimotor alpha- and beta-band rhythms, we test the hypothesis that the beta-band rhythm governs the disinhibition of task-relevant neuronal populations, whereas the alpha-band rhythm suppresses neurons that may interfere with task performance...
August 17, 2016: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
Bryan S Baxter, Bradley J Edelman, Nicholas Nesbitt, Bin He
BACKGROUND: Transcranial direct current stimulation (tDCS) has been used to alter the excitability of neurons within the cerebral cortex. Improvements in motor learning have been found in multiple studies when tDCS was applied to the motor cortex before or during task learning. The motor cortex is also active during the performance of motor imagination, a cognitive task during which a person imagines, but does not execute, a movement. Motor imagery can be used with noninvasive brain computer interfaces (BCIs) to control virtual objects in up to three dimensions, but to master control of such devices requires long training times...
July 15, 2016: Brain Stimulation
Carrie J Finno, Heather J Kaese, Andrew D Miller, Giuliana Gianino, Thomas Divers, Stephanie J Valberg
OBJECTIVE: A pigment retinopathy has been reported in adult horses with equine motor neuron disease (EMND) arising from chronic α-tocopherol (α-TP) deficiency. A pigment retinopathy has not been identified in horses with neuroaxonal dystrophy/equine degenerative myeloencephalopathy (NAD/EDM) that affects genetically susceptible young horses with α-TP deficiency. The objective of this report is to describe, for the first time, a pigment retinopathy in a family of α-TP-deficient Warmbloods (WB) with clinically apparent NAD/EDM or EMND...
August 5, 2016: Veterinary Ophthalmology
Teresa H Sanders, Dieter Jaeger
Electrical deep brain stimulation (DBS) of the subthalamic nucleus (STN) is effective for ameliorating the motor symptoms of Parkinson's disease (PD) including bradykinesia. The STN receives its main excitatory input from cortex; however, the contribution of cortico-subthalamic projection neurons to the effects of DBS remains unclear. To isolate the consequences of stimulating layer 5 primary motor cortex (M1) projections to the STN, we used a dual virus transfection technique to selectively express opsins in these neurons in mice made parkinsonian by unilateral nigrostriatal 6-OHDA lesioning...
November 2016: Neurobiology of Disease
Shounak Baksi, Ajai K Tripathi, Neena Singh
Aggregation of α-synuclein (α-syn) in neurons of the substantia nigra is diagnostic of Parkinson's disease (PD), a neuro-motor disorder with prominent visual symptoms. Here, we demonstrate that α-syn, the principal protein involved in the pathogenesis of PD, is expressed widely in the neuroretina, and facilitates the uptake of transferrin-bound iron (Tf-Fe) by retinal pigment epithelial (RPE) cells that form the outer blood-retinal barrier. Absence of α-syn in knock-out mice (α-syn(-/-)) resulted in down-regulation of ferritin in the neuroretina, indicating depletion of cellular iron stores...
August 2016: Free Radical Biology & Medicine
Maria Angeliki S Pavlou, Nicoló Colombo, Sandra Fuertes-Alvarez, Sarah Nicklas, Laura Gonzalez Cano, Maria C Marín, Jorge Goncalves, Jens C Schwamborn
Alpha-synuclein is an abundant neuronal protein which has been associated with physiological processes like synaptic function, neurogenesis, and neuronal differentiation but also with pathological neurodegeneration. Indeed, alpha-synuclein (snca) is one of the major genes implicated in Parkinson's disease (PD). However, little is known about the regulation of alpha-synuclein expression. Unveiling the mechanisms that control its regulation is of high importance, as it will enable to further investigate and comprehend the physiological role of alpha-synuclein as well as its potential contribution in the aetiology of PD...
June 23, 2016: Molecular Neurobiology
Amelia Maria Gaman, Adriana Uzoni, Aurel Popa-Wagner, Anghel Andrei, Eugen-Bogdan Petcu
Chemobrain or chemotherapy induced cognitive impairment (CICI) represents a new clinical syndrome characterised by memory, learning and motor function impairment. As numerous patients with cancer are long-term survivors, CICI represent a significant factor which may interfere with their quality of life. However, this entity CICI must be distinguished from other cognitive syndromes and addressed accordingly. At the present time, experimental and clinical research suggests that CICI could be induced by numerous factors including oxidative stress...
May 2016: Aging and Disease
Craig A Erickson, Logan K Wink, Bayon Baindu, Balmiki Ray, Tori L Schaefer, Ernest V Pedapati, Debomoy K Lahiri
Angelman Syndrome is a rare neurodevelopmental disorder associated with significant developmental and communication delays, high risk for epilepsy, motor dysfunction, and a characteristic behavioral profile. While Angelman Syndrome is known to be associated with the loss of maternal expression of the ubiquitin-protein ligase E3A gene, the molecular sequelae of this loss remain to be fully understood. Amyloid precursor protein (APP) is involved in neuronal development and APP dysregulation has been implicated in the pathophysiology of other developmental disorders including fragile X syndrome and idiopathic autism...
September 2016: American Journal of Medical Genetics. Part A
Tommaso Fedele, Evgeny Blagovechtchenski, Maria Nazarova, Zafer Iscan, Victoria Moiseeva, Vadim V Nikulin
While variability of the motor responses to transcranial magnetic stimulation (TMS) is widely acknowledged, little is known about its central origin. One plausible explanation for such variability may relate to different neuronal states defining the reactivity of the cortex to TMS. In this study intrinsic spatio-temporal neuronal dynamics were estimated with Long-Range Temporal Correlations (LRTC) in order to predict the inter-individual differences in the strength of intra-cortical facilitation (ICF) and short-interval intracortical inhibition (SICI) produced by paired-pulse TMS (ppTMS) of the left primary motor cortex...
September 7, 2016: Neuroscience
I Hande Yener, Haluk Topaloglu, Sevim Erdem-Özdamar, Didem Dayangac-Erden
BACKGROUND: In single gene disorders patients who have the same genotype may show variations in severity. One of the main factors that affect disease severity is modifier genes. SMA is an autosomal recessive neuromuscular disorder caused by degeneration of alpha motor neurons. It was discovered that PLS3 was a phenotypic modifier of SMA. In the present study we found that the NRN1 gene was a potential modifier gene. We analyzed PLS3 and NRN1 expression in SMA siblings in four families...
June 9, 2016: Pediatrics International: Official Journal of the Japan Pediatric Society
Yulia Solomonov, Nurit Hadad, Rachel Levy
BACKGROUND: Amyotrophic lateral sclerosis (ALS) is a fatal multifactorial neurodegenerative disease characterized by selective death of motor neurons in the cortex, brainstem, and spinal cord. Cytosolic phospholipase A2 alpha (cPLA2α) upregulation and activation in the spinal cord of patients with sporadic ALS and in the spinal cord of human mutant SOD1G93A (hmSOD1) transgenic mice were recently reported. METHODS: cPLA2α upregulation in the brainstem and spinal cord was reduced by brain infusion of a specific antisense oligonucleotide against cPLA2α (AS), and the effect was evaluated on disease progression and brain cell activation...
2016: Journal of Neuroinflammation
Uri Wormser, Jessica Mandrioli, Marco Vinceti, Nicola Fini, Amnon Sintov, Berta Brodsky, Elena Proskura, Yoram Finkelstein
BACKGROUND: Amyotrophic lateral sclerosis (ALS) is an incurable neurodegenerative motor neuron disease that involves activation of the immune system and inflammatory response in the nervous system. Reduced level of the immuno-modulatory and anti-inflammatory protein alpha-1-antitrypsin (AAT) is associated with inflammation-related pathologies. The objective of the present is to determine AAT levels and IL-23 in the cerebrospinal fluid (CSF) of ALS patients and control group. FINDINGS: CSF samples from newly diagnosed ALS patients and age-matched controls were analyzed for AAT and IL-23 by ELISA and magnetic luminex screening, respectively...
2016: Journal of Neuroinflammation
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