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Motor neuron disease

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https://www.readbyqxmd.com/read/29792848/subthalamic-nucleus-deep-brain-stimulation-protects-neurons-by-activating-autophagy-via-pp2a-inactivation-in-a-rat-model-of-parkinson-s-disease
#1
Ting-Ting Du, Ying-Chuan Chen, Yong-Quan Lu, Fan-Gang Meng, Hui Yang, Jian-Guo Zhang
Deep brain stimulation (DBS) of the subthalamic nucleus (STN) is an effective therapeutic strategy for alleviating disability in patients with moderate to severe Parkinson's disease (PD). Preclinical studies have shown that stimulation of the rat STN can protect against nigral dopaminergic neuron loss. However, the underlying mechanism is unclear. To investigate the molecular basis of the neuroprotective effects of STN stimulation, a rat model of PD was established by unilaterally injecting 6-hydroxydopamine (6-OHDA) into the striatum...
May 21, 2018: Experimental Neurology
https://www.readbyqxmd.com/read/29791914/atlas-independent-electrophysiological-mapping-of-the-optimal-locus-of-subthalamic-deep-brain-stimulation-for-the-motor-symptoms-of-parkinson-disease
#2
Erin C Conrad, James M Mossner, Kelvin L Chou, Parag G Patil
BACKGROUND/AIMS: Deep brain stimulation (DBS) of the subthalamic nucleus (STN) improves motor symptoms of Parkinson disease (PD). However, motor outcomes can be variable, perhaps due to inconsistent positioning of the active contact relative to an unknown optimal locus of stimulation. Here, we determine the optimal locus of STN stimulation in a geometrically unconstrained, mathematically precise, and atlas-independent manner, using Unified Parkinson Disease Rating Scale (UPDRS) motor outcomes and an electrophysiological neuronal stimulation model...
May 23, 2018: Stereotactic and Functional Neurosurgery
https://www.readbyqxmd.com/read/29790963/the-presenilin-loop-region-is-essential-for-glycogen-synthase-kinase-3-beta-mediated-functions-on-motor-proteins-during-axonal-transport
#3
Rupkatha Banerjee, Zoe Rudloff, Crystal Naylor, Michael Yu, Shermali Gunawardena
Neurons require intracellular transport of essential components for function and viability, and defects in transport has been implicated in many neurodegenerative diseases including Alzheimer's disease (AD). One possible mechanism by which transport defects could occur is by improper regulation of molecular motors. Previous work showed that reduction of Presenilin (PS) or Glycogen synthase kinase 3 beta (GSK3β) stimulated APP vesicle motility. Excess GSK3β caused transport defects and increased motor binding to membranes, while reduction of PS decreased active GSK3β and motor binding to membranes...
May 22, 2018: Human Molecular Genetics
https://www.readbyqxmd.com/read/29790918/temporal-and-tissue-specific-variability-of-smn-protein-levels-in-mouse-models-of-spinal-muscular-atrophy
#4
Ewout Jn Groen, Elena Perenthaler, Natalie L Courtney, Crispin Y Jordan, Hannah K Shorrock, Dinja van der Hoorn, Yu-Ting Huang, Lyndsay M Murray, Gabriella Viero, Thomas H Gillingwater
Spinal muscular atrophy (SMA) is a progressive motor neuron disease caused by deleterious variants in SMN1 that lead to a marked decrease in survival motor neuron (SMN) protein expression. Humans have a second SMN gene (SMN2) that is almost identical to SMN1. However, due to alternative splicing the majority of SMN2 mRNA is translated into a truncated, unstable protein that is quickly degraded. Because the presence of SMN2 provides a unique opportunity for therapy development in SMA patients, the mechanisms that regulate SMN2 splicing and mRNA expression have been elucidated in great detail...
May 22, 2018: Human Molecular Genetics
https://www.readbyqxmd.com/read/29790195/a-licence-to-drive-neurological-illness-loss-and-disruption
#5
Melissa Stepney, Susan Kirkpatrick, Louise Locock, Suman Prinjha, Sara Ryan
The sense of freedom and independence that being able to drive generates may be taken for granted by many until it is threatened by illness. Drawing on the 'mobility turn' in social sciences that emphasises the social and emotional significance of the car (Sheller and Urry , ), this article presents secondary analysis of narratives of driving and its significance across four neurological conditions (epilepsy, Parkinson's disease, transient ischaemic attack and motor neurone disease). Taking an interactionist approach we explore how the withdrawal of a driving licence can represent not just a practical and emotional loss of independence, but also loss of enjoyment; of a sense and feeling of 'normal' adulthood and social participation; and of an identity (in some cases gendered) of strength and power...
May 22, 2018: Sociology of Health & Illness
https://www.readbyqxmd.com/read/29789966/beh%C3%A3-et-s-syndrome-and-nervous-system-involvement
#6
REVIEW
Uğur Uygunoğlu, Aksel Siva
PURPOSE OF REVIEW: Although Behçet's syndrome (BS) is classified as a rare disease in European countries and the USA, its neurologic involvement "neuro-Behçet's syndrome (NBS)" is commonly included in the differential diagnosis of many inflammatory and vascular central nervous system (CNS) disorders. Clinical and neuroimaging findings support two major forms of NBS: parenchymal NBS (p-NBS) and an extra-parenchymal form that presents with cerebral venous sinus thrombosis (CVST)...
May 23, 2018: Current Neurology and Neuroscience Reports
https://www.readbyqxmd.com/read/29789581/intron-retention-and-nuclear-loss-of-sfpq-are-molecular-hallmarks-of-als
#7
Raphaelle Luisier, Giulia E Tyzack, Claire E Hall, Jamie S Mitchell, Helen Devine, Doaa M Taha, Bilal Malik, Ione Meyer, Linda Greensmith, Jia Newcombe, Jernej Ule, Nicholas M Luscombe, Rickie Patani
Mutations causing amyotrophic lateral sclerosis (ALS) strongly implicate ubiquitously expressed regulators of RNA processing. To understand the molecular impact of ALS-causing mutations on neuronal development and disease, we analysed transcriptomes during in vitro differentiation of motor neurons (MNs) from human control and patient-specific VCP mutant induced-pluripotent stem cells (iPSCs). We identify increased intron retention (IR) as a dominant feature of the splicing programme during early neural differentiation...
May 22, 2018: Nature Communications
https://www.readbyqxmd.com/read/29789529/inhibiting-p38-mapk-alpha-rescues-axonal-retrograde-transport-defects-in-a-mouse-model-of-als
#8
Katherine L Gibbs, Bernadett Kalmar, Elena R Rhymes, Alexander D Fellows, Mahmood Ahmed, Paul Whiting, Ceri H Davies, Linda Greensmith, Giampietro Schiavo
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease caused by the degeneration of upper and lower motor neurons. Defects in axonal transport have been observed pre-symptomatically in the SOD1G93A mouse model of ALS, and have been proposed to play a role in motor neuron degeneration as well as in other pathologies of the nervous system, such as Alzheimer's disease and hereditary neuropathies. In this study, we screen a library of small-molecule kinase inhibitors towards the identification of pharmacological enhancers of the axonal retrograde transport of signalling endosomes, which might be used to normalise the rate of this process in diseased neurons...
May 22, 2018: Cell Death & Disease
https://www.readbyqxmd.com/read/29789378/subthalamic-nucleus-neurons-differentially-encode-early-and-late-aspects-of-speech-production
#9
W J Lipski, A Alhourani, T Pirnia, P W Jones, C Dastolfo-Hromack, L B Helou, D J Crammond, S Shaiman, M W Dickey, L L Holt, R S Turner, J A Fiez, R M Richardson
Basal ganglia-thalamocortical loops mediate all motor behavior, yet little detail is known about the role of basal ganglia nuclei in speech production. Using intracranial recording during deep brain stimulation surgery in humans with Parkinson's disease, we tested the hypothesis that the firing rate of subthalamic nucleus neurons is modulated in sync with motor execution aspects of speech. Nearly half of seventy-nine unit recordings exhibited firing rate modulation, during a syllable reading task across twelve subjects (male and female)...
May 22, 2018: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/29788038/testing-a-longitudinal-compensation-model-in-premanifest-huntington-s-disease
#10
Sarah Gregory, Jeffrey D Long, Stefan Klöppel, Adeel Razi, Elisa Scheller, Lora Minkova, Eileanoir B Johnson, Alexandra Durr, Raymund A C Roos, Blair R Leavitt, James A Mills, Julie C Stout, Rachael I Scahill, Sarah J Tabrizi, Geraint Rees
The initial stages of neurodegeneration are commonly marked by normal levels of cognitive and motor performance despite the presence of structural brain pathology. Compensation is widely assumed to account for this preserved behaviour, but despite the apparent simplicity of such a concept, it has proven incredibly difficult to demonstrate such a phenomenon and distinguish it from disease-related pathology. Recently, we developed a model of compensation whereby brain activation, behaviour and pathology, components key to understanding compensation, have specific longitudinal trajectories over three phases of progression...
May 17, 2018: Brain: a Journal of Neurology
https://www.readbyqxmd.com/read/29787708/emerging-links-between-lipid-droplets-and-motor-neuron-diseases
#11
Giuseppa Pennetta, Michael A Welte
Lipid droplets (LDs) are ubiquitous fat storage organelles and play key roles in lipid metabolism and energy homeostasis; in addition, they contribute to protein storage, folding, and degradation. However, a role for LDs in the nervous system remains largely unexplored. We discuss evidence supporting an intimate functional connection between LDs and motor neuron disease (MND) pathophysiology, examining how LD functions in systemic energy homeostasis, in neuron-glia metabolic coupling, and in protein folding and clearance may affect or contribute to disease pathology...
May 21, 2018: Developmental Cell
https://www.readbyqxmd.com/read/29787578/mth1-driven-expression-of-htdp-43-results-in-typical-als-ftld-neuropathological-symptoms
#12
Barbara Scherz, Roland Rabl, Stefanie Flunkert, Siegfried Rohler, Joerg Neddens, Nicole Taub, Magdalena Temmel, Ute Panzenboeck, Vera Niederkofler, Robert Zimmermann, Birgit Hutter-Paier
Transgenic mouse models are indispensable tools to mimic human diseases and analyze the effectiveness of related new drugs. For a long time amyotrophic lateral sclerosis (ALS) research depended on only a few mouse models that exhibit a very strong and early phenotype, e.g. SOD1 mice, resulting in a short treatment time window. By now, several models are available that need to be characterized to highlight characteristics of each model. Here we further characterized the mThy1-hTDP-43 transgenic mouse model TAR6/6 that overexpresses wild type human TARDBP, also called TDP-43, under control of the neuronal Thy-1 promoter presented by Wils and colleagues, 2010, by using biochemical, histological and behavioral readouts...
2018: PloS One
https://www.readbyqxmd.com/read/29786645/skeletal-muscle-micrornas-as-key-players-in-the-pathogenesis-of-amyotrophic-lateral-sclerosis
#13
REVIEW
Lorena Di Pietro, Wanda Lattanzi, Camilla Bernardini
Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disorder, for which, to date, no effective treatment to ameliorate the clinical manifestations is available. The long-standing view of ALS as affecting only motor neurons has been challenged by the finding that the skeletal muscle plays an active role in the disease pathogenesis and can be a valuable target for therapeutic strategies. In recent years, non-coding RNAs, including microRNAs, have emerged as important molecules that play key roles in several cellular mechanisms involved in the pathogenic mechanisms underlying various human conditions...
May 22, 2018: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/29786422/mechanisms-regulating-the-association-of-protein-phosphatase-1-with-spinophilin-and-neurabin
#14
Michael C Edler, Asma B Salek, Darryl S Watkins, Harjot Kaur, Cameron W Morris, Bryan K Yamamoto, Anthony J Baucum
Protein phosphorylation is a key mediator of signal transduction, allowing for dynamic regulation of substrate activity. Whereas protein kinases obtain substrate specificity by targeting specific amino acid sequences, serine/threonine phosphatase catalytic subunits are much more promiscuous in their ability to dephosphorylate substrates. To obtain substrate specificity, serine/threonine phosphatases utilize targeting proteins to regulate phosphatase subcellular localization and catalytic activity. Spinophilin and its homolog neurabin are two of the most abundant dendritic spine-localized protein phosphatase 1 (PP1) targeting proteins...
May 22, 2018: ACS Chemical Neuroscience
https://www.readbyqxmd.com/read/29786160/aberrant-learning-in-parkinson-s-disease-a-neurocomputational-study-on-bradykinesia
#15
Mauro Ursino, Chiara Baston
Parkinson's disease (PD) is a neurodegenerative disorder characterized by a progressive decline in motor functions, such as bradykinesia, caused by the pathological denervation of nigrostriatal dopaminergic neurons within the basal ganglia (BG). It is acknowledged that dopamine (DA) directly affects the modulatory role of BG towards the cortex. However, a growing body of literature is suggesting that DA induced aberrant synaptic plasticity could play a role in the core symptoms of PD, thus recalling for a "reconceptualization" of the pathophysiology...
May 22, 2018: European Journal of Neuroscience
https://www.readbyqxmd.com/read/29786072/mptp-driven-nlrp3-inflammasome-activation-in-microglia-plays-a-central-role-in-dopaminergic-neurodegeneration
#16
Eunju Lee, Inhwa Hwang, Sangjun Park, Sujeong Hong, Boreum Hwang, Yoeseph Cho, Junghyun Son, Je-Wook Yu
Parkinson's disease (PD) is a progressive neurodegenerative disease characterized by the loss of dopaminergic neurons in the substantia nigra (SN) and the reduction of dopamine levels in the striatum. Although details of the molecular mechanisms underlying dopaminergic neuronal death in PD remain unclear, neuroinflammation is also considered a potent mediator in the pathogenesis and progression of PD. In the present study, we present evidences that microglial NLRP3 inflammasome activation is critical for dopaminergic neuronal loss and the subsequent motor deficits in the 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP) mouse model of PD...
May 21, 2018: Cell Death and Differentiation
https://www.readbyqxmd.com/read/29785199/physical-exercise-for-parkinson-s-disease-clinical-and-experimental-evidence
#17
REVIEW
Alessandro Oliveira de Carvalho, Alberto Souza Sá Filho, Eric Murillo-Rodriguez, Nuno Barbosa Rocha, Mauro Giovanni Carta, Sergio Machado
Background: National projections about the increase in the elderly population over 60 years bring with it an increase in the number of people affected by Parkinson's Disease (PD), making it an important public health problem. Therefore, the establishment of effective strategies for intervention in people with PD needs to be more clearly investigated. Objective: The study aimed to report the effectiveness of exercise on functional capacity and neurobiological mechanisms in people with PD...
2018: Clinical Practice and Epidemiology in Mental Health: CP & EMH
https://www.readbyqxmd.com/read/29784949/neuronal-activity-regulates-drosha-via-autophagy-in-spinal-muscular-atrophy
#18
Inês do Carmo G Gonçalves, Johanna Brecht, Maximilian P Thelen, Wiebke A Rehorst, Miriam Peters, Hyun Ju Lee, Susanne Motameny, Laura Torres-Benito, Darius Ebrahimi-Fakhari, Natalia L Kononenko, Janine Altmüller, David Vilchez, Mustafa Sahin, Brunhilde Wirth, Min Jeong Kye
Dysregulated miRNA expression and mutation of genes involved in miRNA biogenesis have been reported in motor neuron diseases including spinal muscular atrophy (SMA) and amyotrophic lateral sclerosis (ALS). Therefore, identifying molecular mechanisms governing miRNA expression is important to understand these diseases. Here, we report that expression of DROSHA, which is a critical enzyme in the microprocessor complex and essential for miRNA biogenesis, is reduced in motor neurons from an SMA mouse model. We show that DROSHA is degraded by neuronal activity induced autophagy machinery, which is also dysregulated in SMA...
May 21, 2018: Scientific Reports
https://www.readbyqxmd.com/read/29784724/author-response-anti-cytosolic-5-nucleotidase-1a-cn1a-autoantibodies-in-motor-neuron-diseases
#19
Teerin Liewluck
No abstract text is available yet for this article.
May 22, 2018: Neurology
https://www.readbyqxmd.com/read/29784723/reader-response-anti-cytosolic-5-nucleotidase-1a-cn1a-autoantibodies-in-motor-neuron-diseases
#20
Adrian Budhram
No abstract text is available yet for this article.
May 22, 2018: Neurology
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