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https://www.readbyqxmd.com/read/29160009/mirna-in-spinal-muscular-atrophy-pathogenesis-and-therapy
#1
REVIEW
Francesca Magri, Fiammetta Vanoli, Stefania Corti
Spinal muscular atrophy (SMA) is an autosomal recessive neurodegenerative disease characterized by the selective death of lower motor neurons in the brain stem and spinal cord. SMA is caused by mutations in the survival motor neuron 1 gene (SMN1), leading to the reduced expression of the full-length SMN protein. microRNAs (miRNAs) are small RNAs that regulate post-transcriptional gene expression. Recent findings have suggested an important role for miRNAs in the pathogenesis of motor neuron diseases, including SMA...
November 21, 2017: Journal of Cellular and Molecular Medicine
https://www.readbyqxmd.com/read/29159770/jip3-localises-to-exocytic-vesicles-and-focal-adhesions-in-the-growth-cones-of-differentiated-pc12-cells
#2
Patrick T Caswell, Martin Dickens
The JNK-interacting protein 3 (JIP3) is a molecular scaffold, expressed predominantly in neurons, that serves to coordinate the activation of the c-Jun N-terminal kinase (JNK) by binding to JNK and the upstream kinases involved in its activation. The JNK pathway is involved in the regulation of many cellular processes including the control of cell survival, cell death and differentiation. JIP3 also associates with microtubule motor proteins such as kinesin and dynein and is likely an adapter protein involved in the tethering of vesicular cargoes to the motors involved in axonal transport in neurons...
November 20, 2017: Molecular and Cellular Biochemistry
https://www.readbyqxmd.com/read/29159046/imaging-of-glia-activation-in-people-with-primary-lateral-sclerosis
#3
Sabrina Paganoni, Mohamad J Alshikho, Nicole R Zürcher, Paul Cernasov, Suma Babu, Marco L Loggia, James Chan, Daniel B Chonde, David Izquierdo Garcia, Ciprian Catana, Caterina Mainero, Bruce R Rosen, Merit E Cudkowicz, Jacob M Hooker, Nazem Atassi
Background: Glia activation is thought to contribute to neuronal damage in several neurodegenerative diseases based on preclinical and human post-mortem studies, but its role in primary lateral sclerosis (PLS) is unknown. Objectives: To localize and measure glia activation in people with PLS compared to healthy controls (HC). Methods: Ten participants with PLS and ten age-matched HCs underwent simultaneous magnetic resonance (MR) and proton emission tomography (PET)...
2018: NeuroImage: Clinical
https://www.readbyqxmd.com/read/29158983/adult-zebra-finches-rehearse-highly-variable-song-patterns-during-sleep
#4
Brent K Young, Gabriel B Mindlin, Ezequiel Arneodo, Franz Goller
Brain activity during sleep is fairly ubiquitous and the best studied possible function is a role in memory consolidation, including motor memory. One suggested mechanism of how neural activity effects these benefits is through reactivation of neurons in patterns resembling those of the preceding experience. The specific patterns of motor activation replayed during sleep are largely unknown for any system. Brain areas devoted to song production in the songbird brain exhibit spontaneous song-like activity during sleep, but single cell neural recordings did not permit detection of the specific song patterns...
2017: PeerJ
https://www.readbyqxmd.com/read/29158022/activation-of-the-nrf2-signaling-pathway-and-neuroprotection-of-nigral-dopaminergic-neurons-by-a-novel-synthetic-compound-kms99220
#5
Ji Ae Lee, Hyo Jin Son, Ji Won Choi, Jinwoo Kim, Se Hee Han, Nari Shin, Ji Hyun Kim, Soo Jeong Kim, Jun Young Heo, Dong Jin Kim, Ki Duk Park, Onyou Hwang
The transcription factor Nrf2 is known to induce gene expression of antioxidant enzymes and proteasome subunits. Because both oxidative stress and protein aggregation have damaging effects on neurons, activation of the Nrf2 signaling should be beneficial against neurodegeneration. In this study, we report a novel synthetic morpholine-containing chalcone KMS99220 that confers neuroprotection. It showed high binding affinity to the Nrf2 inhibitory protein Keap-1 and increased nuclear translocation of Nrf2 and gene expression of the antioxidant enzymes heme oxygenase-1, NAD(P)H:quinone oxidoreductase-1, and the catalytic and modifier subunits of glutamate-cysteine ligase in dopaminergic CATH...
November 17, 2017: Neurochemistry International
https://www.readbyqxmd.com/read/29157948/neurturin-is-a-pgc-1%C3%AE-1-controlled-myokine-that-promotes-motor-neuron-recruitment-and-neuromuscular-junction-formation
#6
Richard Mills, Hermes Taylor-Weiner, Jorge C Correia, Leandro Z Agudelo, Ilary Allodi, Christina Kolonelou, Vicente Martinez-Redondo, Duarte M S Ferreira, Susanne Nichterwitz, Laura H Comley, Vanessa Lundin, Eva Hedlund, Jorge L Ruas, Ana I Teixeira
OBJECTIVE: We examined whether skeletal muscle overexpression of PGC-1α1 or PGC-1α4 affected myokine secretion and neuromuscular junction (NMJ) formation. METHODS: A microfluidic device was used to model endocrine signaling and NMJ formation between primary mouse myoblast-derived myotubes and embryonic stem cell-derived motor neurons. Differences in hydrostatic pressure allowed for fluidic isolation of either cell type or unidirectional signaling in the fluid phase...
November 7, 2017: Molecular Metabolism
https://www.readbyqxmd.com/read/29157414/synchronizing-the-tracking-eye-movements-with-the-motion-of-a-visual-target-basic-neural-processes
#7
Laurent Goffart, Clara Bourrelly, Julie Quinet
In primates, the appearance of an object moving in the peripheral visual field elicits an interceptive saccade that brings the target image onto the foveae. This foveation is then maintained more or less efficiently by slow pursuit eye movements and subsequent catch-up saccades. Sometimes, the tracking is such that the gaze direction looks spatiotemporally locked onto the moving object. Such a spatial synchronism is quite spectacular when one considers that the target-related signals are transmitted to the motor neurons through multiple parallel channels connecting separate neural populations with different conduction speeds and delays...
2017: Progress in Brain Research
https://www.readbyqxmd.com/read/29157304/a-novel-vector-for-transgenesis-in-the-rat-cns
#8
T Peter Lopez, Kurt Giles, Brittany N Dugger, Abby Oehler, Carlo Condello, Zuzana Krejciova, Julian A Castaneda, George A Carlson, Stanley B Prusiner
The larger brain of the rat enables a much greater repertoire of complex behaviors than mice, likely making rats preferential for investigating neurodegeneration. Because molecular tools for specific expression of transgenes in the rat brain are sparse, we chose Prnp encoding the prion protein (PrP) to develop a novel vector to drive transgene expression in the rat brain. We compared the rat Prnp sequence with mouse and Syrian hamster Prnp sequences, identifying conserved genetic elements and hypothesizing that these elements would be able to drive neuronal transgene expression...
November 21, 2017: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/29156920/supportive-palliative-interventions-in-motor-neurone-disease-what-we-know-from-current-literature
#9
Hon Wai Benjamin Cheng, Kwok Ying Chan, Yuen Kwan Judy Chung, Chun Wai Choi, Chun Hung Chan, Shuk Ching Cheng, Wan Hung Chan, Koon Sim Fung, Kar Yin Wong, Oi Man Iman Chan, Ching Wah Man
Although there is no cure for motor neurone disease (MND), the advent of supportive interventions including multidisciplinary care (MDC) has improved treatment interventions and enhanced quality of life (QOL) for MND patients and their carers. Our integrative review showed evidence-based MDC, respiratory management and disease-modifying therapy that have improved the outcomes of patients diagnosed with MND. Supportive approaches to nutritional maintenance and optimization of symptomatic treatments, including management of communication and neuropsychiatric issues, improve the QOL for MND patients...
October 31, 2017: Annals of Palliative Medicine
https://www.readbyqxmd.com/read/29156917/advance-care-planning-for-patients-with-advanced-neurology-diseases
#10
Ka-Chi Cheung, Vikki Wai-Kee Lau, Ka-Chun Un, Man-Sheung Wong, Kwok-Ying Chan
BACKGROUND: Advanced neurology diseases including motor neuron disease (MND) are usually progressive life-limiting illness and could be devastating for patients, families and caregivers. Although medical technologies, such as enteral feeding and non-invasive ventilation, may prolong life expectancy of the patients, their utilization prompts important ethical questions in regard to their quality of life (QoL). Little attention had been paid on how ACP practice would practically help with patients suffering from different neurology diseases...
October 13, 2017: Annals of Palliative Medicine
https://www.readbyqxmd.com/read/29156906/the-development-of-neurology-palliative-care-service-for-motor-neuron-disease-mnd-patients-hong-kong-experience
#11
Hon Wai Benjamin Cheng, Wai Tsan Tracy Chen, Chun Kwok Angus Chu, Savio Lee, Joo Shium Lee, Yeuk Fai Hong, Yuen Kwan Judy Chung
BACKGROUND: Motor neuron disease (MND) is a neurodegenerative disease characterized by loss of motor neurons in the spinal cord, brainstem and motor cortex. Clinically it is manifested as progressive decline in physical, respiratory, swallowing and communication function and ultimately death. Traditional model of care was fragmented and did not match with patients and carers multi-facet needs. METHODS: A special workgroup for MND patients that includes neurologist, respiratory physician, rehabilitation specialist and palliative care (PC) physician was formed in Hong Kong since year 2013...
September 15, 2017: Annals of Palliative Medicine
https://www.readbyqxmd.com/read/29156412/a-system-level-mathematical-model-of-basal-ganglia-motor-circuit-for-kinematic-planning-of-arm-movements
#12
Armin Salimi-Badr, Mohammad Mehdi Ebadzadeh, Christian Darlot
In this paper, a novel system-level mathematical model of the Basal Ganglia (BG) for kinematic planning, is proposed. An arm composed of several segments presents a geometric redundancy. Thus, selecting one trajectory among an infinite number of possible ones requires overcoming redundancy, according to some kinds of optimization. Solving this optimization is assumed to be the function of BG in planning. In the proposed model, first, a mathematical solution of kinematic planning is proposed for movements of a redundant arm in a plane, based on minimizing energy consumption...
November 10, 2017: Computers in Biology and Medicine
https://www.readbyqxmd.com/read/29155814/optimal-synaptic-signaling-connectome-for-locomotory-behavior-in-caenorhabditis-elegans-design-minimizing-energy-cost
#13
Franciszek Rakowski, Jan Karbowski
The detailed knowledge of C. elegans connectome for 3 decades has not contributed dramatically to our understanding of worm's behavior. One of main reasons for this situation has been the lack of data on the type of synaptic signaling between particular neurons in the worm's connectome. The aim of this study was to determine synaptic polarities for each connection in a small pre-motor circuit controlling locomotion. Even in this compact network of just 7 neurons the space of all possible patterns of connection types (excitation vs...
November 20, 2017: PLoS Computational Biology
https://www.readbyqxmd.com/read/29155768/slice-patch-clamp-technique-for-analyzing-learning-induced-plasticity
#14
Hiroyuki Kida, Yuya Sakimoto, Dai Mitsushima
The slice patch clamp technique is a powerful tool for investigating learning-induced neural plasticity in specific brain regions. To analyze motor-learning induced plasticity, we trained rats using an accelerated rotor rod task. Rats performed the task 10 times at 30-s intervals for 1 or 2 days. Performance was significantly improved on the training days compared to the first trial. We then prepared acute brain slices of the primary motor cortex (M1) in untrained and trained rats. Current-clamp analysis showed dynamic changes in resting membrane potential, spike threshold, afterhyperpolarization, and membrane resistance in layer II/III pyramidal neurons...
November 11, 2017: Journal of Visualized Experiments: JoVE
https://www.readbyqxmd.com/read/29155749/immobilization-of-caenorhabditis-elegans-to-analyze-intracellular-transport-in-neurons
#15
Shinsuke Niwa
Axonal transport and intraflagellar transport (IFT) are essential for axon and cilia morphogenesis and function. Kinesin superfamily proteins and dynein are molecular motors that regulate anterograde and retrograde transport, respectively. These motors use microtubule networks as rails. Caenorhabditis elegans (C. elegans) is a powerful model organism to study axonal transport and IFT in vivo. Here, I describe a protocol to observe axonal transport and IFT in living C. elegans. Transported cargo can be visualized by tagging cargo proteins using fluorescent proteins such as green fluorescent protein (GFP)...
October 18, 2017: Journal of Visualized Experiments: JoVE
https://www.readbyqxmd.com/read/29155276/cortical-plasticity-as-a-basis-of-phantom-limb-pain-fact-or-fiction
#16
J Andoh, C Milde, J W Tsao, H Flor
Cortical reorganization has been proposed as a major factor involved in phantom pain with prior nociceptive input to the deafferented region and input from the non-deafferented cortex creating neuronal activity that is perceived as phantom pain. There is substantial evidence that these processes play a role in neuropathic pain, although causal evidence is lacking. Recently it has been suggested that a maintenance of the cortical representation of the former hand area is related to phantom pain. Although interesting, evidence for this process is so far scarce...
November 16, 2017: Neuroscience
https://www.readbyqxmd.com/read/29155254/association-of-riluzole-and-dantrolene-improves-significant-recovery-after-acute-spinal-cord-injury-in-rats
#17
Bernardo De Caro Martins, Bruno Benetti Junta Torres, Karen Maciel de Oliveira, Mario Sergio Lavor, Carla Maria Osório, Fabiola Bono Fukushima, Isabel Rodrigues Rosado, Eliane Gonçalves de Melo
BACKGROUND CONTEXT: Damage to the spinal cord can result in irreversible impairment or complete loss of motor, sensory, and autonomic functions. Riluzole and dantrolene have been shown to provide neuroprotection by reducing neuronal apoptosis after brain and spinal cord injury (SCI) in several animal models of neurological disorders. As these drugs protect the injured spinal cord through different mechanisms, we investigated the cumulative effects of riluzole and dantrolene. PURPOSE: This study aimed to investigate the neuroprotective efficacy of the combined administration of riluzole and dantrolene in experimental thoracic SCI...
November 15, 2017: Spine Journal: Official Journal of the North American Spine Society
https://www.readbyqxmd.com/read/29155221/motor-axon-guidance-in-drosophila
#18
REVIEW
Aref Arzan Zarin, Juan-Pablo Labrador
The Drosophila motor system starts to assemble during embryonic development. It is composed of 30 muscles per abdominal hemisegment and 36 motor neurons assembling into nerve branches to exit the CNS, navigate within the muscle field and finally establish specific connections with their target muscles. Several families of guidance molecules that play a role controlling this process as well as transcriptional regulators that program the behavior of specific motoneurons have been identified. In this review we summarize the role of both groups of molecules in the motor system as well as their relationship where known...
November 16, 2017: Seminars in Cell & Developmental Biology
https://www.readbyqxmd.com/read/29155115/transtentorial-distortion-syndrome-a-consistent-complication-following-lateral-and-fourth-ventricular-shunting-in-adults
#19
Claudia L Craven, Irene Baudracco, Simon D Thompson, Lewis Thorne, Laurence D Watkins, Ahmed K Toma
BACKGROUND: Complex hydrocephalus affecting lateral and fourth ventricles separately is occasionally managed with CSF diversion via supra- and infra-tentorial ventricular catheters. The optimal configuration to reduce complications is currently unknown in adults. We describe a consistently similar clinical presentation of patients with complex hydrocephalus and a fourth ventricle separately drained by infra-tentorial shunt insertion. METHODS: A retrospective single centre case series...
November 15, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/29154925/membrane-cholesterol-depletion-in-cortical-neurons-highlights-altered-nmda-receptor-functionality-in-a-mouse-model-of-amyotrophic-lateral-sclerosis
#20
Alessia Antonini, Silvia Caioli, Luana Saba, Giulia Vindigni, Silvia Biocca, Nadia Canu, Cristina Zona
Amyotrophic Lateral Sclerosis (ALS) is a chronic neurodegenerative disease affecting upper and lower motor neurons, with unknown aetiology. Lipid rafts, cholesterol enriched microdomains of the plasma membrane, have been linked to neurodegenerative disorders like ALS. The NMDA-receptor subcellular localization in lipid rafts is known to play many roles, from modulating memory strength to neurotoxicity. In this study, performed on the widely used G93A mouse model of ALS, we have shown an equal content of total membrane cholesterol in Control and G93A cortical cultures...
November 14, 2017: Biochimica et Biophysica Acta
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