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Motor neuron development

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https://www.readbyqxmd.com/read/28339401/is-huntingtin-dispensable-in-the-adult-brain
#1
Jeh-Ping Liu, Scott O Zeitlin
Huntingtin (HTT) is an essential protein during early embryogenesis and the development of the central nervous system (CNS). Conditional knock-out of mouse Huntingtin (Htt) expression in the CNS beginning during neural development, as well as reducing Htt expression only during embryonic and early postnatal stages, results in neurodegeneration in the adult brain. These findings suggest that HTT is important for the development and/or maintenance of the CNS, but they do not address the question of whether HTT is required specifically in the adult CNS for its normal functions and/or homeostasis...
March 21, 2017: Journal of Huntington's Disease
https://www.readbyqxmd.com/read/28337138/motor-skill-learning-in-an-insect-inspired-neuro-computational-control-system
#2
Eleonora Arena, Paolo Arena, Roland Strauss, Luca Patané
In nature, insects show impressive adaptation and learning capabilities. The proposed computational model takes inspiration from specific structures of the insect brain: after proposing key hypotheses on the direct involvement of the mushroom bodies (MBs) and on their neural organization, we developed a new architecture for motor learning to be applied in insect-like walking robots. The proposed model is a nonlinear control system based on spiking neurons. MBs are modeled as a nonlinear recurrent spiking neural network (SNN) with novel characteristics, able to memorize time evolutions of key parameters of the neural motor controller, so that existing motor primitives can be improved...
2017: Frontiers in Neurorobotics
https://www.readbyqxmd.com/read/28335910/management-strategies-for-cln2-disease
#3
REVIEW
Ruth E Williams, Heather R Adams, Martin Blohm, Jessica L Cohen-Pfeffer, Emily de Los Reyes, Jonas Denecke, Kristen Drago, Charlie Fairhurst, Margie Frazier, Norberto Guelbert, Szilárd Kiss, Annamaria Kofler, John A Lawson, Lenora Lehwald, Mary-Anne Leung, Svetlana Mikhaylova, Jonathan W Mink, Miriam Nickel, Renée Shediac, Katherine Sims, Nicola Specchio, Meral Topcu, Ina von Löbbecke, Andrea West, Boris Zernikow, Angela Schulz
CLN2 disease (neuronal ceroid lipofuscinosis type 2) is a rare, autosomal recessive, pediatric-onset, rapidly progressive neurodegenerative lysosomal storage disorder caused by tripeptidyl peptidase 1 (TPP1) enzyme deficiency, and is characterized by language delay, seizures, rapid cognitive and motor decline, blindness, and early death. No management guidelines exist and there is a paucity of published disease-specific evidence to inform clinical practice, which currently draws upon experience from the field of childhood neurodisability...
April 2017: Pediatric Neurology
https://www.readbyqxmd.com/read/28332824/indole-substituted-benzothiazoles-and-benzoxazoles-as-selective-and-reversible-mao-b-inhibitors-for-treatment-of-parkinson-s-disease
#4
Min-Ho Nam, Moosung Park, Hyeri Park, Youngjae Kim, Seulki Yoon, Vikram Shahaji Sawant, Ji Won Choi, Jong-Hyun Park, Ki Duk Park, Sun-Joon Min, Changjoon Justin Lee, Hyunah Choo
To develop a novel, selective, and reversible MAO-B inhibitors for safer treatment of Parkinson's disease, benzothiazole and benzoxazole derivatives with indole moiety were designed and synthesized. Most of the synthesized compounds showed inhibitory activities against MAO-B and selectivity over MAO-A. The most active compound was Compound 5b, 6-fluoro-2-(1-methyl-1H-indol-5-yl)benzo[d]thiazole with an IC50 value of 29 nM with no apparent effect on MAO-A activity at 10 M. Based on the reversibility assay, Compound 5b turned out to be a fully reversible with over 95% of recovery of enzyme activity after washout of the compound...
March 23, 2017: ACS Chemical Neuroscience
https://www.readbyqxmd.com/read/28332488/parkinson-disease
#5
REVIEW
Werner Poewe, Klaus Seppi, Caroline M Tanner, Glenda M Halliday, Patrik Brundin, Jens Volkmann, Anette-Eleonore Schrag, Anthony E Lang
Parkinson disease is the second-most common neurodegenerative disorder that affects 2-3% of the population ≥65 years of age. Neuronal loss in the substantia nigra, which causes striatal dopamine deficiency, and intracellular inclusions containing aggregates of α-synuclein are the neuropathological hallmarks of Parkinson disease. Multiple other cell types throughout the central and peripheral autonomic nervous system are also involved, probably from early disease onwards. Although clinical diagnosis relies on the presence of bradykinesia and other cardinal motor features, Parkinson disease is associated with many non-motor symptoms that add to overall disability...
March 23, 2017: Nature Reviews. Disease Primers
https://www.readbyqxmd.com/read/28331322/the-role-of-deep-brain-stimulation-in-parkinson-s-disease-an-overview-and-update-on-new-developments
#6
REVIEW
John Y Fang, Christopher Tolleson
Parkinson's disease (PD) is a progressive neurodegenerative disorder characterized by the loss of neuronal dopamine production in the brain. Oral therapies primarily augment the dopaminergic pathway. As the disease progresses, more continuous delivery of therapy is commonly needed. Deep brain stimulation (DBS) has become an effective therapy option for several different neurologic and psychiatric conditions, including PD. It currently has US Food and Drug Administration approval for PD and essential tremor, as well as a humanitarian device exception for dystonia and obsessive-compulsive disorder...
2017: Neuropsychiatric Disease and Treatment
https://www.readbyqxmd.com/read/28326550/from-neuron-to-behavior-sensory-motor-coordination-of-zebrafish-turning-behavior
#7
REVIEW
Keiko Umeda, Wataru Shoji
Recent development of optogenetics brought non-invasive neural activation in living organisms. Transparent zebrafish larva is one of the suitable animal models for this technique, which enables us to investigate neural circuits for behaviors based on a whole individual nervous system. In this article we review our recent finding that suggests sensory-motor coordination in larval zebrafish escape behavior. When water vibration stimulates mechanosensory Rohon-Beard (RB) neurons, intra-spinal reflex circuit launches contralateral trunk muscle contraction that makes rapid body curvature for turning...
March 21, 2017: Development, Growth & Differentiation
https://www.readbyqxmd.com/read/28326439/a-distinct-functional-distribution-of-%C3%AE-and-%C3%AE-motoneurons-in-the-rat-trigeminal-motor-nucleus
#8
Yukako Morita-Isogai, Hajime Sato, Mitsuru Saito, Eriko Kuramoto, Dong Xu Yin, Takeshi Kaneko, Takashi Yamashiro, Kenji Takada, Seog Bae Oh, Hiroki Toyoda, Youngnam Kang
Gamma-motoneurons (γMNs) play a crucial role in regulating isometric muscle contraction. The slow jaw-closing during mastication is one of the most functional isometric contractions, which is developed by the rank-order recruitment of alpha-motoneurons (αMNs) in a manner that reflects the size distribution of αMNs. In a mouse spinal motor nucleus, there are two populations of small and large MNs; the former was identified as a population of γMNs based on the positive expression of the transcription factor estrogen-related receptor 3 (Err3) and negative expression of the neuronal DNA-binding protein NeuN, and the latter as that of αMNs based on the opposite pattern of immunoreactivity...
March 22, 2017: Brain Structure & Function
https://www.readbyqxmd.com/read/28324946/characterization-of-motor-cortex-activity-of-the-lower-limb-region
#9
Fadi Mikhail, Karthikeyan Balasubramanian, Matthew D Best, Kazutaka Takahashi, Nicholas G Hatsopoulos
Understanding the functional properties of the motor cortex is a key step to developing effective neuroprosthetic and computer interfaces for amputees and patients with spinal cord injury. Most efforts have been directed towards studying the upper limb region of the motor cortex. Little has been done in the way of addressing lower limb motor cortical physiology. In this study, we implanted multiple microelectrode arrays along the central sulcus in the primary motor cortex of a macaque monkey. Using intracortical microstimulation, we identified neurons that evoked leg movements, either exclusively or mostly, and we studied their firing activity during a center-out arm movement task...
August 2016: Conference Proceedings: Annual International Conference of the IEEE Engineering in Medicine and Biology Society
https://www.readbyqxmd.com/read/28322427/the-neuronal-correlates-of-mirror-illusion-in-children-with-spastic-hemiparesis-a-study-with-functional-magnetic-resonance-imaging
#10
Christian Weisstanner, Stefanie Saxer, Roland Wiest, Alain Kaelin-Lang, Christopher J Newman, Maja Steinlin, Sebastian Grunt
AIM: To investigate the neuronal activation pattern underlying the effects of mirror illusion in children/adolescents with normal motor development and in children/adolescents with hemiparesis and preserved contralateral corticospinal organisation. METHOD: The type of cortical reorganisation was classified according to results of transcranial magnetic stimulation. Only subjects with congenital lesions and physiological contralateral cortical reorganisation were included...
March 21, 2017: Swiss Medical Weekly
https://www.readbyqxmd.com/read/28321186/brain-activity-during-mental-imagery-of-gait-versus-gait-like-plantar-stimulation-a-novel-combined-functional-mri-paradigm-to-better-understand-cerebral-gait-control
#11
Matthieu Labriffe, Cédric Annweiler, Liubov E Amirova, Guillemette Gauquelin-Koch, Aram Ter Minassian, Louis-Marie Leiber, Olivier Beauchet, Marc-Antoine Custaud, Mickaël Dinomais
Human locomotion is a complex sensorimotor behavior whose central control remains difficult to explore using neuroimaging method due to technical constraints, notably the impossibility to walk with a scanner on the head and/or to walk for real inside current scanners. The aim of this functional Magnetic Resonance Imaging (fMRI) study was to analyze interactions between two paradigms to investigate the brain gait control network: (1) mental imagery of gait, and (2) passive mechanical stimulation of the plantar surface of the foot with the Korvit boots...
2017: Frontiers in Human Neuroscience
https://www.readbyqxmd.com/read/28320941/potent-neuroprotection-after-stroke-afforded-by-a-double-knot-spider-venom-peptide-that-inhibits-acid-sensing-ion-channel-1a
#12
Irène R Chassagnon, Claudia A McCarthy, Yanni K-Y Chin, Sandy S Pineda, Angelo Keramidas, Mehdi Mobli, Vi Pham, T Michael De Silva, Joseph W Lynch, Robert E Widdop, Lachlan D Rash, Glenn F King
Stroke is the second-leading cause of death worldwide, yet there are no drugs available to protect the brain from stroke-induced neuronal injury. Acid-sensing ion channel 1a (ASIC1a) is the primary acid sensor in mammalian brain and a key mediator of acidosis-induced neuronal damage following cerebral ischemia. Genetic ablation and selective pharmacologic inhibition of ASIC1a reduces neuronal death following ischemic stroke in rodents. Here, we demonstrate that Hi1a, a disulfide-rich spider venom peptide, is highly neuroprotective in a focal model of ischemic stroke...
March 20, 2017: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/28320799/from-first-symptoms-to-diagnosis-of-amyotrophic-lateral-sclerosis-perspectives-of-an-irish-informal-caregiver-cohort-a-thematic-analysis
#13
Miriam Galvin, Rebecca Gaffney, Bernie Corr, Iain Mays, Orla Hardiman
OBJECTIVES: Informal caregivers play an integral part in the management of amyotrophic lateral sclerosis (ALS). The objective of this study was to explore the journey from first problem symptoms to diagnosis from the perspective of informal caregivers providing care to people with ALS. DESIGN: As part of a semistructured interview, information was collected on a range of caregiver demographic details, and from an open-ended question their experiences of the time of symptom onset to diagnosis...
March 20, 2017: BMJ Open
https://www.readbyqxmd.com/read/28318364/defective-retrograde-transport-impairs-autophagic-clearance-in-alzheimer-disease-neurons
#14
Prasad Tammineni, Qian Cai
Macroautophagy/autophagy plays a key role in cellular quality control by eliminating protein aggregates and damaged organelles, which is essential for the maintenance of neuronal homeostasis. Defective autophagy has been implicated in the pathogenesis of Alzheimer disease (AD). In AD brains, autophagic vacuoles (AVs) accumulate massively within dystrophic neurites. This raises a fundamental question as to whether impaired autophagic clearance contributes to AD-associated autophagic stress. We recently revealed that AD neurons display defective retrograde transport and accumulation of amphisomes predominantly in axons and presynaptic terminals...
February 28, 2017: Autophagy
https://www.readbyqxmd.com/read/28315884/complementary-feeding-micronutrients-and-developmental-outcomes-of-children
#15
Jacqueline F Gould
The period of complementary feeding (6-24 months of age) can be a challenging and vulnerable time for infant nutrition due to disproportionately high requirements for metabolic processes, rapid developmental processes, and limited gastric capacity. This is a period of crucial brain development where high caloric intake is necessary to allow synaptogenesis (creation of channels between neurons for communication), and maintenance of established synapses, myelination (laying the myelin sheath around neuronal axons) and everyday psychological functioning...
2017: Nestlé Nutrition Institute Workshop Series
https://www.readbyqxmd.com/read/28315294/neuronal-cell-sheet-of-cortical-motor-neuron-phenotype-derived-from-human-ips-cells
#16
Noboru Suzuki, Nagisa Arimitsu, Jun Shimizu, Kenji Takai, Chieko Hirotsu, Erika Takada, Yuji Ueda, Sueshige Wakisaka, Naruyoshi Fujiwara, Tomoko Suzuki
<p>Transplantation of stem cells which differentiate into more mature neural cells brings about functional improvement in pre-clinical studies of stroke. Previous transplant approaches in diseased brain have utilized injection of the cells in a cell suspension. In addition, neural stem cells were preferentially used as graft. However, these cells had no specific relationship to the damaged tissue of stroke patients and brain injury. The injection of cells in a suspension destroyed the cell-cell interactions that are suggested to be important for promoting functional integrity as cortical motor neurons...
March 17, 2017: Cell Transplantation
https://www.readbyqxmd.com/read/28302159/a-data-driven-approach-links-microglia-to-pathology-and-prognosis-in-amyotrophic-lateral-sclerosis
#17
Johnathan Cooper-Knock, Claire Green, Gabriel Altschuler, Wenbin Wei, Joanna J Bury, Paul R Heath, Matthew Wyles, Catherine Gelsthorpe, J Robin Highley, Alejandro Lorente-Pons, Tim Beck, Kathryn Doyle, Karel Otero, Bryan Traynor, Janine Kirby, Pamela J Shaw, Winston Hide
Amyotrophic lateral sclerosis (ALS) is a devastating neurodegenerative disease that lacks a predictive and broadly applicable biomarker. Continued focus on mutation-specific upstream mechanisms has yet to predict disease progression in the clinic. Utilising cellular pathology common to the majority of ALS patients, we implemented an objective transcriptome-driven approach to develop noninvasive prognostic biomarkers for disease progression. Genes expressed in laser captured motor neurons in direct correlation (Spearman rank correlation, p < 0...
March 16, 2017: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/28301599/exploring-the-role-of-locomotor-sensitization-in-the-circadian-food-entrainment-pathway
#18
Hanna Opiol, Nuria de Zavalia, Tara Delorme, Pavel Solis, Spencer Rutherford, Uri Shalev, Shimon Amir
Food entrainment is the internal mechanism whereby the phase and period of circadian clock genes comes under the control of daily scheduled food availability. Food entrainment allows the body to efficiently realign the internal timing of behavioral and physiological functions such that they anticipate food intake. Food entrainment can occur with or without caloric restriction, as seen with daily schedules of restricted feeding (RF) or restricted treat (RT) that restrict food or treat intake to a single feeding time...
2017: PloS One
https://www.readbyqxmd.com/read/28299654/roles-of-runx-genes-in-nervous-system-development
#19
Jae Woong Wang, Stefano Stifani
Runt-related (Runx) transcription factors play essential roles during development and adult tissue homeostasis and are responsible for several human diseases. They regulate a variety of biological mechanisms in numerous cell lineages. Recent years have seen significant progress in our understanding of the functions performed by Runx proteins in the developing and postnatal mammalian nervous system. In both central and peripheral nervous systems, Runx1 and Runx3 display remarkably specific expression in mostly non-overlapping groups of postmitotic neurons...
2017: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/28290135/spinal-cord-injury-impairs-neurogenesis-and-induces-glial-reactivity-in-the-hippocampus
#20
Ignacio Jure, Luciana Pietranera, Alejandro F De Nicola, Florencia Labombarda
The incorporation of newborn neurons with increased synaptic remodeling and activity-dependent plasticity in the dentate gyrus enhances hippocampal-dependent learning performances. Astrocytes and microglial cells are components of the neurogenic niche and regulate neurogenesis under normal and neurophatological conditions leading to functional consequences for learning and memory. Although cognitive impairments were reported in patients after spinal cord injury (SCI), only few studies have considered remote changes in brain structures which are not related with sensory and motor cortex...
March 13, 2017: Neurochemical Research
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