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https://www.readbyqxmd.com/read/28804448/erythropoietin-protects-against-lipopolysaccharide-induced-microgliosis-and-abnormal-granule-cell-development-in-the-ovine-fetal-cerebellum
#1
Annie R A McDougall, Nadia Hale, Sandra Rees, Richard Harding, Robert De Matteo, Stuart B Hooper, Mary Tolcos
Erythropoietin (EPO) ameliorates inflammation-induced injury in cerebral white matter (WM). However, effects of inflammation on the cerebellum and neuroprotective effects of EPO are unknown. Our aims were to determine: (i) whether lipopolysaccharide (LPS)-induced intrauterine inflammation causes injury to, and/or impairs development of the cerebellum; and (ii) whether recombinant human EPO (rhEPO) mitigates these changes. At 107 ± 1 days gestational age (DGA; ~0.7 of term), fetal sheep received LPS (~0.9 μg/kg; i...
2017: Frontiers in Cellular Neuroscience
https://www.readbyqxmd.com/read/28803710/intrathecal-2-hydroxypropyl-%C3%AE-cyclodextrin-decreases-neurological-disease-progression-in-niemann-pick-disease-type-c1-a-non-randomised-open-label-phase-1-2-trial
#2
Daniel S Ory, Elizabeth A Ottinger, Nicole Yanjanin Farhat, Kelly A King, Xuntian Jiang, Lisa Weissfeld, Elizabeth Berry-Kravis, Cristin D Davidson, Simona Bianconi, Lee Ann Keener, Ravichandran Rao, Ariane Soldatos, Rohini Sidhu, Kimberly A Walters, Xin Xu, Audrey Thurm, Beth Solomon, William J Pavan, Bernardus N Machielse, Mark Kao, Steven A Silber, John C McKew, Carmen C Brewer, Charles H Vite, Steven U Walkley, Christopher P Austin, Forbes D Porter
BACKGROUND: Niemann-Pick disease, type C1 (NPC1) is a lysosomal storage disorder characterised by progressive neurodegeneration. In preclinical testing, 2-hydroxypropyl-β-cyclodextrins (HPβCD) significantly delayed cerebellar Purkinje cell loss, slowed progression of neurological manifestations, and increased lifespan in mouse and cat models of NPC1. The aim of this study was to assess the safety and efficacy of lumbar intrathecal HPβCD. METHODS: In this open-label, dose-escalation phase 1-2a study, we gave monthly intrathecal HPβCD to participants with NPC1 with neurological manifestation at the National Institutes of Health (NIH), Bethesda, MD, USA...
August 10, 2017: Lancet
https://www.readbyqxmd.com/read/28800926/corrigendum-to-expression-of-mutant-disc1-in-purkinje-cells-increases-their-spontaneous-activity-and-impairs-cognitive-and-social-behaviors-in-mice-neurobiol-dis-103-2017-144-153
#3
Alexey V Shevelkin, Chantelle E Terrillion, Bagrat N Abazyan, Tymoteusz J Kajstura, Yan A Jouroukhin, Gay L Rudow, Juan C Troncoso, David J Linden, Mikhail V Pletnikov
No abstract text is available yet for this article.
August 8, 2017: Neurobiology of Disease
https://www.readbyqxmd.com/read/28795396/synaptic-excitation-by-climbing-fibre-collaterals-in-the-cerebellar-nuclei-of-juvenile-and-adult-mice
#4
Marion Najac, Indira M Raman
The inferior olive conveys instructive signals to the cerebellum that drive sensorimotor learning. Inferior olivary neurons transmit their signals via climbing fibres, which powerfully excite Purkinje cells, evoking complex spikes and depressing parallel fibre synapses. Additionally, however, these climbing fibres send collaterals to the cerebellar nuclei (CbN). In vivo and in vitro data suggest that climbing fibre collateral excitation is weak in adult mice, raising the question of whether the primary role of this pathway may be developmental...
August 10, 2017: Journal of Physiology
https://www.readbyqxmd.com/read/28794450/serial-processing-of-kinematic-signals-by-cerebellar-circuitry-during-voluntary-whisking
#5
Susu Chen, George J Augustine, Paul Chadderton
Purkinje cells (PCs) in Crus 1 represent whisker movement via linear changes in firing rate, but the circuit mechanisms underlying this coding scheme are unknown. Here we examine the role of upstream inputs to PCs-excitatory granule cells (GCs) and inhibitory molecular layer interneurons-in processing of whisking signals. Patch clamp recordings in GCs reveal that movement is accompanied by changes in mossy fibre input rate that drive membrane potential depolarisation and high-frequency bursting activity at preferred whisker angles...
August 10, 2017: Nature Communications
https://www.readbyqxmd.com/read/28793271/loss-of-nav%C3%AE-4-mediated-regulation-of-sodium-currents-in-adult-purkinje-neurons-disrupts-firing-and-impairs-motor-coordination-and-balance
#6
Joseph L Ransdell, Edward Dranoff, Brandon Lau, Wan-Lin Lo, David L Donermeyer, Paul M Allen, Jeanne M Nerbonne
No abstract text is available yet for this article.
August 8, 2017: Cell Reports
https://www.readbyqxmd.com/read/28791574/heterotopic-purkinje-cells-a-comparative-postmortem-study-of-essential-tremor-and-spinocerebellar-ataxias-1-2-3-and-6
#7
Elan D Louis, Sheng-Han Kuo, William J Tate, Geoffrey C Kelly, Jesus Gutierrez, Etty P Cortes, Jean-Paul G Vonsattel, Phyllis L Faust
Essential tremor (ET) is among the most common neurological diseases. Postmortem studies have noted a series of pathological changes in the ET cerebellum. Heterotopic Purkinje cells (PCs) are those whose cell body is mis-localized in the molecular layer. In neurodegenerative settings, these are viewed as a marker of the progression of neuronal degeneration. We (1) quantify heterotopias in ET cases vs. controls, (2) compare ET cases to other cerebellar degenerative conditions (spinocerebellar ataxias (SCAs) 1, 2, 3, and 6), (3) compare these SCAs to one another, and (4) assess heterotopia within the context of associated PC loss in each disease...
August 8, 2017: Cerebellum
https://www.readbyqxmd.com/read/28790895/brain-metal-distribution-and-neuro-inflammatory-profiles-after-chronic-vanadium-administration-and-withdrawal-in-mice
#8
Oluwabusayo R Folarin, Amanda M Snyder, Douglas G Peters, Funmilayo Olopade, James R Connor, James O Olopade
Vanadium is a potentially toxic environmental pollutant and induces oxidative damage in biological systems including the central nervous system (CNS). Its deposition in brain tissue may be involved in the pathogenesis of certain neurological disorders which after prolonged exposure can culminate into more severe pathology. Most studies on vanadium neurotoxicity have been done after acute exposure but in reality some populations are exposed for a lifetime. This work was designed to ascertain neurodegenerative consequences of chronic vanadium administration and to investigate the progressive changes in the brain after withdrawal from vanadium treatment...
2017: Frontiers in Neuroanatomy
https://www.readbyqxmd.com/read/28775326/retrograde-bdnf-to-trkb-signaling-promotes-synapse-elimination-in-the-developing-cerebellum
#9
Myeongjeong Choo, Taisuke Miyazaki, Maya Yamazaki, Meiko Kawamura, Takanobu Nakazawa, Jianling Zhang, Asami Tanimura, Naofumi Uesaka, Masahiko Watanabe, Kenji Sakimura, Masanobu Kano
Elimination of early-formed redundant synapses during postnatal development is essential for functional neural circuit formation. Purkinje cells (PCs) in the neonatal cerebellum are innervated by multiple climbing fibers (CFs). A single CF is strengthened whereas the other CFs are eliminated in each PC dependent on postsynaptic activity in PC, but the underlying mechanisms are largely unknown. Here, we report that brain-derived neurotrophic factor (BDNF) from PC facilitates CF synapse elimination. By PC-specific deletion of BDNF combined with knockdown of BDNF receptors in CF, we show that BDNF acts retrogradely on TrkB in CFs, and facilitates elimination of CF synapses from PC somata during the third postnatal week...
August 4, 2017: Nature Communications
https://www.readbyqxmd.com/read/28774570/expression-of-the-glua2-subunit-of-glutamate-receptors-is-required-for-the-normal-dendritic-differentiation-of-cerebellar-purkinje-cells
#10
Masahiko Tanaka, Tomomi Senda, Naohide Hirashima
Cerebellar Purkinje cells differentiate the most elaborate dendritic trees among neurons in the brain and constitute the principal part of cerebellar neuronal circuitry. In the present study, we examined the role of the GluA2 subunit of α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid (AMPA)-type glutamate receptors in the dendritic differentiation of Purkinje cells. Since mature Purkinje cells express the GluA2 subunit, AMPA receptors on them exhibit a low Ca(2+) permeability. Does this expression of GluA2, leading to the loss of Ca(2+) permeability of AMPA receptors, have a positive significance in the dendritic differentiation of Purkinje cells? To answer this question, we introduced GluA2 siRNA into immature Purkinje cells in cerebellar cell cultures using a single-cell electroporation technique...
July 31, 2017: Neuroscience Letters
https://www.readbyqxmd.com/read/28766957/immunohistochemical-detection-of-c9orf72-protein-in-frontotemporal-lobar-degeneration-and-motor-neurone-disease-patterns-of-immunostaining-and-an-evaluation-of-commercial-antibodies
#11
Yvonne S Davidson, Andrew C Robinson, Sara Rollinson, Stuart Pickering-Brown, Shangxi Xiao, Janice Robertson, David M A Mann
We have employed as 'gold standards' two in-house, well-characterised and validated polyclonal antibodies, C9-L and C9-S, which detect the longer and shorter forms of C9orf72, and have compared seven other commercially available antibodies with these in order to evaluate the utility of the latter as credible tools for the demonstration of C9orf72. C9-L and C9-S antibodies immunostained cytoplasmic 'speckles', and the nuclear membrane, respectively, in cerebellar Purkinje cells of the cerebellum in patients with behavioural variant frontotemporal dementia (bvFTD) with amyotrophic lateral sclerosis (ALS), and in patients with ALS alone...
August 2, 2017: Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration
https://www.readbyqxmd.com/read/28750785/cerebellar-cortical-abiotrophy-in-young-labrador-retrievers
#12
Chandreyee Sen, Ashwani Kumar Sharma, Charanjit Singh Randhawa, Kuldip Gupta
Cerebellar abiotrophy is a hereditary degenerative disorder of the central nervous system in humans and animals. Four male and one female Labrador-retriever pups were presented with clinical signs of head swaying with intention tremor, loss of target oriented movement, wide base stance, forelimb hypermetria, and hindlimb ataxia leading to falling off during ambulation. On histopathologic examination of cerebellum, cerebellar cortical abiotrophy was confirmed with extensive loss of Purkinje cells, diminution of granular layer, relative thickening and foliar gliosis in white matter...
March 2017: Topics in Companion Animal Medicine
https://www.readbyqxmd.com/read/28746835/genetic-ataxia-telangiectasia-porcine-model-phenocopies-the-multisystemic-features-of-the-human-disease
#13
Rosanna Beraldi, David K Meyerholz, Alexei Savinov, Attila D Kovács, Jill M Weimer, Jordan A Dykstra, Ryan D Geraets, David A Pearce
Ataxia telangiectasia (AT) is a progressive multisystem autosomal recessive disorder caused by mutations in the AT-mutated (ATM) gene. Early onset AT in children is characterized by cerebellar degeneration, leading to motor impairment. Lung disease and cancer are the two most common causes of death in AT patients. Accelerated thymic involution may contribute to the cancer, and recurrent and/or chronic respiratory infections may be a contributing factor to lung disease in AT. AT patients have fertility issues, are highly sensitive to ionizing radiation and they present oculocutaneous telangiectasia...
July 23, 2017: Biochimica et Biophysica Acta
https://www.readbyqxmd.com/read/28746384/detection-of-brain-directed-autoantibodies-in-the-serum-of-non-small-cell-lung-cancer-patients
#14
Manoj Banjara, Chaitali Ghosh, Aaron Dadas, Peter Mazzone, Damir Janigro
Antibodies against brain proteins were identified in the plasma of cancer patients and are defined to cause paraneoplastic neurological syndromes. The profiles of brain-directed antibodies in non-small cell lung cancer (NSCLC) are largely unknown. Here, for the first time, we compared autoantibodies against brain proteins in NSCLC (n = 18) against those present in age-matched non-cancer control subjects (n = 18) with a similar life-style, habit, and medical history. Self-recognizing immunoglobulin (IgG) are primarily directed against cells in the cortex (P = 0...
2017: PloS One
https://www.readbyqxmd.com/read/28738819/increased-biological-activity-of-protein-kinase-c-gamma-is-not-required-in-spinocerebellar-ataxia-14
#15
Etsuko Shimobayashi, Josef P Kapfhammer
Spinocerebellar ataxia (SCA) is an autosomal dominant neurodegenerative disorder characterized by slowly progressive cerebellar dysfunction. Currently, 42 SCA types are known, some of which are caused by CAG repeat expansions, but others are caused by point mutations or deletions. Spinocerebellar ataxia type 14 (SCA14) is caused by missense mutations or deletions in the PRKCG gene, coding for protein kinase C gamma (PKCγ). It is still not well understood how these mutations eventually cause Purkinje cell dysfunction and death...
July 24, 2017: Molecular Brain
https://www.readbyqxmd.com/read/28736708/usefulness-of-a-multi-spline-duodecapolar-catheter-with-smaller-electrodes-and-closer-spacings-for-mapping-and-ablation-of-purkinje-related-premature-ventricular-contraction-and-triggered-ventricular-fibrillation
#16
Susumu Takase, Yasushi Mukai, Kensuke Ohe, Akiko Chishaki, Hideki Tashiro, Hiroyuki Tsutsui
No abstract text is available yet for this article.
May 2017: HeartRhythm Case Reports
https://www.readbyqxmd.com/read/28733667/rtn1-and-rtn3-protein-are-differentially-associated-with-senile-plaques-in-alzheimer-s-brains
#17
Qi Shi, Yingying Ge, Wanxia He, Xiangyou Hu, Riqiang Yan
Reticulon proteins (RTNs), consisting of RTN1 to RTN4, were previously shown to interact with BACE1 by negatively modulating its secretase activity. In RTN3-null mice, RTN1 expression was slightly elevated. To understand the in vivo role of RTN1, we generated RTN1-null mice and compared the effects of RTN1 and RTN3 on BACE1 modulation. We show that RTN1 is mostly expressed by neurons and not by glial cells under normal conditions, similar to the expression of RTN3. However, RTN1 is more localized in dendrites and is an excellent marker for dendrites of Purkinje cells, while RTN3 expression is less evident in dendrites...
July 21, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28730225/purkinje-cells-pathology-in-schizophrenia-a-morphometric-approach
#18
Ioannis Asterios Mavroudis, Foivos Petrides, Marina Manani, Fotios Chatzinikolaou, Alin Stelian Ciobică, Manuela Pădurariu, Dimitrios Kazis, Samuel Ndugu Njau, Vasiliki George Costa, Stavros John Baloyannis
OBJECTIVES: Schizophrenia is a brain disorder that affects more than 21 million people worldwide. Ventricle enlargement and reduction in the volume of the temporal lobe overall and in medial temporal structures constitutes the main macroscopic findings, whilst synaptic and spinal changes as well as gliosis in the hippocampal formation, the prefrontal and the entorhinal cortex stand among cardinal microscopic findings in the schizophrenic brains. In recent years, accumulated evidence comes to light about the role of cerebellum in the pathophysiology of schizophrenia...
2017: Romanian Journal of Morphology and Embryology, Revue Roumaine de Morphologie et Embryologie
https://www.readbyqxmd.com/read/28723727/longitudinal-analysis-of-motor-symptoms-and-histopathology-in-woozy-mice-a-model-of-cerebellar-ataxia
#19
Takemitsu Hayashi, Tomoya Onozato, Isao Wanajo, Morimichi Hayashi, Hiroo Takeda, Yoshikazu Fujimori
Woozy (wz) mice develop ataxia and carry a mutation in the Sil1 gene. Homozygous wz mice have been characterized histopathologically, but no details of their motor function have been reported. In the present study, to comprehensively understand the relationship between symptomatic progression and pathological feature, we evaluated motor function and neurodegeneration with age from presymptomatic to terminal stages. We evaluated the motor function of homozygous and heterozygous wz mice aged from 5 to 71 weeks...
September 6, 2017: Neuroreport
https://www.readbyqxmd.com/read/28715135/chronic-pharmacological-blockade-of-the-na-ca-2-exchanger-modulates-the-growth-and-development-of-the-purkinje-cell-dendritic-arbor-in-mouse-cerebellar-slice-cultures
#20
Pradeep Sherkhane, Josef P Kapfhammer
The Na(+) /Ca(2+) exchanger (NCX) is a bi-directional plasma membrane antiporter involved in Ca(2+) homeostasis in eukaryotes. NCX has three isoforms, NCX1-3, and all of them are expressed in the cerebellum. Immunostaining on cerebellar slice cultures indicates that NCX is widely expressed in the cerebellum, including expression in Purkinje cells. The pharmacological blockade of the forward mode of NCX (Ca(2+) efflux mode) by Bepridil moderately inhibited growth and development of Purkinje cell dendritic arbor in cerebellar slice cultures...
July 17, 2017: European Journal of Neuroscience
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