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Purkinje cell

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https://www.readbyqxmd.com/read/28733667/rtn1-and-rtn3-protein-are-differentially-associated-with-senile-plaques-in-alzheimer-s-brains
#1
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
#2
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
#3
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...
July 18, 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
#4
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
https://www.readbyqxmd.com/read/28712329/organotypic-cultures-of-cerebellar-slices-as-a-model-to-investigate-demyelinating-disorders
#5
Frédéric Doussau, Jean-Luc Dupont, Dorine Neel, Aline Schneider, Bernard Poulain, Jean Louis Bossu
Demyelinating disorders, characterized by a chronic or episodic destruction of the myelin sheath, are a leading cause of neurological disability in young adults in western countries. Studying the complex mechanisms involved in axon myelination, demyelination and remyelination requires an experimental model preserving the neuronal networks and neuro-glial interactions. Organotypic cerebellar slice cultures appear to be the best alternative to in vivo experiments and the most commonly used model for investigating etiology or novel therapeutic strategies in multiple sclerosis...
July 17, 2017: Expert Opinion on Drug Discovery
https://www.readbyqxmd.com/read/28711710/comparison-of-the-role-of-metabotropic-glutamate-receptor-subtype-1-in-developmental-refinement-of-neuronal-connectivity-between-the-cerebellum-and-the-sensory-thalamus
#6
REVIEW
Madoka Narushima
Developmental refinement of neuronal connectivity is crucial for proper brain function. In the early phase of development, input fibers arrive at their target areas guided by specific molecular cues and form abundant immature synapses. Then, functionally important synapses are preserved and strengthened by neural activity while unnecessary synapses are eliminated. Afferent synapses in the sensory thalamus, such as from retina to lateral geniculate nucleus, and climbing fiber (CF)-Purkinje cell (PC) synapses in the cerebellum are valuable models for studying this developmental refinement of synaptic connectivity because only a limited number of input fibers innervate a given postsynaptic thalamocortical (TC) neuron or PC...
July 12, 2017: Neuroscience Research
https://www.readbyqxmd.com/read/28707805/decreased-parvalbumin-mrna-levels-in-cerebellar-purkinje-cells-in-autism
#7
Jean-Jacques Soghomonian, Kunzhong Zhang, Sujithra Reprakash, Gene J Blatt
Recent neuropathology studies in human brains indicate that several areas of the prefrontal cortex have decreased numbers of parvalbumin interneurons or decreased parvalbumin expression in Autism Spectrum disorders (ASD) [Hashemi, Ariza, Rogers, Noctor, & Martinez-Cerdeno, 2017; Zikopoulos & Barbas, ]. These data suggest that a deficit in parvalbumin may be a key neuropathology of ASD and contribute to altered GABAergic inhibition. However, it is unclear if a deficit in parvalbumin is a phenomenon that occurs in regions other than the cerebral cortex...
July 14, 2017: Autism Research: Official Journal of the International Society for Autism Research
https://www.readbyqxmd.com/read/28705318/progressive-atrial-conduction-defects-associated-with-bone-malformation-caused-by-a-connexin-45-mutation
#8
Akiko Seki, Taisuke Ishikawa, Xavier Daumy, Hiroyuki Mishima, Julien Barc, Ryo Sasaki, Kiyomasa Nishii, Kayoko Saito, Mari Urano, Seiko Ohno, Saki Otsuki, Hiroki Kimoto, Alban-Elouen Baruteau, Aurelie Thollet, Swanny Fouchard, Stéphanie Bonnaud, Philippe Parent, Yosaburo Shibata, Jean-Philippe Perrin, Hervé Le Marec, Nobuhisa Hagiwara, Sandra Mercier, Minoru Horie, Vincent Probst, Koh-Ichiro Yoshiura, Richard Redon, Jean-Jacques Schott, Naomasa Makita
BACKGROUND: Inherited cardiac conduction disease is a rare bradyarrhythmia associated with mutations in various genes that affect action potential propagation. It is often characterized by isolated conduction disturbance of the His-Purkinje system, but it is rarely described as a syndromic form. OBJECTIVES: The authors sought to identify the genetic defect in families with a novel bradyarrhythmia syndrome associated with bone malformation. METHODS: The authors genetically screened 15 European cases with genotype-negative de novo atrioventricular (AV) block and their parents by trio whole-exome sequencing, plus 31 Japanese cases with genotype-negative familial AV block or sick sinus syndrome by targeted exon sequencing of 457 susceptibility genes...
July 18, 2017: Journal of the American College of Cardiology
https://www.readbyqxmd.com/read/28701537/climbing-fibers-predict-movement-kinematics-and-performance-errors
#9
Martha L Streng, Laurentiu S Popa, Timothy J Ebner
Requisite for understanding cerebellar function is a complete characterization of the signals provided by complex spike (CS) discharge of Purkinje cells, the output neurons of the cerebellar cortex. Numerous studies have provided insights into CS function, with the most predominant view being that they are evoked by error events. However, several reports suggest that CSs encode other aspects of movements and do not always respond to errors or unexpected perturbations. Here, we evaluated CS firing during a pseudo-random manual tracking task in the monkey (Macaca mulatta)...
July 12, 2017: Journal of Neurophysiology
https://www.readbyqxmd.com/read/28700469/microarray-analysis-of-copy-number-variations-and-gene-expression-profiles-in-prostate-cancer
#10
Yuping Han, Xuefei Jin, Hongyan Li, Kaichen Wang, Ji Gao, Lide Song, Yanting Lv
BACKGROUND: This study aimed to identify potential prostate cancer (PC)-related variations in gene expression profiles. METHODS: Microarray data from the GSE21032 dataset that contained the whole-transcript and exon-level expression profile (GSE21034) and Agilent 244K array-comparative genomic hybridization data (GSE21035) were downloaded from the Gene Expression Omnibus database. Differentially expressed genes (DEGs) and copy-number variations (CNVs) were identified between PC and normal tissue samples...
July 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/28698888/modulation-of-complex-spike-duration-and-probability-during-cerebellar-motor-learning-in-visually-guided-smooth-pursuit-eye-movements-of-monkeys
#11
Yan Yang, Stephen G Lisberger
Activation of an inferior olivary neuron powerfully excites Purkinje cells via its climbing fiber input and triggers a characteristic high-frequency burst, known as the complex spike (CS). The theory of cerebellar learning postulates that the CS induces long-lasting depression of the strength of synapses from active parallel fibers onto Purkinje cells, and that synaptic depression leads to changes in behavior. Prior reports showed that a CS on one learning trial is linked to a properly timed depression of simple spikes on the subsequent trial, as well as a learned change in pursuit eye movement...
May 2017: ENeuro
https://www.readbyqxmd.com/read/28688851/characterization-of-the-dominant-inheritance-mechanism-of-episodic-ataxia-type-2
#12
Kevin Dorgans, Julie Salvi, Federica Bertaso, Ludivine Bernard, Philippe Lory, Frederic Doussau, Alexandre Mezghrani
Episodic Ataxia type 2 (EA2) is an autosomal dominant neuronal disorder linked to mutations in the Cav2.1 subunit of P/Q-type calcium channels. In vitro studies have established that EA2 mutations induce loss of channel activity and that EA2 mutants can exert a dominant negative effect, suppressing normal Cav2.1 activity through protein misfolding and trafficking defects. To date, the role of this mechanism in the disease pathogenesis is unknown because no animal model exists. To address this issue, we have generated a mouse bearing the R1497X nonsense mutation in Cav2...
July 5, 2017: Neurobiology of Disease
https://www.readbyqxmd.com/read/28685241/ascorbic-acid-ameliorates-gestational-lead-exposure-induced-developmental-alteration-in-gad67-and-c-kit-expression-in-the-rat-cerebellar-cortex
#13
Sung Min Nam, Sung Chuel Ahn, Tae-Hun Go, Jin Seok Seo, Sang-Soep Nahm, Byung-Joon Chang, Jong-Hwan Lee
In the present study, we investigated the effects of ascorbic acid on lead-exposed developing cerebellum. Female rats were divided into the following three groups: control (distilled water), lead (0.2% lead acetate), and lead plus ascorbic acid (100 mg/kg/day, 10% solution). To evaluate the effect of lead exposure and ascorbic acid treatment accurately on the cerebellar development for the gestational period, we halted further treatment with lead and ascorbic acid in the dams after delivery of the pups. Although the ascorbic acid slightly decreased the lead level in pups, lead level was still high in the group treated with lead plus ascorbic acid group compared with the control group...
July 6, 2017: Biological Trace Element Research
https://www.readbyqxmd.com/read/28677370/neuronal-vacuolation-and-spinocerebellar-degeneration-associated-with-altered-neurotransmission
#14
Aggeliki Giannakopoulou
<i>Inherited neurodegenerative disorders are debilitating diseases that occur across different species, such as the domestic dog (Canis lupus familiaris), and many are caused by mutations in the same genes as corresponding human conditions. In the present study, we report an inherited neurodegenerative condition, termed 'neuronal vacuolation and spinocerebellar degeneration' (NVSD) which affects neonatal or young dogs, mainly Rottweilers, which recently has been linked with the homozygosity for the RAB3GAP1:c...
2017: Folia Neuropathologica
https://www.readbyqxmd.com/read/28658620/downregulation-of-the-glial-glt1-glutamate-transporter-and-purkinje-cell-dysfunction-in-a-mouse-model-of-myotonic-dystrophy
#15
Géraldine Sicot, Laurent Servais, Diana M Dinca, Axelle Leroy, Cynthia Prigogine, Fadia Medja, Sandra O Braz, Aline Huguet-Lachon, Cerina Chhuon, Annie Nicole, Noëmy Gueriba, Ruan Oliveira, Bernard Dan, Denis Furling, Maurice S Swanson, Ida Chiara Guerrera, Guy Cheron, Geneviève Gourdon, Mário Gomes-Pereira
Brain function is compromised in myotonic dystrophy type 1 (DM1), but the underlying mechanisms are not fully understood. To gain insight into the cellular and molecular pathways primarily affected, we studied a mouse model of DM1 and brains of adult patients. We found pronounced RNA toxicity in the Bergmann glia of the cerebellum, in association with abnormal Purkinje cell firing and fine motor incoordination in DM1 mice. A global proteomics approach revealed downregulation of the GLT1 glutamate transporter in DM1 mice and human patients, which we found to be the result of MBNL1 inactivation...
June 27, 2017: Cell Reports
https://www.readbyqxmd.com/read/28658323/using-c-kit-to-genetically-target-cerebellar-molecular-layer-interneurons-in-adult-mice
#16
Samantha B Amat, Matthew J M Rowan, Michael A Gaffield, Audrey Bonnan, Chikako Kikuchi, Hiroki Taniguchi, Jason M Christie
The cerebellar system helps modulate and fine-tune motor action. Purkinje cells (PCs) provide the sole output of the cerebellar cortex, therefore, any cerebellar involvement in motor activity must be driven by changes in PC firing rates. Several different cell types influence PC activity including excitatory input from parallel fibers and inhibition from molecular layer interneurons (MLIs). Similar to PCs, MLI activity is driven by parallel fibers, therefore, MLIs provide feed-forward inhibition onto PCs. To aid in the experimental assessment of how molecular layer inhibition contributes to cerebellar function and motor behavior, we characterized a new knock-in mouse line with Cre recombinase expression under control of endogenous c-kit transcriptional machinery...
2017: PloS One
https://www.readbyqxmd.com/read/28655840/glutamate-transporter-glast-controls-synaptic-wrapping-by-bergmann-glia-and-ensures-proper-wiring-of-purkinje-cells
#17
Taisuke Miyazaki, Miwako Yamasaki, Kouichi Hashimoto, Kazuhisa Kohda, Michisuke Yuzaki, Keiko Shimamoto, Kohichi Tanaka, Masanobu Kano, Masahiko Watanabe
Astrocytes regulate synaptic transmission through controlling neurotransmitter concentrations around synapses. Little is known, however, about their roles in neural circuit development. Here we report that Bergmann glia (BG), specialized cerebellar astrocytes that thoroughly enwrap Purkinje cells (PCs), are essential for synaptic organization in PCs through the action of the l-glutamate/l-aspartate transporter (GLAST). In GLAST-knockout mice, dendritic innervation by the main ascending climbing fiber (CF) branch was significantly weakened, whereas the transverse branch, which is thin and nonsynaptogenic in control mice, was transformed into thick and synaptogenic branches...
July 11, 2017: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/28655644/pregnenolone-does-not-interfere-with-the-effects-of-cannabinoids-on-synaptic-transmission-in-the-cerebellum-and-the-nucleus-accumbens
#18
Anna Krohmer, Martin Brehm, Volker Auwärter, Bela Szabo
The steroid hormone pregnenolone attenuates several in vivo behavioural and somatic effects of the phytocannabinoid Δ(9)-tetrahydrocannabinol, and it was suggested that pregnenolone can protect the brain from cannabis intoxication. The primary neuronal cannabinoid action behind most of the behavioural and somatic effects of cannabinoids is presynaptic inhibition of synaptic transmission. Therefore, the hypothesis of the present study was that pregnenolone attenuates the inhibition of synaptic transmission elicited by cannabinoids...
June 24, 2017: Pharmacological Research: the Official Journal of the Italian Pharmacological Society
https://www.readbyqxmd.com/read/28649766/inferior-olivary-projection-to-the-zebrin-ii-stripes-in-lobule-ixcd-of-the-pigeon-flocculus-a-retrograde-tracing-study
#19
Douglas R Wylie, Cristian Gutierrez-Ibanez, Jeremy R Corfield, Iulia Craciun, David J Graham, Peter L Hurd
Zebrin II (ZII; a.k.a. aldolase C) is expressed heterogeneously in Purkinje cells (PCs) such that there are sagittal stripes of high expression (ZII+) interdigitated with stripes of little or no expression (ZII-). The pigeon flocculus receives visual-optokinetic information and is important for generating compensatory eye movements. It consists of 4 sagittal zones based on PC complex spike activity (CSA) in response to rotational optokinetic stimuli. There are two zones where CSA responds best to rotation about the vertical axis (VA), interdigitated with two zones where CSA responds best to rotation about an horizontal axis (HA)...
June 26, 2017: Journal of Comparative Neurology
https://www.readbyqxmd.com/read/28648497/selective-optogenetic-control-of-purkinje-cells-in-monkey-cerebellum
#20
Yasmine El-Shamayleh, Yoshiko Kojima, Robijanto Soetedjo, Gregory D Horwitz
Purkinje cells of the primate cerebellum play critical but poorly understood roles in the execution of coordinated, accurate movements. Elucidating these roles has been hampered by a lack of techniques for manipulating spiking activity in these cells selectively-a problem common to most cell types in non-transgenic animals. To overcome this obstacle, we constructed AAV vectors carrying the channelrhodopsin-2 (ChR2) gene under the control of a 1 kb L7/Pcp2 promoter. We injected these vectors into the cerebellar cortex of rhesus macaques and tested vector efficacy in three ways...
July 5, 2017: Neuron
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