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cerebellar escape

Yuan-Bo Wu, Li Zhang, Wen-Ting Li, Yi Yang, Jiang-Ming Zhao
BACKGROUND: Artesunate (ART) is an antimalarial drug with potential anti-inflammatory effect. This study aimed to explore the potential protective role of ART in hepatic encephalopathy (HE), involving its function against ammonia toxicity. METHODS: HE rats were induced by the administration of thioacetamide (TAA, 300mg/kg/day). Spatial learning ability was tested in both Morris water and eight-arm radial maze. Rat cerebellar granule neurons (CGNs) were prepared for ammonia treatment in vitro, in line with SH-SY5Y and C6 cells...
October 17, 2016: Biomedicine & Pharmacotherapy, Biomédecine & Pharmacothérapie
Mary F Barbe, Jessica J Krueger, Regina Loomis, Jessica Otte, Jennifer Gordon
Pur-alpha is a highly conserved sequence-specific DNA and RNA binding protein with established roles in DNA replication, RNA translation, cell cycle regulation, and maintenance of neuronal differentiation. Prior studies have shown that mice lacking Pur-alpha (-/-) display decreased neurogenesis and impaired neuronal differentiation. We sought to examine for the first time, the behavioral phenotype and brain histopathology of mice that are heterozygous (+/-) for Pur-alpha. Standardized behavioral phenotyping revealed a decreased escape response to touch, limb and abdominal hypotonia, and gait abnormalities in heterozygous Pur-alpha (+/-) mice, compared to wild-type (+/+) littermates...
September 17, 2016: Neuroscience
Gemma Huguet, Elisabet Kadar, Yasin Temel, Lee Wei Lim
The electrical stimulation of specific brain targets has been shown to induce striking antidepressant effects. Despite that recent data have indicated that cerebellum is involved in emotional regulation, the mechanisms by which stimulation improved mood-related behaviors in the cerebellum remained largely obscure. Here, we investigated the stimulation effects of the ventromedial prefrontal cortex (vmPFC), nucleus accumbens (NAc), and lateral habenular nucleus on the c-Fos neuronal activity in various deep cerebellar and vestibular nuclei using the unpredictable chronic mild stress (CMS) animal model of depression...
July 19, 2016: Cerebellum
R Abeti, M H Parkinson, I P Hargreaves, P R Angelova, C Sandi, M A Pook, P Giunti, A Y Abramov
Friedreich's ataxia (FRDA) is an inherited neurodegenerative disease. The mutation consists of a GAA repeat expansion within the FXN gene, which downregulates frataxin, leading to abnormal mitochondrial iron accumulation, which may in turn cause changes in mitochondrial function. Although, many studies of FRDA patients and mouse models have been conducted in the past two decades, the role of frataxin in mitochondrial pathophysiology remains elusive. Are the mitochondrial abnormalities only a side effect of the increased accumulation of reactive iron, generating oxidative stress? Or does the progressive lack of iron-sulphur clusters (ISCs), induced by reduced frataxin, cause an inhibition of the electron transport chain complexes (CI, II and III) leading to reactive oxygen species escaping from oxidative phosphorylation reactions? To answer these crucial questions, we have characterised the mitochondrial pathophysiology of a group of disease-relevant and readily accessible neurons, cerebellar granule cells, from a validated FRDA mouse model...
2016: Cell Death & Disease
Bruna Silva-Marques, Anna Carolyna Lepesteur Gianlorenço, Rosana Mattioli
Experimental evidence suggests that the cerebellum plays a more complex role in learning than simply regulating the motor response. Rather, it is thought to play a significant role in the consolidation of emotional memory in mice. Due to the difficulty of interpreting fear and anxiety behaviors-the standard methodology for the study of the histaminergic system and emotional memory-in mice, we propose a behavioral assessment of mice subjected to the Elevated T-maze after histamine microinjection of the cerebellar vermis...
May 4, 2016: Neuroscience Letters
Tamar Harel, Gozde Yesil, Yavuz Bayram, Zeynep Coban-Akdemir, Wu-Lin Charng, Ender Karaca, Ali Al Asmari, Mohammad K Eldomery, Jill V Hunter, Shalini N Jhangiani, Jill A Rosenfeld, Davut Pehlivan, Ayman W El-Hattab, Mohammed A Saleh, Charles A LeDuc, Donna Muzny, Eric Boerwinkle, Richard A Gibbs, Wendy K Chung, Yaping Yang, John W Belmont, James R Lupski
The paradigm of a single gene associated with one specific phenotype and mode of inheritance has been repeatedly challenged. Genotype-phenotype correlations can often be traced to different mutation types, localization of the variants in distinct protein domains, or the trigger of or escape from nonsense-mediated decay. Using whole-exome sequencing, we identified homozygous variants in EMC1 that segregated with a phenotype of developmental delay, hypotonia, scoliosis, and cerebellar atrophy in three families...
March 3, 2016: American Journal of Human Genetics
Suyi Zhang, Hiroaki Mano, Gowrishankar Ganesh, Trevor Robbins, Ben Seymour
Pavlovian conditioning underlies many aspects of pain behavior, including fear and threat detection [1], escape and avoidance learning [2], and endogenous analgesia [3]. Although a central role for the amygdala is well established [4], both human and animal studies implicate other brain regions in learning, notably ventral striatum and cerebellum [5]. It remains unclear whether these regions make different contributions to a single aversive learning process or represent independent learning mechanisms that interact to generate the expression of pain-related behavior...
January 11, 2016: Current Biology: CB
Takahiro Ishikawa, Shinji Kakei, Hiroshi Mitoma
Holmes proposed not only the term ataxia, but also opposite clinical signs related to muscle recruitment, which have escaped clinical attention; (1) asthenia, representing delay in initiating muscle contraction and slowness in attaining exertion of full power, and (2) adventitiousness, representing adventitious movements. Recent physiological studies have shown that cerebellar outputs are modified by release or facilitation of Purkinje cell-mediated inhibition on dentate neurons. We believe that asthenia and adventitiousness, which correlate with deficits in the control of disinhibition and inhibition, respectively, deserve more attention in clinical examination...
2015: Cerebellum & Ataxias
Stella Koutsikou, Thomas C Watson, Jonathan J Crook, J Lianne Leith, Charlotte L Lawrenson, Richard Apps, Bridget M Lumb
UNLABELLED: The periaqueductal gray (PAG) coordinates behaviors essential to survival, including striking changes in movement and posture (e.g., escape behaviors in response to noxious stimuli vs freezing in response to fear-evoking stimuli). However, the neural circuits underlying the expression of these behaviors remain poorly understood. We demonstrate in vivo in rats that activation of the ventrolateral PAG (vlPAG) affects motor systems at multiple levels of the neuraxis through the following: (1) differential control of spinal neurons that forward sensory information to the cerebellum via spino-olivo-cerebellar pathways (nociceptive signals are reduced while proprioceptive signals are enhanced); (2) alterations in cerebellar nuclear output as revealed by changes in expression of Fos-like immunoreactivity; and (3) regulation of spinal reflex circuits, as shown by an increase in α-motoneuron excitability...
October 21, 2015: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
Stephen R F Twigg, Jennifer Forecki, Jacqueline A C Goos, Ivy C A Richardson, A Jeannette M Hoogeboom, Ans M W van den Ouweland, Sigrid M A Swagemakers, Maarten H Lequin, Daniel Van Antwerp, Simon J McGowan, Isabelle Westbury, Kerry A Miller, Steven A Wall, Peter J van der Spek, Irene M J Mathijssen, Erwin Pauws, Christa S Merzdorf, Andrew O M Wilkie
Human ZIC1 (zinc finger protein of cerebellum 1), one of five homologs of the Drosophila pair-rule gene odd-paired, encodes a transcription factor previously implicated in vertebrate brain development. Heterozygous deletions of ZIC1 and its nearby paralog ZIC4 on chromosome 3q25.1 are associated with Dandy-Walker malformation of the cerebellum, and loss of the orthologous Zic1 gene in the mouse causes cerebellar hypoplasia and vertebral defects. We describe individuals from five families with heterozygous mutations located in the final (third) exon of ZIC1 (encoding four nonsense and one missense change) who have a distinct phenotype in which severe craniosynostosis, specifically involving the coronal sutures, and variable learning disability are the most characteristic features...
September 3, 2015: American Journal of Human Genetics
Catherine Vaillant, Paola Valdivieso, Sandro Nuciforo, Marcel Kool, Alexandra Schwarzentruber-Schauerte, Hélène Méreau, Erik Cabuy, Johannes A Lobrinus, Stefan Pfister, Aimée Zuniga, Stephan Frank, Rolf Zeller
BACKGROUND: Medulloblastomas are malignant childhood brain tumors that arise due to the aberrant activity of developmental pathways during postnatal cerebellar development and in adult humans. Transcriptome analysis has identified four major medulloblastoma subgroups. One of them, the Sonic hedgehog (SHH) subgroup, is caused by aberrant Hedgehog signal transduction due to mutations in the Patched1 (PTCH1) receptor or downstream effectors. Mice carrying a Patched-1 null allele (Ptch1∆/+) are a good model to study the alterations underlying medulloblastoma development as a consequence of aberrant Hedgehog pathway activity...
2015: PloS One
Arnulf H Koeppen, R Liane Ramirez, Alyssa B Becker, Paul J Feustel, Joseph E Mazurkiewicz
Atrophy of large neurons in the dentate nucleus (DN) is an important pathologic correlate of neurologic disability in patients with Friedreich ataxia (FA). Thinning of the DN was quantified in 29 autopsy cases of FA and 2 carriers by measuring the thickness of the gray matter ribbon on stains with anti-glutamic acid decarboxylase, the rate-limiting enzyme in the biosynthesis of γ-amino-butyric acid (GABA). The DN was thinner than normal in all cases of FA, and atrophy correlated inversely with disease duration but not with age at onset or length of the homozygous guanine-adenine-adenine trinucleotide expansions...
February 2015: Journal of Neuropathology and Experimental Neurology
Katrien Smets, Tine Deconinck, Jonathan Baets, Anne Sieben, Jean-Jacques Martin, Iris Smouts, Shuaiyu Wang, Franco Taroni, Daniela Di Bella, Wim Van Hecke, Paul M Parizel, Christina Jadoul, Robert De Potter, Francine Couvreur, Elena Rugarli, Peter De Jonghe
OBJECTIVE: To identify the genetic cause of autosomal dominant spinocerebellar ataxia type 28 (SCA28) with ptosis in 2 Belgian families without AFG3L2 point mutations and further extend the clinical spectrum of SCA28 through the study of a brain autopsy, advanced MRI, and cell-based functional assays exploring the underlying disease mechanism. METHODS: Two large families were clinically examined in detail. Linkage analysis and multiplex amplicon quantification were performed...
June 10, 2014: Neurology
Pamela Magini, Tommaso Pippucci, I-Chun Tsai, Simona Coppola, Emilia Stellacci, Anna Bartoletti-Stella, Daniela Turchetti, Claudio Graziano, Giovanna Cenacchi, Iria Neri, Duccio Maria Cordelli, Valentina Marchiani, Rosalba Bergamaschi, Giuseppe Gasparre, Giovanni Neri, Laura Mazzanti, Annalisa Patrizi, Emilio Franzoni, Giovanni Romeo, Domenico Bordo, Marco Tartaglia, Nicholas Katsanis, Marco Seri
Loss-of-function mutations in PAK3 contribute to non-syndromic X-linked intellectual disability (NS-XLID) by affecting dendritic spine density and morphology. Linkage analysis in a three-generation family with affected males showing ID, agenesis of corpus callosum, cerebellar hypoplasia, microcephaly and ichthyosis, revealed a candidate disease locus in Xq21.33q24 encompassing over 280 genes. Subsequent to sequencing all coding exons of the X chromosome, we identified a single novel variant within the linkage region, affecting a conserved codon of PAK3...
July 1, 2014: Human Molecular Genetics
Sergiy Sylantyev, Leonid P Savtchenko, Yaroslav Ermolyuk, Piotr Michaluk, Dmitri A Rusakov
Electric fields of synaptic currents can influence diffusion of charged neurotransmitters, such as glutamate, in the synaptic cleft. However, this phenomenon has hitherto been detected only through sustained depolarization of large principal neurons, and its adaptive significance remains unknown. Here, we find that in cerebellar synapses formed on electrically compact granule cells, a single postsynaptic action potential can retard escape of glutamate released into the cleft. This retardation boosts activation of perisynaptic group I metabotropic glutamate receptors (mGluRs), which in turn rapidly facilitates local NMDA receptor currents...
February 6, 2013: Neuron
Izak J Bisschoff, Christine Zeschnigk, Denise Horn, Brigitte Wellek, Angelika Rieß, Maja Wessels, Patrick Willems, Peter Jensen, Andreas Busche, Jens Bekkebraten, Maya Chopra, Hanne Dahlgaard Hove, Christina Evers, Ketil Heimdal, Ann-Sophie Kaiser, Erdmut Kunstmann, Kristina Lagerstedt Robinson, Maja Linné, Patricia Martin, James McGrath, Winnie Pradel, Katrina E Prescott, Bernd Roesler, Gorazd Rudolf, Ulrike Siebers-Renelt, Nataliya Tyshchenko, Dagmar Wieczorek, Gerhard Wolff, William B Dobyns, Deborah J Morris-Rosendahl
OFD1, now recognized as a ciliopathy, is characterized by malformations of the face, oral cavity and digits, and is transmitted as an X-linked condition with lethality in males. Mutations in OFD1 also cause X-linked Joubert syndrome (JBTS10) and Simpson-Golabi-Behmel syndrome type 2 (SGBS2). We have studied 55 sporadic and six familial cases of suspected OFD1. Comprehensive mutation analysis in OFD1 revealed mutations in 37 female patients from 30 families; 22 mutations have not been previously described including two heterozygous deletions spanning OFD1 and neighbouring genes...
January 2013: Human Mutation
C Jacquelin, C Strazielle, R Lalonde
Homozygous Dab1(scm) mouse mutants with cell ectopias in cerebellar cortex, hippocampus, and neocortex were compared to non-ataxic controls on the SHIRPA primary screening battery on postnatal days 8, 15, and 22, as well as in the adult period. Dab1(scm) mutants were distinguished from non-ataxic controls as early as postnatal day 8 based on body tremor, gait anomalies, and body weight. On postnatal day 15, motor coordination deficits were evident on horizontal bar and inclined or vertical grid tests in association with a weaker grip strength...
January 1, 2012: Behavioural Brain Research
Véronique M P Moers-Hornikx, Johan S H Vles, Lee Wei Lim, Mustafa Ayyildiz, Suleyman Kaplan, Antonio W D Gavilanes, Govert Hoogland, Harry W M Steinbusch, Yasin Temel
Until recently, the cerebellum was primarily considered to be a structure involved in motor behaviour. New anatomical and clinical evidence has shown that the cerebellum is also involved in higher cognitive functions and non-motor behavioural changes. Functional imaging in patients with anxiety disorders and in cholecystokinin tetrapeptide-induced panic-attacks shows activation changes in the cerebellum. Deep brain stimulation of the dorsolateral periaqueductal grey (dlPAG) and the ventromedial hypothalamus (VMH) in rats has been shown to induce escape behaviour, which mimics a panic attack in humans...
March 2011: Cerebellum
Y Takayasu, M Iino, Y Takatsuru, K Tanaka, S Ozawa
Glutamate transporters play a critical role in the maintenance of low extracellular concentrations of glutamate, which prevents the overactivation of post-synaptic glutamate receptors. Four distinct glutamate transporters, GLAST/EAAT1, GLT-1/EAAT2, EAAC1/EAAT3 and EAAT4, are distributed in the molecular layer of the cerebellum, especially near glutamatergic synapses in Purkinje cells (PCs). This review summarizes the current knowledge about the differential roles of these transporters at excitatory synapses of PCs...
September 2009: Acta Physiologica
Yanyan Wang, Sheng Li, Fengyuan Piao, Yan Hong, Peng Liu, Yufeng Zhao
To elucidate the molecular mechanism of arsenic (As) on motor learning and memory, concentration of As in cerebellar tissue of mice exposed to 1 ppm and 4 ppm As(2)O(3) subchronically was determined by ICP-MS, neurobehavioral changes associated with memory was examined by the Morris Water Maze test, and the critical gene expression profiles related to the Creb-dependent phase of cerebellar long-term depression (LTD) were analyzed by GeneChip. Our results showed the increased level of As concentration in cerebellar tissue of the exposed mice in a dose-response manner, longer escape latency in the experimental group than controls and the down-regulated expression of Ca(2+)/calmodulin dependent protein kinase IV (Camk4), a very important regulator in the LTD pathway...
September 2009: Neurotoxicology and Teratology
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