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https://www.readbyqxmd.com/read/28092268/phenotypic-outcomes-in-mouse-and-human-foxc1-dependent-dandy-walker-cerebellar-malformation-suggest-shared-mechanisms
#1
Parthiv Haldipur, Derek Dang, Kimberly A Aldinger, Olivia K Janson, Fabien Guimiot, Homa Adle-Biasette, William B Dobyns, Joseph R Siebert, Rosa Russo, Kathleen J Millen
FOXC1 loss contributes to Dandy-Walker malformation (DWM), a common human cerebellar malformation.  Previously we found that complete Foxc1 loss leads to aberrations in proliferation, neuronal differentiation and migration in the embryonic mouse cerebellum (Haldipur et al., 2014). We now demonstrate that hypomorphic Foxc1 mutant mice have granule and Purkinje cell abnormalities causing subsequent disruptions in postnatal cerebellar foliation and lamination. Particularly striking is the presence of a partially formed posterior lobule echoing the posterior vermis DW "tail sign" observed in human imaging studies...
January 16, 2017: ELife
https://www.readbyqxmd.com/read/28091578/temporal-dynamics-of-cerebellar-and-motor-cortex-physiological-processes-during-motor-skill-learning
#2
D Spampinato, P Celnik
Learning motor tasks involves distinct physiological processes in the cerebellum (CB) and primary motor cortex (M1). Previous studies have shown that motor learning results in at least two important neurophysiological changes: modulation of cerebellar output mediated in-part by long-term depression of parallel fiber-Purkinje cell synapse and induction of long-term plasticity (LTP) in M1, leading to transient occlusion of additional LTP-like plasticity. However, little is known about the temporal dynamics of these two physiological mechanisms during motor skill learning...
January 16, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28091557/the-same-oculomotor-vermal-purkinje-cells-encode-the-different-kinematics-of-saccades-and-of-smooth-pursuit-eye-movements
#3
Zongpeng Sun, Aleksandra Smilgin, Marc Junker, Peter W Dicke, Peter Thier
Saccades and smooth pursuit eye movements (SPEM) are two types of goal-directed eye movements whose kinematics differ profoundly, a fact that may have contributed to the notion that the underlying cerebellar substrates are separated. However, it is suggested that some Purkinje cells (PCs) in the oculomotor vermis (OMV) of monkey cerebellum may be involved in both saccades and SPEM, a puzzling finding in view of the different kinematic demands of the two types of eye movements. Such 'dual' OMV PCs might be oddities with little if any functional relevance...
January 16, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28088524/possible-roles-of-the-transcription-factor-nrf1-nfe2l1-in-neural-homeostasis-by-regulating-the-gene-expression-of-deubiquitinating-enzymes
#4
Hiroaki Taniguchi, Shota Okamuro, Misaki Kohji, Tsuyoshi Waku, Kaori Kubo, Atsushi Hatanaka, Yimeng Sun, A M Masudul Azad Chowdhury, Akiyoshi Fukamizu, Akira Kobayashi
The transcription factor Nrf1 (NFE2L1) maintains protein homeostasis (proteostasis) by regulating the gene expression of proteasome subunits in response to proteasome inhibition. The deletion of the Nrf1 gene in neural stem/progenitor cells causes severe neurodegeneration due to the accumulation of ubiquitinated proteins in Purkinje cells and motor neurons (Nrf1 NKO mice). However, the molecular mechanisms governing this neurodegenerative process remain unclear. We demonstrate herein that the loss of Nrf1 leads to the reduced gene expression of the deubiquitinating enzymes (DUBs) but not proteasome subunits in Nrf1 NKO mice between P7 and P18...
January 11, 2017: Biochemical and Biophysical Research Communications
https://www.readbyqxmd.com/read/28077726/climbing-fibers-control-purkinje-cell-representations-of-behavior
#5
Martha L Streng, Laurentiu S Popa, Timothy J Ebner
: A crucial issue in understanding cerebellar function is the interaction between simple spike (SS) and complex spike (CS) discharge, the two fundamentally different activity modalities of Purkinje cells. Although several hypotheses have provided insights into the interaction, none fully explain or are completely consistent with the spectrum of experimental observations. Here, we show that during a pseudo-random manual tracking task in the monkey (Macaca mulatta), climbing fiber discharge dynamically controls the information present in the SS firing, triggering robust and rapid changes in the SS encoding of motor signals in 67% of Purkinje cells...
January 11, 2017: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/28076782/gfr%C3%AE-1-regulates-purkinje-cell-migration-by-counteracting-ncam-function
#6
Maria Christina Sergaki, Carlos F Ibáñez
During embryonic development of the cerebellum, Purkinje cells (PCs) migrate away from the ventricular zone to form the PC plate. The mechanisms that regulate PC migration are incompletely understood. Here, we report that the neurotrophic receptor GFRα1 is transiently expressed in developing PCs and loss of GFRα1 delays PC migration. Neither GDNF nor RET, the canonical GFRα1 ligand and co-receptor, respectively, contribute to this process. Instead, we found that the neural cell adhesion molecule NCAM is co-expressed and directly interacts with GFRα1 in embryonic PCs...
January 10, 2017: Cell Reports
https://www.readbyqxmd.com/read/28068280/landolphia-owariensis-attenuates-alcohol-induced-cerebellar-neurodegeneration-significance-of-neurofilament-protein-alteration-in-the-purkinje-cells
#7
Charles A Oyinbo, Patrick S Igbigbi, Godwin O Avwioro
BACKGROUND: Alcohol-induced cerebellar neurodegeneration is a neuroadaptation that is associated with chronic alcohol abuse. Conventional drugs have been largely unsatisfactory in preventing neurodegeneration. Yet, multimodal neuro-protective therapeutic agents have been hypothesised to have high therapeutic potential for the treatment of CNS conditions; there is yet a dilemma of how this would be achieved. Contrarily, medicinal botanicals are naturally multimodal in their mechanism of action...
December 1, 2016: Folia Medica
https://www.readbyqxmd.com/read/28064346/effects-of-cerebellar-transcranial-alternating-current-stimulation-on-motor-cortex-excitability-and-motor-function
#8
Antonino Naro, Alessia Bramanti, Antonino Leo, Alfredo Manuli, Francesca Sciarrone, Margherita Russo, Placido Bramanti, Rocco Salvatore Calabrò
The cerebellum regulates several motor functions through two main mechanisms, the cerebellum-brain inhibition (CBI) and the motor surround inhibition (MSI). Although the exact cerebellar structures and functions involved in such processes are partially known, Purkinje cells (PC) and their surrounding interneuronal networks may play a pivotal role concerning CBI and MSI. Cerebellar transcranial alternating current stimulation (tACS) has been proven to shape specific cerebellar components in a feasible, safe, effective, and non-invasive manner...
January 7, 2017: Brain Structure & Function
https://www.readbyqxmd.com/read/28058675/neuropeptide-cycloprolylglycine-is-an-endogenous-positive-modulator-of-ampa-receptors
#9
T A Gudasheva, V V Grigoriev, K N Koliasnikova, V L Zamoyski, S B Seredenin
We have previously shown that neuropeptide cycloprolylglycine (CPG) increases the content of brain-derived neurotrophic factor (BDNF) in the culture of neuronal cells under normal conditions and in pathology. This is the first study to show that CPG at a physiological concentration of 10(-6) M significantly enhances the transmembrane AMPA currents in rat cerebellar Purkinje cells. Thus, CPG is a positive endogenous modulator of AMPA receptors. It was assumed that the neuropsychotropic effects of CPG are implemented as a result of BDNF accumulation after the activation of AMPA receptors by this neuropeptide...
November 2016: Doklady. Biochemistry and Biophysics
https://www.readbyqxmd.com/read/28053186/intracardiac-injection-of-aav9-npc1-significantly-ameliorates-purkinje-cell-death-and-behavioral-abnormalities-in-mouse-niemann-pick-type-c-disease
#10
Chang Xie, Xue-Min Gong, Jie Luo, Bo-Liang Li, Bao-Liang Song
Niemann-Pick type C (NPC) disease is a fatal, inherited neurodegenerative disorder caused by loss-of-function mutations in the NPC1 or NPC2 gene. There is no effective way to treat NPC disease. In this study, we used adeno-associated virus (AAV) serotype 9 to deliver a functional NPC1 gene systemically into NPC1-/- mice at postnatal day 4 (P4). One single AAV9-NPC1 injection resulted in robust NPC1 expression in various tissues including the brain, heart and lung. Strikingly, AAV9-mediated NPC1 delivery significantly promoted Purkinje cell survival, restored locomotor activity and coordination, as well as increased the lifespan of NPC1-/- mice...
January 4, 2017: Journal of Lipid Research
https://www.readbyqxmd.com/read/28050270/downregulation-of-purkinje-cell-activity-by-modulators-of-small-conductance-calcium-activated-potassium-channels-in-rat-cerebellum
#11
T V Karelina, Yu D Stepanenko, P A Abushik, D A Sibarov, S M Antonov
Small-conductance calcium-activated potassium channels (SK channels) are widely expressed in CNS tissues. Their functions, however, have not been well studied. Participation of SK channels in Purkinje cell (PC) pacemaker activity has been studied predominantly in vitro. Here we studied for the first time the effects of SK channel activation by NS309 or CyPPA on the PC simple spike frequency in vivo in adult (3 - 6 months) and aged (22 - 28 months) rats using extracellular microelectrode recordings. Both pharmacological agents caused a statistically significant decrease in the PC simple spike frequency...
October 2016: Acta Naturae
https://www.readbyqxmd.com/read/28042664/well-formed-cerebellum-and-brainstem-like-structures-in-a-mature-ovarian-teratoma-neuropathological-observations
#12
Masayuki Shintaku 新宅雅幸, Toshiko Sakuma, Chiho Ohbayashi, Motoyoshi Maruo
In the surgical case of a mature cystic teratoma of the ovary that arose in a 16-year-old girl, a large amount of well-differentiated and highly organized cerebellar tissue was found. Three layers of the cerebellar cortex were well formed, and synaptophysin-positive "glomeruli" were found in the granule cell layer. Some Purkinje cells exhibited focal expansion and a dysmorphic appearance of the dendrites. Adjacent to the cerebellar tissue, a large space lined by the ependymal layer and a club-shaped CNS tissue mass resembling the brainstem were found, and structures reminiscent of the midbrain tectum and pontine nuclei were distinguished within this mass...
January 2, 2017: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/28040459/cerebellar-purkinje-cells-die-by-apoptosis-in-the-shaker-mutant-rat
#13
Nour S Erekat
Cerebellar Purkinje cells (PCs) die in humans leading to a variety of diseases including ataxia and essential tremor. The etiology underlying PC death is poorly understood. Shaker mutant rat is a unique animal model of progressive PC degeneration that is compartmentalized and of adult-onset. In shaker mutant rats, hereditary degeneration of at risk PCs occurs between 7 and 14 postnatal weeks of age as a natural phenotypic expression of the shaker mutation and at earlier or later ages depending upon experimental conditions in restricted anterior (ADC) and posterior (PDC) vermal degeneration compartments...
December 28, 2016: Brain Research
https://www.readbyqxmd.com/read/28032667/caffeine-alleviates-progressive-motor-deficits-in-a-transgenic-mouse-model-of-spinocerebellar-ataxia
#14
Nélio Gonçalves, Ana T Simões, Rui S Prediger, Hirokazu Hirai, Rodrigo A Cunha, Luís Pereira de Almeida
OBJECTIVE: Machado-Joseph disease (MJD) is a neurodegenerative spinocerebellar ataxia (SCA) associated with an expanded polyglutamine tract within ataxin-3 for which there is currently no available therapy. We previously showed that caffeine, a non-selective adenosine receptor antagonist, delays the appearance of striatal damage resulting from expression of full-length mutant ataxin-3. Here we investigated the ability of caffeine to alleviate behavioral deficits and cerebellar neuropathology in transgenic mice with a severe ataxia resulting from expression of a truncated fragment of polyglutamine-expanded ataxin-3 in Purkinje cells...
December 29, 2016: Annals of Neurology
https://www.readbyqxmd.com/read/28028212/loss-of-the-golgin-gm130-causes-golgi-disruption-purkinje-neuron-loss-and-ataxia-in-mice
#15
Chunyi Liu, Mei Mei, Qiuling Li, Peristera Roboti, Qianqian Pang, Zhengzhou Ying, Fei Gao, Martin Lowe, Shilai Bao
The Golgi apparatus lies at the heart of the secretory pathway where it is required for secretory trafficking and cargo modification. Disruption of Golgi architecture and function has been widely observed in neurodegenerative disease, but whether Golgi dysfunction is causal with regard to the neurodegenerative process, or is simply a manifestation of neuronal death, remains unclear. Here we report that targeted loss of the golgin GM130 leads to a profound neurological phenotype in mice. Global KO of mouse GM130 results in developmental delay, severe ataxia, and postnatal death...
December 27, 2016: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/28012927/role-of-scoparia-dulcis-linn-on-noise-induced-nitric-oxide-synthase-nos-expression-and-neurotransmitter-assessment-on-motor-function-in-wistar-albino-rats
#16
Wankupar Wankhar, Sakthivel Srinivasan, Loganathan Sundareswaran, Dapkupar Wankhar, Ravindran Rajan, Rathinasamy Sheeladevi
Noise pollution is one of the most widespread and fast growing environmental and occupational menaces in the modern era. Exposure to noise above 100dB is not adaptable through the brain homeostatic mechanism. Yet, the detrimental effects of noise have often been ignored. Developing reliable animal models to understand the neurobiology of noise stress and advance our research in the field of medicine to impede this growing stressor is needed. In this study experimental animals were divided into four groups, (i) Control and (ii) S...
December 22, 2016: Biomedicine & Pharmacotherapy, Biomédecine & Pharmacothérapie
https://www.readbyqxmd.com/read/28009294/synaptic-specializations-support-frequency-independent-purkinje-cell-output-from-the-cerebellar-cortex
#17
Josef Turecek, Skyler L Jackman, Wade G Regehr
The output of the cerebellar cortex is conveyed to the deep cerebellar nuclei (DCN) by Purkinje cells (PCs). Here, we characterize the properties of the PC-DCN synapse in juvenile and adult mice and find that prolonged high-frequency stimulation leads to steady-state responses that become increasingly frequency independent within the physiological firing range of PCs in older animals, resulting in a linear relationship between charge transfer and activation frequency. We used a low-affinity antagonist to show that GABAA-receptor saturation occurs at this synapse but does not underlie frequency-invariant transmission...
December 20, 2016: Cell Reports
https://www.readbyqxmd.com/read/28009283/calcium-imaging-reveals-coordinated-simple-spike-pauses-in-populations-of-cerebellar-purkinje-cells
#18
Jorge E Ramirez, Brandon M Stell
The brain's control of movement is thought to involve coordinated activity between cerebellar Purkinje cells. The results reported here demonstrate that somatic Ca(2+) imaging is a faithful reporter of Na(+)-dependent "simple spike" pauses and enables us to optically record changes in firing rates in populations of Purkinje cells in brain slices and in vivo. This simultaneous calcium imaging of populations of Purkinje cells reveals a striking spatial organization of pauses in Purkinje cell activity between neighboring cells...
December 20, 2016: Cell Reports
https://www.readbyqxmd.com/read/28008682/homozygous-ppt1-splice-donor-mutation-in-a-cane-corso-dog-with-neuronal-ceroid-lipofuscinosis
#19
A Kolicheski, H L Barnes Heller, S Arnold, R D Schnabel, J F Taylor, C A Knox, T Mhlanga-Mutangadura, D P O'Brien, G S Johnson, J Dreyfus, M L Katz
A 10-month-old spayed female Cane Corso dog was evaluated after a 2-month history of progressive blindness, ataxia, and lethargy. Neurologic examination abnormalities indicated a multifocal lesion with primarily cerebral and cerebellar signs. Clinical worsening resulted in humane euthanasia. On necropsy, there was marked astrogliosis throughout white matter tracts of the cerebrum, most prominently in the corpus callosum. In the cerebral cortex and midbrain, most neurons contained large amounts of autofluorescent storage material in the perinuclear area of the cells...
December 23, 2016: Journal of Veterinary Internal Medicine
https://www.readbyqxmd.com/read/28007900/extensive-cryptic-splicing-upon-loss-of-rbm17-and-tdp43-in-neurodegeneration-models
#20
Qiumin Tan, Hari Krishna Yalamanchili, Jeehye Park, Antonia De Maio, Hsiang-Chih Lu, Ying-Wooi Wan, Joshua J White, Vitaliy V Bondar, Layal S Sayegh, Xiuyun Liu, Yan Gao, Roy V Sillitoe, Harry T Orr, Zhandong Liu, Huda Y Zoghbi
Splicing regulation is an important step of post-transcriptional gene regulation. It is a highly dynamic process orchestrated by RNA-binding proteins (RBPs). RBP dysfunction and global splicing dysregulation have been implicated in many human diseases, but the in vivo functions of most RBPs and the splicing outcome upon their loss remain largely unexplored. Here we report that constitutive deletion of Rbm17, which encodes an RBP with a putative role in splicing, causes early embryonic lethality in mice and that its loss in Purkinje neurons leads to rapid degeneration...
October 7, 2016: Human Molecular Genetics
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