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Bergmann glia

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https://www.readbyqxmd.com/read/28500648/bergmann-glia-translocation-a-new-disease-marker-for-vanishing-white-matter-identifies-therapeutic-effects-of-guanabenz-treatment
#1
Stephanie Dooves, Marianna Bugiani, Lisanne Wisse, Truus E M Abbink, Marjo S van der Knaap, Vivi M Heine
Vanishing White Matter (VWM) is a devastating leukoencephalopathy without effective treatment options. Patients have mutations in the EIF2B1-5 genes, encoding the five subunits of eIF2B, a guanine exchange factor that is an important regulator of protein translation. We recently developed mouse models for VWM that replicate the human disease. To study disease improvement after treatment in these mice it is essential to have sensitive biomarkers related to disease stage. The Bergmann glia of the cerebellum, an astrocytic subpopulation, translocate into the molecular layer in symptomatic VWM mice and patients...
May 13, 2017: Neuropathology and Applied Neurobiology
https://www.readbyqxmd.com/read/28489004/analogous-mechanism-regulating-formation-of-neocortical-basal-radial-glia-and-cerebellar-bergmann-glia
#2
Xin Heng, Qiuxia Guo, Alan W Leung, James Yh Li
Neocortical basal radial glia (bRG) and cerebellar Bergmann glia (BG) are basal progenitors derived from ventricular apical radial glia (aRG) that selectively lose their apical processes. bRG and BG have been implicated in the expansion and folding of the cerebrum and cerebellum, respectively. Here, we analyzed the molecular characteristics and development of bRG and BG. Transcriptomic comparison revealed striking similarity of the molecular features of bRG and BG. We found that heightened ERK signaling activity in aRG is tightly linked to the temporal formation and the relative abundance of bRG in human and mouse cortices...
May 10, 2017: ELife
https://www.readbyqxmd.com/read/28450263/wls-expression-in-the-rhombic-lip-orchestrates-the-embryonic-development-of-the-mouse-cerebellum
#3
Joanna Yeung, Daniel Goldowitz
Wntless (Wls) is implicated in the Wnt signaling pathway by regulating the secretion of Wnt molecules. During brain development, Wls is expressed in the isthmic organizer (ISO) and rhombic lip (RL). Wls regulates Wnt1 secretion at the ISO which is required to induce midbrain-hindbrain structures. However, Wls function in the RL is not known. Here, we employed Nestin-cre to delete Wls specifically in the RL during mid-gestation. The loss-of-Wls leads to an abnormal RL during development and cerebellar vermis hypoplasia at birth...
April 24, 2017: Neuroscience
https://www.readbyqxmd.com/read/28448983/intrauterine-growth-restriction-alters-the-postnatal-development-of-the-rat-cerebellum
#4
Annie R A McDougall, Vanny Wiradjaja, Aminath Azhan, Anqi Li, Nadia Hale, Mary E Wlodek, Stuart B Hooper, Megan J Wallace, Mary Tolcos
Intrauterine growth restriction (IUGR) is a major cause of antenatal brain injury. We aimed to characterize cerebellar deficits following IUGR and to investigate the potential underlying cellular and molecular mechanisms. At embryonic day 18, pregnant rats underwent either sham surgery (controls; n = 23) or bilateral uterine vessel ligation to restrict blood flow to fetuses (IUGR; n = 20). Offspring were collected at postnatal day 2 (P2), P7, and P35. Body weights were reduced at P2, P7, and P35 in IUGR offspring (p < 0...
April 28, 2017: Developmental Neuroscience
https://www.readbyqxmd.com/read/28439461/quantitative-assessment-of-fibroblast-growth-factor-receptor-1-expression-in-neurons-and-glia
#5
Lisha Choubey, Jantzen C Collette, Karen Müller Smith
BACKGROUND: Fibroblast growth factors (FGFs) and their receptors (FGFRs) have numerous functions in the developing and adult central nervous system (CNS). For example, the FGFR1 receptor is important for proliferation and fate specification of radial glial cells in the cortex and hippocampus, oligodendrocyte proliferation and regeneration, midline glia morphology and soma translocation, Bergmann glia morphology, and cerebellar morphogenesis. In addition, FGFR1 signaling in astrocytes is required for postnatal maturation of interneurons expressing parvalbumin (PV)...
2017: PeerJ
https://www.readbyqxmd.com/read/28388016/remodeling-of-peripheral-nerve-ensheathment-during-the-larval-to-adult-transition-in-drosophila
#6
Aswati Subramanian, Matthew Siefert, Soumya Banerjee, Kumar Vishal, Kayla A Bergmann, Clay C M Curts, Meredith Dorr, Camillo Molina, Joyce Fernandes
Over the course of a four-day period of metamorphosis, the Drosophila larval nervous system is remodeled to prepare for adult-specific behaviors. One example is the reorganization of peripheral nerves in the abdomen, where five pairs of abdominal nerves (A4-A8) fuse to form the terminal nerve trunk. This reorganization is associated with selective remodeling of four layers that ensheath each peripheral nerve. The neural lamella (NL), is the first to dismantle; its breakdown is initiated by 6 hours after puparium formation, and is completely removed by the end of the first day...
April 7, 2017: Developmental Neurobiology
https://www.readbyqxmd.com/read/28370142/morphological-study-of-a-connexin-43-gfp-reporter-mouse-highlights-glial-heterogeneity-amacrine-cells-and-olfactory-ensheathing-cells
#7
Panos Theofilas, Christian Steinhäuser, Martin Theis, Amin Derouiche
Connexin 43 (Cx43) is the main astrocytic connexin and forms the basis of the glial syncytium. The morphology of connexin-expressing cells can be best studied in transgenic mouse lines expressing cytoplasmic fluorescent reporters, since immunolabeling the plaques can obscure the shapes of the individual cells. The Cx43kiECFP mouse generated by Degen et al. (FASEBJ 26:4576, 2012) expresses cytosolic ECFP and has previously been used to establish that Cx43 may not be expressed by all astrocytes within a population, and this can vary in a region-dependent way...
March 30, 2017: Journal of Neuroscience Research
https://www.readbyqxmd.com/read/28314814/respiration-deficient-astrocytes-survive-as-glycolytic-cells-in-vivo
#8
Lotti M Supplie, Tim Düking, Graham Campbell, Francisca Diaz, Carlos T Moraes, Magdalena Götz, Bernd Hamprecht, Susann Boretius, Don Mahad, Klaus-Armin Nave
Neurons and glial cells exchange energy-rich metabolites and it has been suggested, originally based on in vitro data, that astrocytes provide lactate to glutamatergic synapses ("lactate shuttle"). Here, we have studied astrocytes that lack mitochondrial respiration in vitro and in vivo A novel mouse mutant (GLAST(CreERT2)::Cox10(flox/flox)) was generated, in which the administration of tamoxifen causes mutant astrocytes to fail in the assembly of mitochondrial cytochrome c oxidase (COX). Focusing on cerebellar Bergmann glia (BG) cells, which exhibit the highest rate of Cre-mediated recombination, we found a normal density of viable astrocytes even 1 year after tamoxifen-induced Cox10 gene targeting...
April 19, 2017: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/28237844/characterization-of-the-cystine-glutamate-antiporter-in-cultured-bergmann-glia-cells
#9
Edna Suárez-Pozos, Zila Martínez-Lozada, Orquidia G Méndez-Flores, Alain M Guillem, Luisa C Hernández-Kelly, Francisco Castelán, Tatiana N Olivares-Bañuelos, Donaji Chi-Castañeda, Mustapha Najimi, Arturo Ortega
Glutamate, the major excitatory transmitter in the vertebrate brain is a potent neurotoxin through the over-stimulation of its specific membrane receptors. In accordance, a tight regulation of its extracellular levels by plasma membrane transporters is present. A family of excitatory amino acid transporters is expressed in neurons and glia cells and is responsible of the removal of the neurotransmitter from the synaptic cleft. Glial transporters account for more than 80% of the brain uptake activity. The cystine/glutamate antiporter is another plasma membrane-bound protein critically involved in glutamatergic transmission...
February 24, 2017: Neurochemistry International
https://www.readbyqxmd.com/read/28193238/transient-ikk2-activation-in-astrocytes-initiates-selective-non-cell-autonomous-neurodegeneration
#10
Michael Lattke, Stephanie N Reichel, Alexander Magnutzki, Alireza Abaei, Volker Rasche, Paul Walther, Dinis P Calado, Boris Ferger, Thomas Wirth, Bernd Baumann
BACKGROUND: Neuroinflammation is associated with a wide range of neurodegenerative disorders, however the specific contribution to individual disease pathogenesis and selective neuronal cell death is not well understood. Inflammatory cerebellar ataxias are neurodegenerative diseases occurring in various autoimmune/inflammatory conditions, e.g. paraneoplastic syndromes. However, how inflammatory insults can cause selective cerebellar neurodegeneration in the context of these diseases remains open, and appropriate animal models are lacking...
February 13, 2017: Molecular Neurodegeneration
https://www.readbyqxmd.com/read/27878595/cell-type-specific-expression-of-nfix-in-the-developing-and-adult-cerebellum
#11
James Fraser, Alexandra Essebier, Richard M Gronostajski, Mikael Boden, Brandon J Wainwright, Tracey J Harvey, Michael Piper
Transcription factors from the nuclear factor one (NFI) family have been shown to play a central role in regulating neural progenitor cell differentiation within the embryonic and post-natal brain. NFIA and NFIB, for instance, promote the differentiation and functional maturation of granule neurons within the cerebellum. Mice lacking Nfix exhibit delays in the development of neuronal and glial lineages within the cerebellum, but the cell-type-specific expression of this transcription factor remains undefined...
November 23, 2016: Brain Structure & Function
https://www.readbyqxmd.com/read/27859594/glutamate-transporter-associated-anion-channels-adjust-intracellular-chloride-concentrations-during-glial-maturation
#12
Verena Untiet, Peter Kovermann, Niklas J Gerkau, Thomas Gensch, Christine R Rose, Christoph Fahlke
Astrocytic volume regulation and neurotransmitter uptake are critically dependent on the intracellular anion concentration, but little is known about the mechanisms controlling internal anion homeostasis in these cells. Here we used fluorescence lifetime imaging microscopy (FLIM) with the chloride-sensitive dye MQAE to measure intracellular chloride concentrations in murine Bergmann glial cells in acute cerebellar slices. We found Bergmann glial [Cl(-) ]int to be controlled by two opposing transport processes: chloride is actively accumulated by the Na(+) -K(+) -2Cl(-) cotransporter NKCC1, and chloride efflux through anion channels associated with excitatory amino acid transporters (EAATs) reduces [Cl(-) ]int to values that vary upon changes in expression levels or activity of these channels...
February 2017: Glia
https://www.readbyqxmd.com/read/27708533/rice-bran-dietary-supplementation-improves-neurological-symptoms-and-loss-of-purkinje-cells-in-vitamin-e-deficient-mice
#13
Toru Takahashi, Kazuhiro Nakaso, Yosuke Horikoshi, Takehiko Hanaki, Miho Yamakawa, Masato Nakasone, Yoshinori Kitagawa, Taisuke Koike, Tatsuya Matsura
BACKGROUND: Vitamin E (VE, α-tocopherol) is a fat-soluble vitamin and is well known as an antioxidant. A deficiency in VE induces oxidative stress in the brain and causes motor and memory dysfunction. The consumption of a VE-rich diet has been given much attention in recent years, in regards to anti-aging and the prevention of age-related neuronal disorders. METHODS: A VE-deficient mouse model was prepared by feeding the animals a diet lacking VE. In addition, to evaluate the effect of VE-containing rice bran (RB) on VE deficiency, a diet including RB was also provided...
September 2016: Yonago Acta Medica
https://www.readbyqxmd.com/read/27667772/t-cells-underlie-some-but-not-all-of-the-cerebellar-pathology-in-a-neonatal-rat-model-of-congenital-lymphocytic-choriomeningitis-virus-infection
#14
Hannah Klein, Glenda K Rabe, Bahri Karacay, Daniel J Bonthius
Lymphocytic choriomeningitis virus (LCMV) infection during pregnancy injures the human fetal brain. Neonatal rats inoculated with LCMV are an excellent model of congenital LCMV infection because they develop cerebellar injuries similar to those in humans. To evaluate the role of T-lymphocytes in LCMV-induced cerebellar pathology, congenitally athymic rats, deficient in T-lymphocytes were compared with euthymic rats. Peak viral titers and cellular targets of infection were similar, but viral clearance from astrocytes was impaired in the athymic rats...
September 25, 2016: Journal of Neuropathology and Experimental Neurology
https://www.readbyqxmd.com/read/27649671/dystroglycan-induced-muscular-dystrophies-a-review
#15
Q-Z Zhang
Dystroglycanopathies are muscular dystrophies caused by mutations in genes involved the in O-linked glycosylation of α-dystroglycan. Severe forms of these conditions result in abnormalities in exhibit brain and ocular developmental too, in addition to muscular dystrophy. The full spectrum of developmental pathology is caused mainly by loss of dystroglycan from Bergmann glia. Moreover, cognitive deficits are constant features of severe forms of dystroglycanopathies. However, the precise molecular mechanism leading to neuronal dysfunction in these diseases is not fully known yet...
September 2016: European Review for Medical and Pharmacological Sciences
https://www.readbyqxmd.com/read/27586038/developmental-delay-in-motor-skill-acquisition-in-niemann-pick-c1-mice-reveals-abnormal-cerebellar-morphogenesis
#16
Paola Caporali, Francesco Bruno, Giampiero Palladino, Jessica Dragotto, Laura Petrosini, Franco Mangia, Robert P Erickson, Sonia Canterini, Maria Teresa Fiorenza
Niemann-Pick type C1 (NPC1) disease is a lysosomal storage disorder caused by defective intracellular trafficking of exogenous cholesterol. Purkinje cell (PC) degeneration is the main sign of cerebellar dysfunction in both NPC1 patients and animal models. It has been recently shown that a significant decrease in Sonic hedgehog (Shh) expression reduces the proliferative potential of granule neuron precursors in the developing cerebellum of Npc1 (-/-) mice. Pursuing the hypothesis that this developmental defect translates into functional impairments, we have assayed Npc1-deficient pups belonging to the milder mutant mouse strain Npc1 (nmf164) for sensorimotor development from postnatal day (PN) 3 to PN21...
2016: Acta Neuropathologica Communications
https://www.readbyqxmd.com/read/27530255/posterior-cerebellar-purkinje-cells-in-an-sca5-sparca1-mouse-model-are-especially-vulnerable-to-the-synergistic-effect-of-loss-of-%C3%AE-iii-spectrin-and-glast
#17
Emma M Perkins, Daumante Suminaite, Yvonne L Clarkson, Sin Kwan Lee, Alastair R Lyndon, Jeffrey D Rothstein, David J A Wyllie, Kohichi Tanaka, Mandy Jackson
Clinical phenotypes of spinocerebellar ataxia type-5 (SCA5) and spectrin-associated autosomal recessive cerebellar ataxia type-1 (SPARCA1) are mirrored in mice lacking β-III spectrin (β-III(-/-)). One function of β-III spectrin is the stabilization of the Purkinje cell-specific glutamate transporter EAAT4 at the plasma membrane. In β-III(-/-) mice EAAT4 levels are reduced from an early age. In contrast levels of the predominant cerebellar glutamate transporter GLAST, expressed in Bergmann glia, only fall progressively from 3 months onwards...
August 15, 2016: Human Molecular Genetics
https://www.readbyqxmd.com/read/27458343/dystrophin-distribution-and-expression-in-human-and-experimental-temporal-lobe-epilepsy
#18
Ruben G F Hendriksen, Sandra Schipper, Govert Hoogland, Olaf E M G Schijns, Jim T A Dings, Marlien W Aalbers, Johan S H Vles
OBJECTIVE: Dystrophin is part of a protein complex that connects the cytoskeleton to the extracellular matrix. In addition to its role in muscle tissue, it functions as an anchoring protein within the central nervous system such as in hippocampus and cerebellum. Its presence in the latter regions is illustrated by the cognitive problems seen in Duchenne Muscular Dystrophy (DMD). Since epilepsy is also supposed to constitute a comorbidity of DMD, it is hypothesized that dystrophin plays a role in neuronal excitability...
2016: Frontiers in Cellular Neuroscience
https://www.readbyqxmd.com/read/27291422/inflammation-induced-reversible-switch-of-the-neuron-specific-enolase-promoter-from-purkinje-neurons-to-bergmann-glia
#19
Yusuke Sawada, Ayumu Konno, Jun Nagaoka, Hirokazu Hirai
Neuron-specific enolase (NSE) is a glycolytic isoenzyme found in mature neurons and cells of neuronal origin. Injecting adeno-associated virus serotype 9 (AAV9) vectors carrying the NSE promoter into the cerebellar cortex is likely to cause the specific transduction of neuronal cells, such as Purkinje cells (PCs) and interneurons, but not Bergmann glia (BG). However, we found BG-predominant transduction without PC transduction along a traumatic needle tract for viral injection. The enhancement of neuroinflammation by the co-application of lipopolysaccharide (LPS) with AAV9 significantly expanded the BG-predominant area concurrently with the potentiated microglial activation...
2016: Scientific Reports
https://www.readbyqxmd.com/read/27184733/coupling-of-glutamate-and-glucose-uptake-in-cultured-bergmann-glial-cells
#20
Orquidia G Mendez-Flores, Luisa C Hernández-Kelly, Edna Suárez-Pozos, Mustapha Najimi, Arturo Ortega
Glutamate, the main excitatory neurotransmitter in the vertebrate brain, exerts its actions through specific membrane receptors present in neurons and glial cells. Over-stimulation of glutamate receptors results in neuronal death, phenomena known as excitotoxicity. A family of sodium-dependent, glutamate uptake transporters mainly expressed in glial cells, removes the amino acid from the synaptic cleft preventing neuronal death. The sustained sodium influx associated to glutamate removal in glial cells, activates the sodium/potassium ATPase restoring the ionic balance, additionally, glutamate entrance activates glutamine synthetase, both events are energy demanding, therefore glia cells increase their ATP expenditure favouring glucose uptake, and triggering several signal transduction pathways linked to proper neuronal glutamate availability, via the glutamate/glutamine shuttle...
September 2016: Neurochemistry International
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