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https://www.readbyqxmd.com/read/28214917/mtss1-promotes-maturation-and-maintenance-of-cerebellar-neurons-via-splice-variant-specific-effects
#1
Thorsten Sistig, Fanziska Lang, Sebastian Wrobel, Stephan L Baader, Karl Schilling, Britta Eiberger
Efficient coupling of the actin cytoskeleton to the cell membrane is crucial for histogenesis and maintenance of the nervous system. At this critical interface, BAR (Bin-Amphiphysin-Rvs) proteins regulate membrane bending, shown to be instrumental for mobility and morphogenesis of individual cells. Yet, the systemic significance of these proteins remains largely unexplored. Here, we probe the role of a prominent member of this protein family, the inverse-BAR protein Mtss1, for the development and function of a paradigmatic neuronal circuit, the cerebellar cortex...
February 18, 2017: Brain Structure & Function
https://www.readbyqxmd.com/read/28205531/wnt5a-is-a-crucial-regulator-of-neurogenesis-during-cerebellum-development
#2
Chandramohan Subashini, Sivadasan Bindu Dhanesh, Chih-Ming Chen, Paul Ann Riya, Vadakkath Meera, Thulasi Sheela Divya, Rejji Kuruvilla, Kerstin Buttler, Jackson James
The role of Wnt5a has been extensively explored in various aspects of development but its role in cerebellar development remains elusive. Here, for the first time we unravel the expression pattern and functional significance of Wnt5a in cerebellar development using Wnt5a(-/-) and Nestin-Cre mediated conditional knockout mouse models. We demonstrate that loss of Wnt5a results in cerebellar hypoplasia and depletion of GABAergic and glutamatergic neurons. Besides, Purkinje cells of the mutants displayed stunted, poorly branched dendritic arbors...
February 16, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28202777/nodding-syndrome-may-be-an-autoimmune-reaction-to-the-parasitic-worm-onchocerca-volvulus
#3
Tory P Johnson, Richa Tyagi, Paul R Lee, Myoung-Hwa Lee, Kory R Johnson, Jeffrey Kowalak, Abdel Elkahloun, Marie Medynets, Alina Hategan, Joseph Kubofcik, James Sejvar, Jeffrey Ratto, Sudhir Bunga, Issa Makumbi, Jane R Aceng, Thomas B Nutman, Scott F Dowell, Avindra Nath
Nodding syndrome is an epileptic disorder of unknown etiology that occurs in children in East Africa. There is an epidemiological association with Onchocerca volvulus, the parasitic worm that causes onchocerciasis (river blindness), but there is limited evidence that the parasite itself is neuroinvasive. We hypothesized that nodding syndrome may be an autoimmune-mediated disease. Using protein chip methodology, we detected autoantibodies to leiomodin-1 more abundantly in patients with nodding syndrome compared to unaffected controls from the same village...
February 15, 2017: Science Translational Medicine
https://www.readbyqxmd.com/read/28198698/a-role-for-cerebellum-in-the-hereditary-dystonia-dyt1
#4
Rachel Fremont, Ambika Tewari, Chantal Angueyra, Kamran Khodakhah
DYT1 is a debilitating movement disorder caused by loss-of-function mutations in torsinA. How these mutations cause dystonia remains unknown. Mouse models which have embryonically targeted torsinA have failed to recapitulate the dystonia seen in patients, possibly due to differential development compensation between rodents and humans. To address this issue, torsinA was acutely knocked down in select brain regions of adult mice using shRNAs. TorsinA knockdown in the cerebellum, but not in the basal ganglia, was sufficient to induce dystonia...
February 15, 2017: ELife
https://www.readbyqxmd.com/read/28197345/ventricular-tachycardia-originating-from-moderator-band-new-perspective-on-catheter-ablation
#5
Jin-Yi Li, Jing-Bo Jiang, Yan He, Jian-Chun Luo, Guo-Qiang Zhong
A 59-year-old woman was referred to the institution with burdens of idiopathic ventricular tachycardia (IVT). Electroanatomic mapping revealed a complex fractionated, high frequency potential with long duration preceding the QRS onset of the IVT. The real end point of ablation was the disappearance of the conduction block of Purkinje potential during the sinus rhythm besides the disappearance of the inducible tachycardia. Location of distal catheter was at the moderator band (MB) by transthoracic echocardiography (TTE)...
2017: Case Reports in Cardiology
https://www.readbyqxmd.com/read/28194096/morphological-plasticity-of-emerging-purkinje-cells-in-response-to-exogenous-vegf
#6
Leonard Herrfurth, Verena Theis, Veronika Matschke, Caroline May, Katrin Marcus, Carsten Theiss
Vascular endothelial growth factor (VEGF) is well known as the growth factor with wide-ranging functions even in the central nervous system (CNS). Presently, most attention is given to the investigation of its role in neuronal protection, growth and maturation processes, whereby most effects are mediated through VEGF receptor 2 (VEGFR-2). The purpose of our current study is to provide new insights into the impact of VEGF on immature and mature Purkinje cells (PCs) in accordance with maturity and related receptor expression...
2017: Frontiers in Molecular Neuroscience
https://www.readbyqxmd.com/read/28193810/thresholds-and-noise-limitations-of-colour-vision-in-dim-light
#7
REVIEW
Almut Kelber, Carola Yovanovich, Peter Olsson
Colour discrimination is based on opponent photoreceptor interactions, and limited by receptor noise. In dim light, photon shot noise impairs colour vision, and in vertebrates, the absolute threshold of colour vision is set by dark noise in cones. Nocturnal insects (e.g. moths and nocturnal bees) and vertebrates lacking rods (geckos) have adaptations to reduce receptor noise and use chromatic vision even in very dim light. In contrast, vertebrates with duplex retinae use colour-blind rod vision when noisy cone signals become unreliable, and their transition from cone- to rod-based vision is marked by the Purkinje shift...
April 5, 2017: Philosophical Transactions of the Royal Society of London. Series B, Biological Sciences
https://www.readbyqxmd.com/read/28193238/transient-ikk2-activation-in-astrocytes-initiates-selective-non-cell-autonomous-neurodegeneration
#8
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/28191598/diacylglycerol-kinase-%C3%AE%C2%B5-localizes-to-subsurface-cisterns-of-cerebellar-purkinje-cells
#9
Yasukazu Hozumi, Hiroki Fujiwara, Kenya Kaneko, Satoshi Fujii, Matthew K Topham, Masahiko Watanabe, Kaoru Goto
Following activation of Gq protein-coupled receptors, phospholipase C yields a pair of second messengers: diacylglycerol (DG) and inositol 1,4,5-trisphosphate. Diacylglycerol kinase (DGK) phosphorylates DG to produce phosphatidic acid, another second messenger. Of the DGK family, DGKε is the only DGK isoform that exhibits substrate specificity for DG with an arachidonoyl acyl chain at the sn-2 position. Recently, we demonstrated that hydrophobic residues in the N-terminus of DGKε play an important role in targeting the endoplasmic reticulum in transfected cells...
February 13, 2017: Cell and Tissue Research
https://www.readbyqxmd.com/read/28174627/night-syncope-a-case-report
#10
Léopold Loumaye, Dominique Blommaert
"Idiopathic" ventricular fibrillations are rare and are estimated between 5% and 10% of survivors of hospital cardiac arrest, for at least some of them have as origin, a trigger in the Purkinje fibers. Interventional therapy could be an effective, long-lasting solution for these recurrent malignant arrhythmias and should be considered.
February 2017: Clinical Case Reports
https://www.readbyqxmd.com/read/28174028/immunoreactivity-pattern-of-calretinin-in-the-developing-human-cerebellar-cortex
#11
Valeria Pibiri, Clara Gerosa, Laura Vinci, Gavino Faa, Rossano Ambu
The immunohistochemical expression of the calcium-binding protein calretinin during human cerebellar development has been investigated in this study. Human cerebellum samples, obtained from 7 fetuses and newborns ranging from 11 to 38 weeks of gestation, were 10% formalin-fixed, routinely processed and paraffin-embedded. 3μm-tick sections were immunostained with an anti-calretinin antibody. Our study evidenced a different immunoreactivity for calretinin in Purkinje cells and in several cerebellar interneurons at different intrauterine developmental stages...
February 4, 2017: Acta Histochemica
https://www.readbyqxmd.com/read/28173092/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
#12
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
No abstract text is available yet for this article.
October 15, 2016: Human Molecular Genetics
https://www.readbyqxmd.com/read/28152183/efficient-generation-of-cardiac-purkinje-like-cells-from-embryonic-stem-cells-by-activating-camp-signaling
#13
Su-Yi Tsai, Shuibing Chen, Todd Evans
Strategies to derive cardiac conduction system (CCS) cells including Purkinje cells (PC) would facilitate models for mechanistic studies and drug discovery, and also provide new cellular materials for regenerative therapies. However, using current cardiac differentiation protocols, the differentiation efficiency of CCS cells is extremely low, typically below 1% of the culture. High-throughput chemical screening is a powerful strategy for identifying small molecules that can activate signaling pathways to enhance embryonic stem cell (ESC) differentiation...
February 2, 2017: Current Protocols in Stem Cell Biology
https://www.readbyqxmd.com/read/28133547/accumulation-of-%C3%AE-synuclein-in-cerebellar-purkinje-cells-of-diabetic-rats-and-its-potential-relationship-with-inflammation-and-oxidative-stress-markers
#14
Volkan Solmaz, Hatice Köse Özlece, Hüseyin Avni Eroglu, Hüseyin Aktuğ, Oytun Erbaş, Dilek Taşkıran
Objective. The present study was conducted to evaluate the relationship between plasma oxidative stress markers such as malondialdehyde (MDA) and glutathione (GSH), inflammatory marker pentraxin-3 (PTX3), and cerebellar accumulation of α-synuclein in streptozotocin- (STZ-) induced diabetes model in rats. Methods. Twelve rats were included in the study. Diabetes (n = 6) was induced with a single intraperitoneal injection of streptozotocin (STZ, 60 mg/kg). Diabetes was verified after 48 h by measuring blood glucose levels...
2017: Neurology Research International
https://www.readbyqxmd.com/read/28131213/gene-dosage-effect-in-spinocerebellar-ataxia-type-6-homozygotes-a-clinical-and-neuropathological-study
#15
Kazumasa Soga, Kinya Ishikawa, Tokuro Furuya, Tadatsune Iida, Tetsuo Yamada, Noboru Ando, Kiyobumi Ota, Hiromi Kanno-Okada, Shinya Tanaka, Masayuki Shintaku, Yoshinobu Eishi, Hidehiro Mizusawa, Takanori Yokota
Spinocerebellar ataxia type 6 (SCA6) is an autosomal dominant neurodegenerative disorder. However, it remains unclear whether SCA6 shows a gene dosage effect, defined by earlier age-of-onset in homozygotes than heterozygotes. Herein, we retrospectively analyzed four homozygous SCA6 subjects from our single institution cohort of 120 SCA6 subjects. We also performed a neuropathological investigation into an SCA6 individual with compound heterozygous expansions. In the 116 heterozygotes, there was an inverse correlation of age-of-onset with the number of CAG repeats in the expanded allele, and with the total number of CAG repeats, in both normal and expanded alleles...
February 15, 2017: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/28124605/increase-signaling-of-wnt-%C3%AE-catenin-pathway-and-presence-of-apoptosis-in-cerebellum-of-kindled-rats
#16
Carmen Rubio-Osornio, Artemio Rosiles-Abonce, Cristina Trejo-Solís, Moisés Rubio-Osornio, Cesar Mendoza, Verónica Custodio, Juan Carlos Martínez-Lazcano, Edith González, Carlos Paz
Epilepsy is one of the most common neurological disorders in humans, and the role of the cerebellum in its physiopathology remains the subject of study. The Purkinje cells (PC), whose axons target the dentate and interpositus nuclei, form the main cerebellar output to forebrain structures involved in epilepsy. Cerebellar atrophy related to loss of PC has been reported in chronic epilepsy although its mechanism remains unclear. Taking in account that an overexpression of β-Catenin has been related with cell death, here we present the signaling of β-Catenin and the type of PC death in cerebellum of rats with chronic seizures induced by the amygdaloid kindling model...
January 17, 2017: CNS & Neurological Disorders Drug Targets
https://www.readbyqxmd.com/read/28122232/vulnerability-of-purkinje-cells-generated-from-spinocerebellar-ataxia-type-6-patient-derived-ipscs
#17
Yoshihito Ishida, Hideshi Kawakami, Hiroyuki Kitajima, Ayaka Nishiyama, Yoshiki Sasai, Haruhisa Inoue, Keiko Muguruma
No abstract text is available yet for this article.
January 24, 2017: Cell Reports
https://www.readbyqxmd.com/read/28117367/the-sorting-receptor-rer1-controls-purkinje-cell-function-via-voltage-gated-sodium-channels
#18
Christina Valkova, Lutz Liebmann, Andreas Krämer, Christian A Hübner, Christoph Kaether
Rer1 is a sorting receptor in the early secretory pathway that controls the assembly and the cell surface transport of selected multimeric membrane protein complexes. Mice with a Purkinje cell (PC) specific deletion of Rer1 showed normal polarization and differentiation of PCs and normal development of the cerebellum. However, PC-specific loss of Rer1 led to age-dependent motor deficits in beam walk, ladder climbing and gait. Analysis of brain sections revealed a specific degeneration of PCs in the anterior cerebellar lobe in old animals...
January 24, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28114483/bayesian-microsaccade-detection
#19
Andra Mihali, Bas van Opheusden, Wei Ji Ma
Microsaccades are high-velocity fixational eye movements, with special roles in perception and cognition. The default microsaccade detection method is to determine when the smoothed eye velocity exceeds a threshold. We have developed a new method, Bayesian microsaccade detection (BMD), which performs inference based on a simple statistical model of eye positions. In this model, a hidden state variable changes between drift and microsaccade states at random times. The eye position is a biased random walk with different velocity distributions for each state...
January 1, 2017: Journal of Vision
https://www.readbyqxmd.com/read/28111173/cardiac-expression-of-ryanodine-receptor-subtype-3-a-strategic-component-in-the-intracellular-ca-2-release-system-of-purkinje-fibers-in-large-mammalian-heart
#20
Rebecca E Daniels, Kazi T Haq, Lawson S Miller, Elizabeth W Chia, Masahito Miura, Vincenzo Sorrentino, John J McGuire, Bruno D Stuyvers
BACKGROUND: Three distinct Ca(2+) release channels were identified in dog P-cells: the ryanodine receptor subtype 2 (RyR2) was detected throughout the cell, while the ryanodine receptor subtype 3 (RyR3) and inositol phosphate sensitive Ca(2+) release channel (InsP3R) were found in the cell periphery. How each of these channels contributes to the Ca(2+) cycling of P-cells is unclear. Recent modeling of Ca(2+) mobilization in P-cells suggested that Ca(2+) sensitivity of Ca(2+)induced Ca(2+)release (CICR) was larger at the P-cell periphery...
January 20, 2017: Journal of Molecular and Cellular Cardiology
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