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https://www.readbyqxmd.com/read/28335020/znhit3-is-defective-in-peho-syndrome-a-severe-encephalopathy-with-cerebellar-granule-neuron-loss
#1
Anna-Kaisa Anttonen, Anni Laari, Maria Kousi, Yawei J Yang, Tiina Jääskeläinen, Mirja Somer, Eija Siintola, Eveliina Jakkula, Mikko Muona, Saara Tegelberg, Tuula Lönnqvist, Helena Pihko, Leena Valanne, Anders Paetau, Melody P Lun, Johanna Hästbacka, Outi Kopra, Tarja Joensuu, Nicholas Katsanis, Maria K Lehtinen, Jorma J Palvimo, Anna-Elina Lehesjoki
Progressive encephalopathy with oedema, hypsarrhythmia, and optic atrophy (PEHO) syndrome is an early childhood onset, severe autosomal recessive encephalopathy characterized by extreme cerebellar atrophy due to almost total granule neuron loss. By combining homozygosity mapping in Finnish families with Sanger sequencing of positional candidate genes and with exome sequencing a homozygous missense substitution of leucine for serine at codon 31 in ZNHIT3 was identified as the primary cause of PEHO syndrome. ZNHIT3 encodes a nuclear zinc finger protein previously implicated in transcriptional regulation and in small nucleolar ribonucleoprotein particle assembly and thus possibly to pre-ribosomal RNA processing...
March 1, 2017: Brain: a Journal of Neurology
https://www.readbyqxmd.com/read/28321129/cerebellar-granule-cells-encode-the-expectation-of-reward
#2
Mark J Wagner, Tony Hyun Kim, Joan Savall, Mark J Schnitzer, Liqun Luo
The human brain contains approximately 60 billion cerebellar granule cells, which outnumber all other brain neurons combined. Classical theories posit that a large, diverse population of granule cells allows for highly detailed representations of sensorimotor context, enabling downstream Purkinje cells to sense fine contextual changes. Although evidence suggests a role for the cerebellum in cognition, granule cells are known to encode only sensory and motor context. Here, using two-photon calcium imaging in behaving mice, we show that granule cells convey information about the expectation of reward...
March 20, 2017: Nature
https://www.readbyqxmd.com/read/28319608/cerebellar-granule-cells-acquire-a-widespread-predictive-feedback-signal-during-motor-learning
#3
Andrea Giovannucci, Aleksandra Badura, Ben Deverett, Farzaneh Najafi, Talmo D Pereira, Zhenyu Gao, Ilker Ozden, Alexander D Kloth, Eftychios Pnevmatikakis, Liam Paninski, Chris I De Zeeuw, Javier F Medina, Samuel S-H Wang
Cerebellar granule cells, which constitute half the brain's neurons, supply Purkinje cells with contextual information necessary for motor learning, but how they encode this information is unknown. Here we show, using two-photon microscopy to track neural activity over multiple days of cerebellum-dependent eyeblink conditioning in mice, that granule cell populations acquire a dense representation of the anticipatory eyelid movement. Initially, granule cells responded to neutral visual and somatosensory stimuli as well as periorbital airpuffs used for training...
March 20, 2017: Nature Neuroscience
https://www.readbyqxmd.com/read/28317875/chd7-is-indispensable-for-mammalian-brain-development-through-activation-of-a-neuronal-differentiation-programme
#4
Weijun Feng, Daisuke Kawauchi, Huiqin Körkel-Qu, Huan Deng, Elisabeth Serger, Laura Sieber, Jenna Ariel Lieberman, Silvia Jimeno-González, Sander Lambo, Bola S Hanna, Yassin Harim, Malin Jansen, Anna Neuerburg, Olga Friesen, Marc Zuckermann, Vijayanad Rajendran, Jan Gronych, Olivier Ayrault, Andrey Korshunov, David T W Jones, Marcel Kool, Paul A Northcott, Peter Lichter, Felipe Cortés-Ledesma, Stefan M Pfister, Hai-Kun Liu
Mutations in chromatin modifier genes are frequently associated with neurodevelopmental diseases. We herein demonstrate that the chromodomain helicase DNA-binding protein 7 (Chd7), frequently associated with CHARGE syndrome, is indispensable for normal cerebellar development. Genetic inactivation of Chd7 in cerebellar granule neuron progenitors leads to cerebellar hypoplasia in mice, due to the impairment of granule neuron differentiation, induction of apoptosis and abnormal localization of Purkinje cells, which closely recapitulates known clinical features in the cerebella of CHARGE patients...
March 20, 2017: Nature Communications
https://www.readbyqxmd.com/read/28315868/cerebellar-fastigial-nucleus-electrical-stimulation-alleviates-depressive-like-behaviors-in-post-stroke-depression-rat-model-and-potential-mechanisms
#5
Lei Zhang, Mingkui Zhao, Ru-Bo Sui
OBJECTIVE: To identify the molecular mechanism of post-stroke depression (PSD), and observe the therapeutic effects of cerebellar fastigial nucleus electrical stimulation (FNS) on the behaviors and regional cerebral blood flow (rCBF) in a PSD rat model. METHODS: Healthy SD rats were randomly divided into four groups (sham, stroke, post-stroke depress and FNS group). Sham group (n = 6) underwent sham operation. The other three groups (n = 6*3) underwent MCAO. Rats were examined twice a week in open filed test...
March 16, 2017: Cellular Physiology and Biochemistry
https://www.readbyqxmd.com/read/28315396/ischemia-deteriorates-spike-encoding-at-cortical-gabaergic-neurons-and-cerebellar-purkinje-cells-by-increasing-the-intracellular-ca-2
#6
Li Huang, Chun Wang, Rongjing Ge, Hong Ni, Shidi Zhao
GABAergic neurons play a critical role in the central nervous system, such as well-organized behaviors. The ischemic cell death is presumably initiated by neuronal excitotoxicity resulted from the dysfunction of GABAergic neurons. It is not clear how ischemia influences different types of GABAergic neurons and whether intracellular Ca(2+) plays a key role in the ischemic excitotoxicity. We have investigated this issue at cortical GABAergic neurons and cerebellar Purkinje cells by whole-cell recording in mouse brain slices, and the roles of intracellular Ca(2+) are examined by BABTA infusion...
March 14, 2017: Brain Research Bulletin
https://www.readbyqxmd.com/read/28300552/monoaminergic-modulation-of-gabaergic-transmission-onto-cerebellar-globular-cells
#7
Moritoshi Hirono, Soichi Nagao, Yuchio Yanagawa, Shiro Konishi
Cerebellar Purkinje cells (PCs) project their axon collaterals to underneath of the PC layer and make GABAergic synaptic contacts with globular cells, a subgroup of Lugaro cells. GABAergic transmission derived from the PC axon collaterals is so powerful that it could inhibit globular cells and regulate their firing patterns. However, the physiological properties and implications of the GABAergic synapses on globular cells remain unknown. Using whole-cell patch-clamp recordings from globular cells in the mouse cerebellum, we examined the monoaminergic modulation of GABAergic inputs to these cells...
March 11, 2017: Neuropharmacology
https://www.readbyqxmd.com/read/28299779/the-related-neuronal-endosomal-proteins-neep21-nsg1-and-p19-nsg2-have-divergent-expression-profiles-in-vivo
#8
Kelly Barford, Chan Choo Yap, Noelle D Dwyer, Bettina Winckler
Endosomal maturation and transport constitutes a complex trafficking system present in all cell types. Neurons have adapted their endosomal system to meet their unique and complex needs. These adaptations include repurposing existing proteins to diversify endocytosis and trafficking, as well as preferential expression of certain regulators more highly in neurons than other cell types. These neuronal regulators include the family of Neuron-Specific Gene family members (Nsg), NEEP21 (Nsg1), and P19 (Nsg2). NEEP21/Nsg1 plays a role in the trafficking of multiple receptors, including the cell adhesion molecule L1/NgCAM, the neurotransmitter receptor GluA2, and β-APP...
March 15, 2017: Journal of Comparative Neurology
https://www.readbyqxmd.com/read/28297843/spiral-wave-dynamics-in-a-mathematical-model-of-human-ventricular-tissue-with-myocytes-and-purkinje-fibers
#9
Alok Ranjan Nayak, A V Panfilov, Rahul Pandit
We present systematic numerical studies of the possible effects of the coupling of human endocardial and Purkinje cells at cellular and two-dimensional tissue levels. We find that the autorhythmic-activity frequency of the Purkinje cell in a composite decreases with an increase in the coupling strength; this can even eliminate the autorhythmicity. We observe a delay between the beginning of the action potentials of endocardial and Purkinje cells in a composite; such a delay increases as we decrease the diffusive coupling, and eventually a failure of transmission occurs...
February 2017: Physical Review. E
https://www.readbyqxmd.com/read/28293172/ethanol-modulates-the-spontaneous-complex-spike-waveform-of-cerebellar-purkinje-cells-recorded-in-vivo-in-mice
#10
Guang-Jian Zhang, Mao-Cheng Wu, Jin-Di Shi, Yin-Hua Xu, Chun-Ping Chu, Song-Biao Cui, De-Lai Qiu
Cerebellar Purkinje cells (PCs) are sensitive to ethanol, but the effect of ethanol on spontaneous complex spike (CS) activity in these cells in vivo is currently unknown. Here, we investigated the effect of ethanol on spontaneous CS activity in PCs in urethane-anesthetized mice using in vivo patch-clamp recordings and pharmacological manipulation. Ethanol (300 mM) induced a decrease in the CS-evoked pause in simple spike (SS) firing and in the amplitude of the afterhyperpolarization (AHP) under current clamp conditions...
2017: Frontiers in Cellular Neuroscience
https://www.readbyqxmd.com/read/28283581/mtcl1-plays-an-essential-role-in-maintaining-purkinje-neuron-axon-initial-segment
#11
Tomoko Satake, Kazunari Yamashita, Kenji Hayashi, Satoko Miyatake, Miwa Tamura-Nakano, Hiroshi Doi, Yasuhide Furuta, Go Shioi, Eriko Miura, Yukari H Takeo, Kunihiro Yoshida, Hiroyuki Yahikozawa, Naomichi Matsumoto, Michisuke Yuzaki, Atsushi Suzuki
The axon initial segment (AIS) is a specialized domain essential for neuronal function, the formation of which begins with localization of an ankyrin-G (AnkG) scaffold. However, the mechanism directing and maintaining AnkG localization is largely unknown. In this study, we demonstrate that in vivo knockdown of microtubule cross-linking factor 1 (MTCL1) in cerebellar Purkinje cells causes loss of axonal polarity coupled with AnkG mislocalization. MTCL1 lacking MT-stabilizing activity failed to restore these defects, and stable MT bundles spanning the AIS were disorganized in knockdown cells...
March 10, 2017: EMBO Journal
https://www.readbyqxmd.com/read/28269518/whole-heart-modeling-spatiotemporal-dynamics-of-electrical-wave-conduction-and-propagation
#12
Hui Yang, Yun Chen, Fabio M Leonelli
Cardiac electrical activities are varying in both space and time. Human heart consists of a fractal network of muscle cells, Purkinje fibers, arteries and veins. Whole-heart modeling of electrical wave conduction and propagation involves a greater level of complexity. Our previous work developed a computer model of the anatomically realistic heart and simulated the electrical conduction with the use of cellular automata. However, simplistic assumptions and rules limit its ability to provide an accurate approximation of real-world dynamics on the complex heart surface, due to sensitive dependence of nonlinear dynamical systems on initial conditions...
August 2016: Conference Proceedings: Annual International Conference of the IEEE Engineering in Medicine and Biology Society
https://www.readbyqxmd.com/read/28268302/role-of-cytosolic-calcium-diffusion-in-cardiac-purkinje-cells
#13
Bijay Limbu, Kushal Shah, Makarand Deo
The Cardiac Purkinje cells (PCs) exhibit distinct calcium (Ca2+) homeostasis than that in ventricular myocytes (VMs). Due to lack of t-tubules in PCs, the Ca2+ ions entering the cell have to diffuse through the cytoplasm to reach the sarcoplasmic reticulum (SR) before triggering Ca2+-induced-Ca2+-release (CICR). In recent experimental studies PCs have been shown to be more susceptible to action potential (AP) abnormalities than the VMs, however the exact mechanisms are poorly understood. In this study, we utilize morphologically realistic detailed biophysical mathematical model of a murine PC to systematically examine the role intracellular Ca2+ diffusion in the APs of PCs...
August 2016: Conference Proceedings: Annual International Conference of the IEEE Engineering in Medicine and Biology Society
https://www.readbyqxmd.com/read/28268246/cerebellar-compartmentation-of-prion-pathogenesis
#14
Audrey Ragagnin, Juliette Ezpeleta, Aurélie Guillemain, François Boudet-Devaud, Anne-Marie Haeberlé, Valérie Demais, Catherine Vidal, Stanislas Demuth, Vincent Béringue, Odile Kellermann, Benoit Schneider, Nancy J Grant, Yannick Bailly
In prion diseases, the brain lesion profile is influenced by the prion "strain" properties, the invasion route to the brain, and still unknown host cell-specific parameters. To gain insight into those endogenous factors, we analyzed the histopathological alterations induced by distinct prion strains in the mouse cerebellum. We show that 22L and ME7 scrapie prion proteins (PrP(22L) , PrP(ME7) ), but not bovine spongiform encephalopathy PrP(6PB1) , accumulate in a reproducible parasagittal banding pattern in the cerebellar cortex of infected mice...
March 7, 2017: Brain Pathology
https://www.readbyqxmd.com/read/28264639/a-multiple-plasticity-spiking-neural-network-embedded-in-a-closed-loop-control-system-to-model-cerebellar-pathologies
#15
Alice Geminiani, Claudia Casellato, Alberto Antonietti, Egidio D'Angelo, Alessandra Pedrocchi
The cerebellum plays a crucial role in sensorimotor control and cerebellar disorders compromise adaptation and learning of motor responses. However, the link between alterations at network level and cerebellar dysfunction is still unclear. In principle, this understanding would benefit of the development of an artificial system embedding the salient neuronal and plastic properties of the cerebellum and operating in closed-loop. To this aim, we have exploited a realistic spiking computational model of the cerebellum to analyze the network correlates of cerebellar impairment...
January 10, 2017: International Journal of Neural Systems
https://www.readbyqxmd.com/read/28261874/samp8-mice-as-a-neuropathological-model-of-accelerated-brain-aging-and-dementia-toshio-takeda-s-legacy-and-future-directions
#16
REVIEW
Ichiro Akiguchi, Mercè Pallàs, Herbert Budka, Haruhiko Akiyama, Masaki Ueno, Jingxian Han, Hideo Yagi, Tomohumi Nishikawa, Yoichi Chiba, Hiroshi Sugiyama, Ryoya Takahashi, Keiko Unno, Keiichi Higuchi, Masanori Hosokawa
Senescence accelerated mice P8 (SAMP8) show significant age-related deteriorations in memory and learning ability in accordance with early onset and rapid advancement of senescence. Brains of SAMP8 mice reveal an age-associated increase of PAS-positive granular structures in the hippocampal formation and astrogliosis in the brain stem and hippocampus. A spongy degeneration in the brain stem appears at 1 month of age and reaches a maximum at 4-8 months. In addition, clusters of activated microglia also appear around the vacuoles in the brain stem...
March 6, 2017: Neuropathology: Official Journal of the Japanese Society of Neuropathology
https://www.readbyqxmd.com/read/28256093/a-quantitative-structural-and-morphometric-analysis-of-the-purkinje-network-and-the-purkinje-myocardial-junctions-in-pig-hearts
#17
V Garcia-Bustos, R Sebastian, M Izquierdo, P Molina, F J Chorro, A Ruiz-Sauri
The morpho-functional properties of the distal section of the cardiac Purkinje network (PN) and the Purkinje-myocardial junctions (PMJs) are fundamental to understanding the sequence of electrical activation in the heart. The overall structure of the system has already been described, and several computational models have been developed to gain insight into its involvement in cardiac arrhythmias or its interaction with implantable devices, such as pacemakers. However, anatomical descriptions of the PN in the literature have not enabled enough improvements in the accuracy of anatomical-based electrophysiological simulations of the PN in 3D hearts models...
March 3, 2017: Journal of Anatomy
https://www.readbyqxmd.com/read/28254550/electric-injury-induced-purkinje-cell-apoptosis-in-rat-cerebellum-histological-and-immunohistochemical-study
#18
Samah Kandeel, Naema Mahmoud Elhosary, Mona Mohamed Abo El-Noor, Mohamed Balaha
INTRODUCTION: Electrical injury is a prominent problem in low income countries with increased morbidity and mortality rate. Nervous system is one of the most susceptible systems to electrical current because of its low resistance. There were different studies demonstrated electrocution effect on the nervous system, however little was made on the cerebellum. AIM: This study was conducted to produce an experimental suggestion concerning injury of the nervous system through evaluating Purkinje cell apoptosis and number in rat cerebellum by fatal and non-fatal electric current using histological and immunohistochemical study...
February 28, 2017: Journal of Chemical Neuroanatomy
https://www.readbyqxmd.com/read/28243504/aminopyridines-for-the-treatment-of-neurologic-disorders
#19
REVIEW
Michael Strupp, Julian Teufel, Andreas Zwergal, Roman Schniepp, Kamran Khodakhah, Katharina Feil
PURPOSE OF REVIEW: To identify the different indications for the treatment of neurologic disorders with the potassium channel blockers 4-aminopyridine (4-AP) and 3,4-diaminopyridine (3,4-DAP). RECENT FINDINGS: 4-AP is an effective symptomatic treatment for downbeat nystagmus (DBN), episodic ataxia type 2 (EA2) (5-10 mg TID), and impaired gait in multiple sclerosis (MS) (10 mg BID). 3,4-DAP (5 mg/d-20 mg TID) improves symptoms in Lambert-Eaton myasthenic syndrome (LEMS) (randomized placebo-controlled trials for all 4 entities)...
February 2017: Neurology. Clinical Practice
https://www.readbyqxmd.com/read/28237314/preventive-motor-training-but-not-progenitor-grafting-ameliorates-cerebellar-ataxia-and-deregulated-autophagy-in-tambaleante-mice
#20
Elisa Fucà, Michela Guglielmotto, Enrica Boda, Ferdinando Rossi, Ketty Leto, Annalisa Buffo
Treatment options for degenerative cerebellar ataxias are currently very limited. A large fraction of such disorders is represented by hereditary cerebellar ataxias, whose familiar transmission facilitates an early diagnosis and may possibly allow to start preventive treatments before the onset of the neurodegeneration and appearance of first symptoms. In spite of the heterogeneous aetiology, histological alterations of ataxias often include the primary degeneration of the cerebellar cortex caused by Purkinje cells (PCs) loss...
February 24, 2017: Neurobiology of Disease
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