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https://www.readbyqxmd.com/read/28411148/behavioural-characterization-of-ankyring-deficient-mice-a-model-for-ank3-related-disorders
#1
I M van der Werf, D Van Dam, S Missault, B Yalcin, P P De Deyn, G Vandeweyer, R F Kooy
ANK3 encodes AnkyrinG (AnkG), a member of the Ankyrin family that is expressed in several different isoforms in many tissues. A unique serine-rich domain and tail domain in the two largest isoforms of AnkG (270 and 480kDa), restrict AnkG to the axon initial segment and nodes of Ranvier of myelinated neurons. At these sites, AnkG is a master regulator, coordinating the strict clustering of components necessary for proper action potential initiation and propagation along the axon. These components include voltage-gated sodium channels, potassium channels and members of the L1 cell adhesion molecule family...
April 11, 2017: Behavioural Brain Research
https://www.readbyqxmd.com/read/28405806/rearrangement-of-potassium-ions-and-kv1-1-kv1-2-potassium-channels-in-regenerating-axons-following-end-to-end-neurorrhaphy-ionic-images-from-tof-sims
#2
Chiung-Hui Liu, Hung-Ming Chang, Tsung-Huan Wu, Li-You Chen, Yin-Shuo Yang, To-Jung Tseng, Wen-Chieh Liao
The voltage-gated potassium channels Kv1.1 and Kv1.2 that cluster at juxtaparanodal (JXP) regions are essential in the regulation of nerve excitability and play a critical role in axonal conduction. When demyelination occurs, Kv1.1/Kv1.2 activity increases, suppressing the membrane potential nearly to the equilibrium potential of K(+), which results in an axonal conduction blockade. The recovery of K(+)-dependent communication signals and proper clustering of Kv1.1/Kv1.2 channels at JXP regions may directly reflect nerve regeneration following peripheral nerve injury...
April 12, 2017: Histochemistry and Cell Biology
https://www.readbyqxmd.com/read/28400815/delayed-treatment-of-secondary-degeneration-following-acute-optic-nerve-transection-using-a-combination-of-ion-channel-inhibitors
#3
Nathanael J Yates, Marcus K Giacci, Ryan L O'Hare Doig, Wissam Chiha, Bethany E Ashworth, Jade Kenna, Carole A Bartlett, Melinda Fitzgerald
Studies have shown that a combined application of several ion channel inhibitors immediately after central nervous system injury can inhibit secondary degeneration. However, for clinical use, it is necessary to determine how long after injury the combined treatment of several ion channel inhibitors can be delayed and efficacy maintained. In this study, we delivered Ca(2+) entry-inhibiting P2X7 receptor antagonist oxidized-ATP and AMPA receptor antagonist YM872 to the optic nerve injury site via an iPRECIO(@) pump immediately, 6 hours, 24 hours and 7 days after partial optic nerve transection surgery...
February 2017: Neural Regeneration Research
https://www.readbyqxmd.com/read/28340093/the-molecular-and-morphologic-structures-that-make-saltatory-conduction-possible-in-peripheral-nerve
#4
Steven L Carroll
Saltatory conduction is the process by which action potentials are rapidly and efficiently propagated along myelinated axons. In the peripheral nervous system, saltatory conduction is made possible by a series of morphologically and molecularly distinct subdomains in both axons and their associated myelinating Schwann cells. This review briefly summarizes current knowledge on the molecular structure and physiology of the node of Ranvier and adjacent regions of the axoglial unit in peripheral nerve.
April 1, 2017: Journal of Neuropathology and Experimental Neurology
https://www.readbyqxmd.com/read/28334857/co-cultures-with-stem-cell-derived-human-sensory-neurons-reveal-regulators-of-peripheral-myelination
#5
Alex J Clark, Malte S Kaller, Jorge Galino, Hugh J Willison, Simon Rinaldi, David L H Bennett
See Saporta and Shy (doi:10.1093/awx048) for a scientific commentary on this article.Effective bidirectional signalling between axons and Schwann cells is essential for both the development and maintenance of peripheral nerve function. We have established conditions by which human induced pluripotent stem cell-derived sensory neurons can be cultured with rat Schwann cells, and have produced for the first time long-term and stable myelinating co-cultures with human neurons. These cultures contain the specialized domains formed by axonal interaction with myelinating Schwann cells, such as clustered voltage-gated sodium channels at the node of Ranvier and Shaker-type potassium channel (Kv1...
April 1, 2017: Brain: a Journal of Neurology
https://www.readbyqxmd.com/read/28318976/assembly-of-cns-nodes-of-ranvier-in-myelinated-nerves-is-promoted-by-the-axon-cytoskeleton
#6
Veronica Brivio, Catherine Faivre-Sarrailh, Elior Peles, Diane L Sherman, Peter J Brophy
Nodes of Ranvier in the axons of myelinated neurons are exemplars of the specialized cell surface domains typical of polarized cells. They are rich in voltage-gated sodium channels (Nav) and thus underpin rapid nerve impulse conduction in the vertebrate nervous system [1]. Although nodal proteins cluster in response to myelination, how myelin-forming glia influence nodal assembly is poorly understood. An axoglial adhesion complex comprising glial Neurofascin155 and axonal Caspr/Contactin flanks mature nodes [2]...
April 3, 2017: Current Biology: CB
https://www.readbyqxmd.com/read/28288671/microglia-derived-il-1%C3%AE-contributes-to-axon-development-disorders-and-synaptic-deficit-through-p38-mapk-signal-pathway-in-septic-neonatal-rats
#7
Qianpeng Han, Qiongyu Lin, Peixian Huang, Mengmeng Chen, Xin Hu, Hui Fu, Shaoru He, Fengcai Shen, Hongke Zeng, Yiyu Deng
BACKGROUND: Axon development plays a pivotal role in the formation of synapse, nodes of Ranvier, and myelin sheath. Interleukin-1β (IL-1β) produced by microglia may cause myelination disturbances through suppression of oligodendrocyte progenitor cell maturation in the septic neonatal rats. Here, we explored if a microglia-derived IL-1β would disturb axon development in the corpus callosum (CC) following lipopolysaccharide (LPS) administration, and if so, whether it is associated with disorder of synapse formation in the cerebral cortex and node of Ranvier...
March 14, 2017: Journal of Neuroinflammation
https://www.readbyqxmd.com/read/28283543/kcnq-smit-complex-formation-facilitates-ion-channel-solute-transporter-cross-talk
#8
Daniel L Neverisky, Geoffrey W Abbott
Voltage-gated potassium channels formed by KCNQ2 and KCNQ3 are essential for normal neuronal excitability. KCNQ2/3 channel activity is augmented in vivo by phosphatidylinositol 4,5-bisphosphate (PIP2), which is generated from myo-inositol, an osmolyte transported into cells by sodium-dependent myo-inositol transporters (SMITs). Here, we discovered that KCNQ2/3 channels isoform-specifically colocalize with SMIT1 and SMIT2 at sciatic nerve nodes of Ranvier and in axon initial segments, and form channel-transporter complexes in vitro and in vivo KCNQ2/3 coexpression protected SMIT1 activity from the otherwise inhibitory effects of cellular depolarization imposed by elevating extracellular [K(+)], and KCNQ2 was required for potentiation of SMIT activity by myo-inositol preincubation...
March 10, 2017: FASEB Journal: Official Publication of the Federation of American Societies for Experimental Biology
https://www.readbyqxmd.com/read/28235672/neurobiology-of-axonal-transport-defects-in-motor-neuron-diseases-opportunities-for-translational-research
#9
REVIEW
Kurt J De Vos, Majid Hafezparast
Intracellular trafficking of cargoes is an essential process to maintain the structure and function of all mammalian cell types, but especially of neurons because of their extreme axon/dendrite polarisation. Axonal transport mediates the movement of cargoes such as proteins, mRNA, lipids, membrane-bound vesicles and organelles that are mostly synthesised in the cell body and in doing so is responsible for their correct spatiotemporal distribution in the axon, for example at specialised sites such as nodes of Ranvier and synaptic terminals...
February 22, 2017: Neurobiology of Disease
https://www.readbyqxmd.com/read/28217083/postnatal-loss-of-neuronal-and-glial-neurofascins-differentially-affects-node-of-ranvier-maintenance-and-myelinated-axon-function
#10
Anna M Taylor, Julia Saifetiarova, Manzoor A Bhat
Intricate molecular interactions between neurons and glial cells underlie the creation of unique domains that are essential for saltatory conduction of action potentials by myelinated axons. Previously, the cell surface adhesion molecule Neurofascin (Nfasc) has been shown to have a dual-role in the establishment of axonal domains from both the glial and neuronal interface. While the neuron-specific isoform of Neurofascin (NF186) is indispensable for clustering of voltage-gated sodium channels at nodes of Ranvier; the glial-specific isoform of Neurofascin (NF155) is required for myelinating glial cells to organize the paranodal domain...
2017: Frontiers in Cellular Neuroscience
https://www.readbyqxmd.com/read/28214515/igg-degrading-enzyme-of-streptococcus-pyogenes-ides-prevents-disease-progression-and-facilitates-improvement-in-a-rabbit-model-of-guillain-barr%C3%A3-syndrome
#11
Yuzhong Wang, Qiguang Shi, Hui Lv, Ming Hu, Weifang Wang, Quanquan Wang, Baojun Qiao, Guorong Zhang, Zhanyun Lv, Christian Kjellman, Sofia Järnum, Lena Winstedt, Yong Zhang, Jiao Wen, Yanlei Hao, Nobuhiro Yuki
Autoantibodies binding to peripheral nerves followed by complement deposition and membrane attack complex formation results in nerve damage in Guillain-Barré syndrome (GBS). Strategies to remove the pathogenic autoantibodies or block the complement deposition benefit most patients with GBS. Immunoglobulin G-degrading enzyme of Streptococcus pyogenes (IdeS) is a cysteine protease which cleaves IgG antibodies into F(ab')2 and Fc fragments. In this study, using a rabbit model of axonal GBS, acute motor axonal neuropathy (AMAN), we demonstrated that IdeS treatment significantly reduced the disruption of Nav channels as well as activated C3 deposition at the anterior spinal root nodes of Ranvier in AMAN rabbits...
February 16, 2017: Experimental Neurology
https://www.readbyqxmd.com/read/28199072/genetic-risk-score-analysis-in-early-onset-bipolar-disorder
#12
Paul E Croarkin, Joan L Luby, Kelly Cercy, Jennifer R Geske, Marin Veldic, Matthew Simonson, Paramjit T Joshi, Karen Dineen Wagner, John T Walkup, Malik M Nassan, Alfredo B Cuellar-Barboza, Leah Casuto, Susan L McElroy, Peter S Jensen, Mark A Frye, Joanna M Biernacka
OBJECTIVE: In this study, we performed a candidate genetic risk score (GRS) analysis of early-onset bipolar disorder (BD). METHODS: Treatment of Early Age Mania (TEAM) study enrollment and sample collection took place from 2003 to 2008. Mayo Clinic Bipolar Biobank samples were collected from 2009 to 2013. Genotyping and analyses for the present study took place from 2013 to 2014. The diagnosis of BD was based on Diagnostic and Statistical Manual of Mental Disorders, Fourth Edition, Text Revision criteria...
February 14, 2017: Journal of Clinical Psychiatry
https://www.readbyqxmd.com/read/28155939/proposal-of-a-new-mechanism-for-the-directional-propagation-of-the-action-potential-using-a-mimicking-system
#13
Y Takano, O Shirai, Y Kitazumi, K Kano
A nerve conduction model is constructed by using some liquid-membrane cells that mimic the function of the K(+) and Na(+) channels. By imitating two types of Na(+) channels (ligand-gated Na(+) channels and voltage-gated Na(+) channels), a new mechanism for the directional propagation of the action potential along the axon toward the axon terminal is proposed. When the nerve cell is excited by an external (outer) stimulus, it can be presumed that the ligand-gated channels work as power sources at the synapse to propagate the change in the membrane potential, and then the voltage-gated channels locally assist the propagation at each site of the axon (nodes of Ranvier)...
February 3, 2017: Physical Chemistry Chemical Physics: PCCP
https://www.readbyqxmd.com/read/28148727/early-and-late-loss-of-the-cytoskeletal-scaffolding-protein-ankyrin-g-reveals-its-role-in-maturation-and-maintenance-of-nodes-of-ranvier-in-myelinated-axons
#14
Julia Saifetiarova, Anna M Taylor, Manzoor A Bhat
The mechanisms that govern node of Ranvier organization, stability, and long-term maintenance remain to be fully elucidated. One of the molecular components of the node is the cytoskeletal scaffolding protein, ankyrin G (AnkG), which interacts with multiple members of the nodal complex. The role of AnkG in nodal organization and maintenance is still not clearly defined as to whether AnkG functions as an initial nodal organizer or whether it functions as a nodal stabilizer after the nodal complex has been assembled...
March 8, 2017: Journal of Neuroscience: the Official Journal of the Society for Neuroscience
https://www.readbyqxmd.com/read/28134616/the-paranodal-cytoskeleton-clusters-na-channels-at-nodes-of-ranvier
#15
Veronique Amor, Chuansheng Zhang, Anna Vainshtein, Ao Zhang, Daniel R Zollinger, Yael Eshed-Eisenbach, Peter J Brophy, Matthew N Rasband, Elior Peles
A high density of Na(+) channels at nodes of Ranvier is necessary for rapid and efficient action potential propagation in myelinated axons. Na+ channel clustering is thought to depend on two axonal cell adhesion molecules that mediate interactions between the axon and myelinating glia at the nodal gap (i.e., NF186) and the paranodal junction (i.e., Caspr). Here we show that while Na(+) channels cluster at nodes in the absence of NF186, they fail to do so in double conditional knockout mice lacking both NF186 and the paranodal cell adhesion molecule Caspr, demonstrating that a paranodal junction-dependent mechanism can cluster Na(+) channels at nodes...
January 30, 2017: ELife
https://www.readbyqxmd.com/read/28130923/node-of-ranvier-length-as-a-potential-regulator-of-myelinated-axon-conduction-speed
#16
I Lorena Arancibia-Cárcamo, Marc C Ford, Lee Cossell, Kinji Ishida, Koujiro Tohyama, David Attwell
Myelination speeds conduction of the nerve impulse, enhancing cognitive power. Changes of white matter structure contribute to learning, and are often assumed to reflect an altered number of myelin wraps. We now show that, in rat optic nerve and cerebral cortical axons, the node of Ranvier length varies over a 4.4-fold and 8.7-fold range respectively and that variation of the node length is much less along axons than between axons. Modelling predicts that these node length differences will alter conduction speed by ~20%, similar to the changes produced by altering the number of myelin wraps or the internode length...
January 28, 2017: ELife
https://www.readbyqxmd.com/read/28073817/paranodal-dissection-in-chronic-inflammatory-demyelinating-polyneuropathy-with-antineurofascin-155-and-anticontactin-1-antibodies
#17
Haruki Koike, Masato Kadoya, Ken-Ichi Kaida, Shohei Ikeda, Yuichi Kawagashira, Masahiro Iijima, Daisuke Kato, Hidenori Ogata, Ryo Yamasaki, Noriyuki Matsukawa, Jun-Ichi Kira, Masahisa Katsuno, Gen Sobue
OBJECTIVE: To investigate the morphological features of chronic inflammatory demyelinating polyneuropathy (CIDP) with autoantibodies directed against paranodal junctional molecules, particularly focusing on the fine structures of the paranodes. METHODS: We assessed sural nerve biopsy specimens obtained from 9 patients with CIDP with antineurofascin-155 antibodies and 1 patient with anticontactin-1 antibodies. 13 patients with CIDP without these antibodies were also examined to compare pathological findings...
January 10, 2017: Journal of Neurology, Neurosurgery, and Psychiatry
https://www.readbyqxmd.com/read/28064059/par1-activation-affects-the-neurotrophic-properties-of-schwann-cells
#18
Elena Pompili, Cinzia Fabrizi, Francesca Somma, Virginia Correani, Bruno Maras, Maria Eugenia Schininà, Viviana Ciraci, Marco Artico, Francesco Fornai, Lorenzo Fumagalli
Protease-activated receptor-1 (PAR1) is the prototypic member of a family of four G-protein-coupled receptors that signal in response to extracellular proteases. In the peripheral nervous system, the expression and/or the role of PARs are still poorly investigated. High PAR1 mRNA expression was found in the rat dorsal root ganglia and the signal intensity of PAR1 mRNA increased in response to sciatic nerve transection. In the sciatic nerve, functional PAR1 receptor was reported at the level of non-compacted Schwann cell myelin microvilli of the nodes of Ranvier...
January 4, 2017: Molecular and Cellular Neurosciences
https://www.readbyqxmd.com/read/28017237/serum-antibodies-targeting-neurons-of-the-monoaminergic-systems-in-guillain-barr%C3%A3-syndrome
#19
Claudia Rink, Angelika Görtzen, Rüdiger W Veh, Harald Prüss
Guillain-Barré syndrome (GBS) is an autoimmune disease with progressive flaccid paralysis of the extremities. Several auto-antibodies have been identified, binding to myelin, gangliosides, astrocytes or proteins at the nodes of Ranvier. Some epitopes are not confined to the peripheral nerve, suggesting that auto-antibodies may also contribute to symptoms of the central nervous system, which are common in GBS and include anxiety, depression, hallucinations, oneiroid psychosis or fatigue. This notion is supported by treating patients with plasma exchange, resulting in improvement of both central and peripheral symptoms...
January 15, 2017: Journal of the Neurological Sciences
https://www.readbyqxmd.com/read/28008944/amyloid-precursor-protein-modulates-nav1-6-sodium-channel-currents-through-a-go-coupled-jnk-pathway
#20
Shao Li, Xi Wang, Quan-Hong Ma, Wu-Lin Yang, Xiao-Gang Zhang, Gavin S Dawe, Zhi-Cheng Xiao
Amyloid precursor protein (APP), commonly associated with Alzheimer's disease, also marks axonal degeneration. In the recent studies, we demonstrated that APP aggregated at nodes of Ranvier (NORs) in myelinated central nervous system (CNS) axons and interacted with Nav1.6. However, the physiological function of APP remains unknown. In this study, we described reduced sodium current densities in APP knockout hippocampal neurons. Coexpression of APP or its intracellular domains containing a VTPEER motif with Nav1...
December 23, 2016: Scientific Reports
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