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Larisa C Kruger et al.
The Journal of neuroscience : the official journal of the Society for Neuroscience, 36(23), 6213-6224 (2016-06-10)
Voltage-gated sodium channel (VGSC) β subunits signal through multiple pathways on multiple time scales. In addition to modulating sodium and potassium currents, β subunits play nonconducting roles as cell adhesion molecules, which allow them to function in cell-cell communication, neuronal
Kathleen K Catignas et al.
Glia, 69(1), 91-108 (2020-08-04)
In the developing peripheral nervous system, Schwann cells (SCs) extend their processes to contact, sort, and myelinate axons. The mechanisms that contribute to the interaction between SCs and axons are just beginning to be elucidated. Using a SC-neuron coculture system
Ramón Martínez-Mármol et al.
Cardiovascular research, 76(3), 430-441 (2007-09-29)
Cellular cardiomyoplasty using skeletal myoblasts is a promising therapy for myocardial infarct repair. Once transplanted, myoblasts grow, differentiate and adapt their electrophysiological properties towards more cardiac-like phenotypes. Voltage-dependent Na(+) channels (Na(v)) are the main proteins involved in the propagation of
Musaad A Alshammari et al.
Frontiers in cellular neuroscience, 10, 5-5 (2016-02-26)
The axonal initial segment (AIS) is the subcellular compartment required for initiation of the action potential in neurons. Scaffolding and regulatory proteins at the AIS cluster with ion channels ensuring the integrity of electrical signaling. Interference with the configuration of
Feline W Lindhout et al.
eLife, 9 (2020-09-18)
The differentiation of neuronal stem cells into polarized neurons is a well-coordinated process which has mostly been studied in classical non-human model systems, but to what extent these findings are recapitulated in human neurons remains unclear. To study neuronal polarization
Anna-Karin Persson et al.
The Journal of neuroscience : the official journal of the Society for Neuroscience, 33(49), 19250-19261 (2013-12-07)
Axonal degeneration occurs in multiple neurodegenerative disorders of the central and peripheral nervous system. Although the underlying molecular pathways leading to axonal degeneration are incompletely understood, accumulating evidence suggests contributions of impaired mitochondrial function, disrupted axonal transport, and/or dysfunctional intracellular
Benoit Gautier et al.
Nature communications, 12(1), 2356-2356 (2021-04-23)
Charcot-Marie-Tooth disease 1 A (CMT1A) results from a duplication of the PMP22 gene in Schwann cells and a deficit of myelination in peripheral nerves. Patients with CMT1A have reduced nerve conduction velocity, muscle wasting, hand and foot deformations and foot drop
Yuki Miyamoto et al.
Molecular biology of the cell, 26(19), 3489-3503 (2015-08-01)
During early development of the peripheral nervous system, Schwann cell precursors proliferate, migrate, and differentiate into premyelinating Schwann cells. After birth, Schwann cells envelop neuronal axons with myelin sheaths. Although some molecular mechanisms underlying myelination by Schwann cells have been
Fabrizia Cesca et al.
The Journal of biological chemistry, 290(29), 18045-18055 (2015-06-04)
Kidins220 (kinase D-interacting substrate of 220 kDa)/ankyrin repeat-rich membrane spanning (ARMS) acts as a signaling platform at the plasma membrane and is implicated in a multitude of neuronal functions, including the control of neuronal activity. Here, we used the Kidins220(-/-)
Pierre-Marie Martin et al.
The Journal of biological chemistry, 292(6), 2441-2456 (2016-12-17)
The nodes of Ranvier are essential regions for action potential conduction in myelinated fibers. They are enriched in multimolecular complexes composed of voltage-gated Nav and Kv7 channels associated with cell adhesion molecules. Cytoskeletal proteins ankyrin-G (AnkG) and βIV-spectrin control the
Louise Ritchie et al.
Neuropharmacology, 135, 1-10 (2018-03-06)
Toll like receptor 3 (TLR3) belongs to a family of pattern recognition receptors that recognise molecules found on pathogens referred to as pathogen associated molecular patterns (PAMPs). Its involvement in innate immunity is well known but despite its presence in
Davide Aprile et al.
Cell death and differentiation, 26(11), 2464-2478 (2019-03-13)
Mutations in TBC1D24 are described in patients with a spectrum of neurological diseases, including mild and severe epilepsies and complex syndromic phenotypes such as Deafness, Onycodystrophy, Osteodystrophy, Mental Retardation and Seizure (DOORS) syndrome. The product of TBC1D24 is a multifunctional
Maxime Lorenzini et al.
The Journal of general physiology, 153(2) (2021-01-08)
Phosphorylation of the voltage-gated Na+ (NaV) channel NaV1.5 regulates cardiac excitability, yet the phosphorylation sites regulating its function and the underlying mechanisms remain largely unknown. Using a systematic, quantitative phosphoproteomic approach, we analyzed NaV1.5 channel complexes purified from nonfailing and
Ryan B Griggs et al.
ASN neuro, 10, 1759091418766175-1759091418766175 (2018-04-21)
Nodes of Ranvier and associated paranodal and juxtaparanodal domains along myelinated axons are essential for normal function of the peripheral and central nervous systems. Disruption of these domains as well as increases in the reactive carbonyl species methylglyoxal are implicated
Young Gi Min et al.
Journal of neuropathology and experimental neurology, 81(12), 1018-1025 (2022-09-23)
Immune-mediated neuropathies are a heterogenous group of inflammatory peripheral nerve disorders. They can be classified according to the domain where the autoimmune process begins: the internode, paranode, or node. However, conventional diagnostic tools, electrodiagnosis (EDX), and autoantibody testing do not
Maria Eirini Terzenidou et al.
PLoS genetics, 13(4), e1006656-e1006656 (2017-04-05)
The mitochondrial protein SLC25A46 has been recently identified as a novel pathogenic cause in a wide spectrum of neurological diseases, including inherited optic atrophy, Charcot-Marie-Tooth type 2, Leigh syndrome, progressive myoclonic ataxia and lethal congenital pontocerebellar hypoplasia. SLC25A46 is an
Annisa N Chand et al.
The Journal of neuroscience : the official journal of the Society for Neuroscience, 35(4), 1573-1590 (2015-01-30)
The axon initial segment (AIS) is a specialized structure near the start of the axon that is a site of neuronal plasticity. Changes in activity levels in vitro and in vivo can produce structural AIS changes in excitatory cells that
Katarzyna Dover et al.
Nature communications, 7, 12895-12895 (2016-09-27)
Neurons in vertebrate central nervous systems initiate and conduct sodium action potentials in distinct subcellular compartments that differ architecturally and electrically. Here, we report several unanticipated passive and active properties of the cerebellar granule cell's unmyelinated axon. Whereas spike initiation
Lakshmi Srinivasan et al.
The Journal of biological chemistry, 298(4), 101763-101763 (2022-02-25)
Voltage-gated sodium channels, NaVs, are responsible for the rapid rise of action potentials in excitable tissues. NaV channel mutations have been implicated in several human genetic diseases, such as hypokalemic periodic paralysis, myotonia, and long-QT and Brugada syndromes. Here, we
Takuya Hayashi et al.
The Journal of clinical investigation, 123(1), 272-284 (2012-12-04)
A cell-based therapy for the replacement of dopaminergic neurons has been a long-term goal in Parkinson's disease research. Here, we show that autologous engraftment of A9 dopaminergic neuron-like cells induced from mesenchymal stem cells (MSCs) leads to long-term survival of
Mario A Saporta et al.
Methods in molecular biology (Clifton, N.J.), 1739, 359-369 (2018-03-17)
The human skin is richly innervated by nerve fibers of different calibers and functions, including thickly myelinated large fibers that act as afferents for mechanoreceptors in the dermal papillae. Skin biopsies offer minimally invasive access to these myelinated fibers, in
Peter Dongmin Sohn et al.
Neuron, 104(3), 458-470 (2019-09-23)
Dysregulation of neuronal excitability underlies the pathogenesis of tauopathies, including frontotemporal dementia (FTD) with tau inclusions. A majority of FTD-causing tau mutations are located in the microtubule-binding domain, but how these mutations alter neuronal excitability is largely unknown. Here, using
Kaveh Mirmoeini et al.
Investigative ophthalmology & visual science, 64(4), 7-7 (2023-04-11)
Corneal sensory nerves protect the cornea from injury. They are also thought to stimulate limbal stem cells (LSCs) to produce transparent epithelial cells constantly, enabling vision. In other organs, Schwann cells (SCs) associated with tissue-innervating axon terminals mediate tissue regeneration.
Mourad Bouzidi et al.
The Journal of biological chemistry, 277(32), 28996-29004 (2002-05-31)
Voltage-dependant sodium channels at the axon initial segment and nodes of Ranvier colocalize with the nodal isoforms of ankyrin(G) (Ank(G) node). Using fusion proteins derived from the intracellular regions of the Nav1.2a subunit and the Ank repeat domain of Ank(G)
Anne-Laure Dubessy et al.
Glia, 67(12), 2248-2263 (2019-07-23)
The fast and reliable propagation of action potentials along myelinated fibers relies on the clustering of voltage-gated sodium channels at nodes of Ranvier. Axo-glial communication is required for assembly of nodal proteins in the central nervous system, yet the underlying
Andrew A Jarjour et al.
Glia, 68(9), 1840-1858 (2020-03-04)
During axonal ensheathment, noncompact myelin channels formed at lateral edges of the myelinating process become arranged into tight paranodal spirals that resemble loops when cut in cross section. These adhere to the axon, concentrating voltage-dependent sodium channels at nodes of
Chih H King et al.
The Journal of comparative neurology, 522(14), 3262-3280 (2014-04-02)
The Kv7 (KCNQ) family of voltage-gated K(+) channels regulates cellular excitability. The functional role of Kv7.2 has been hampered by the lack of a viable Kcnq2-null animal model. In this study, we generated homozygous Kcnq2-null sensory neurons using the Cre-Lox
Wei Zhang et al.
Molecular neurobiology, 58(12), 6153-6169 (2021-08-31)
The axon initial segment (AIS) is essential for maintaining neuronal polarity, modulating protein transport into the axon, and action potential generation. These functions are supported by a distinctive actin and microtubule cytoskeleton that controls axonal trafficking and maintains a high
Laura Dumas et al.
Glia, 63(4), 699-717 (2014-12-23)
Oligodendrocytes are the myelinating cells of the central nervous system. Multiple markers are available to analyze the populations of oligodendroglial cells and their precursors during development and in pathological conditions. However, the behavior of oligodendrocytes remains poorly characterized in vivo
Laura F Gumy et al.
Neuron, 94(2), 347-362 (2017-04-21)
Polarized cargo transport is essential for neuronal function. However, the minimal basic components required for selective cargo sorting and distribution in neurons remain elusive. We found that in sensory neurons the axon initial segment is largely absent and that microtubule-associated
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