Sodium Channelopathies of the Central Nervous System

2019 ◽  
Author(s):  
Paul G. DeCaen ◽  
Alfred L. George ◽  
Christopher H. Thompson
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Sodium Channel ◽  
Sodium Channels ◽  
Channel Structure ◽  
Atomic Scale ◽  
Voltage Gated Sodium Channels ◽  
Functional Consequences ◽  
The Central Nervous System ◽  
Consequences Of Disease

This chapter presents information about the structure, function, and molecular genetics of voltage-gated sodium channels expressed in the central nervous system. Sodium channels are essential for the generation and propagation of neuronal action potentials. Recent advances in structural biology have provided atomic-scale descriptions of sodium channel structure that can be related to specific functional properties. We further discuss cellular and subcellular localization, as well as the primary physiological functions mediated by sodium channels within the central nervous system. Finally, this chapter examines the association of various sodium channel isoforms with common brain disorders, including epilepsy, autism, and migraine, and explains the range of functional consequences of disease-associated mutations that are correlated with diverse human phenotypes.

Voltage-gated sodium channels and pain

e-Neuroforum ◽  
2017 ◽  
Vol 23 (3) ◽  
Author(s):  
Carla Nau ◽  
Enrico Leipold
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Sodium Channels ◽  
Action Potentials ◽  
Afferent Pathways ◽  
Voltage Gated ◽  
Voltage Gated Sodium Channels ◽  
The Central Nervous System

AbstractPainful stimuli are detected by specialized neurons, nociceptors, and are translated into action potentials, that are conducted along afferent pathways into the central nervous system, where they are conceived as pain. Voltage-gated sodium channels (Na

scholarly journals Morpho-Functional Consequences of Swiss Cheese Knockdown in Glia of Drosophila melanogaster

Cells ◽  
2021 ◽  
Vol 10 (3) ◽  
pp. 529
Author(s):  
Elena V. Ryabova ◽  
Pavel A. Melentev ◽  
Artem E. Komissarov ◽  
Nina V. Surina ◽  
Ekaterina A. Ivanova ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Drosophila Melanogaster ◽  
Peripheral Nervous System ◽  
Neuronal Cell ◽  
Cortical Region ◽  
Swiss Cheese ◽  
Neuronal Cell Bodies ◽  
Functional Consequences ◽  
The Central Nervous System

Glia are crucial for the normal development and functioning of the nervous system in many animals. Insects are widely used for studies of glia genetics and physiology. Drosophila melanogaster surface glia (perineurial and subperineurial) form a blood–brain barrier in the central nervous system and blood–nerve barrier in the peripheral nervous system. Under the subperineurial glia layer, in the cortical region of the central nervous system, cortex glia encapsulate neuronal cell bodies, whilst in the peripheral nervous system, wrapping glia ensheath axons of peripheral nerves. Here, we show that the expression of the evolutionarily conserved swiss cheese gene is important in several types of glia. swiss cheese knockdown in subperineurial glia leads to morphological abnormalities of these cells. We found that the number of subperineurial glia nuclei is reduced under swiss cheese knockdown, possibly due to apoptosis. In addition, the downregulation of swiss cheese in wrapping glia causes a loss of its integrity. We reveal transcriptome changes under swiss cheese knockdown in subperineurial glia and in cortex + wrapping glia and show that the downregulation of swiss cheese in these types of glia provokes reactive oxygen species acceleration. These results are accompanied by a decline in animal mobility measured by the negative geotaxis performance assay.

scholarly journals Glial and neuronal isoforms of Neurofascin have distinct roles in the assembly of nodes of Ranvier in the central nervous system

2008 ◽  
Vol 181 (7) ◽  
pp. 1169-1177 ◽  
Author(s):  
Barbara Zonta ◽  
Steven Tait ◽  
Shona Melrose ◽  
Heather Anderson ◽  
Sheila Harroch ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Sodium Channels ◽  
Molecular Mechanisms ◽  
Nerve Impulse ◽  
Node Of Ranvier ◽  
Nodes Of Ranvier ◽  
Additional Function ◽  
The Central Nervous System ◽  
Adhesion Complex

Rapid nerve impulse conduction in myelinated axons requires the concentration of voltage-gated sodium channels at nodes of Ranvier. Myelin-forming oligodendrocytes in the central nervous system (CNS) induce the clustering of sodium channels into nodal complexes flanked by paranodal axoglial junctions. However, the molecular mechanisms for nodal complex assembly in the CNS are unknown. Two isoforms of Neurofascin, neuronal Nfasc186 and glial Nfasc155, are components of the nodal and paranodal complexes, respectively. Neurofascin-null mice have disrupted nodal and paranodal complexes. We show that transgenic Nfasc186 can rescue the nodal complex when expressed in Nfasc−/− mice in the absence of the Nfasc155–Caspr–Contactin adhesion complex. Reconstitution of the axoglial adhesion complex by expressing transgenic Nfasc155 in oligodendrocytes also rescues the nodal complex independently of Nfasc186. Furthermore, the Nfasc155 adhesion complex has an additional function in promoting the migration of myelinating processes along CNS axons. We propose that glial and neuronal Neurofascins have distinct functions in the assembly of the CNS node of Ranvier.

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