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Cells
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Brain Channelopathies: From Molecular Mechanisms to Therapeutic Approach
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Brain Channelopathies: From Molecular Mechanisms to Therapeutic Approach

A special issue of Cells (ISSN 2073-4409). This special issue belongs to the section "Cell Signaling".

Deadline for manuscript submissions: closed (15 March 2023) | Viewed by 4055

Special Issue Editor

Prof. Dr. Dominique Debanne

E-Mail Website
Guest Editor
INSERM, Aix Marseille University, UNIS, UMR1072, Marseille, France
Interests: axon physiology; STDP; plasticity of neuronal excitability; ion channels; inhibition
Special Issues, Collections and Topics in MDPI journals

Special Issue Information

Dear Colleagues,

Brain channelopathies are a primary cause of numerous brain disorders, including epilepsy, pain, headache, ataxia, and tinnitus, among others. In most cases, the cause genetic or autoimmune loss of function of voltage-gated or ligand-gated ion channels whose function cannot be compensated
for by other channels sharing a similar function. For instance, loss of potassium channel function was found to be at the origin of temporal lobe epilepsy as well as headaches. Ion channels interact
with regulatory proteins, the absence of which can directly lead to the loss of ion channel function.

In this Special Issue, we expect to shed new light on key cellular and molecular pathways involved in brain channelopathies. We are anticipating contributions from cellular neurophysiologists as well as cellular neurobiologists.

The current Special Issue will accept original studies and state-of-art reviews in the field of brain channelopathies, written by scientists active in the field.

Best wishes,

Prof. Dr. Dominique Debanne
Guest Editor

Manuscript Submission Information

Manuscripts should be submitted online at www.mdpi.com by registering and logging in to this website. Once you are registered, click here to go to the submission form. Manuscripts can be submitted until the deadline. All submissions that pass pre-check are peer-reviewed. Accepted papers will be published continuously in the journal (as soon as accepted) and will be listed together on the special issue website. Research articles, review articles as well as short communications are invited. For planned papers, a title and short abstract (about 100 words) can be sent to the Editorial Office for announcement on this website.

Submitted manuscripts should not have been published previously, nor be under consideration for publication elsewhere (except conference proceedings papers). All manuscripts are thoroughly refereed through a single-blind peer-review process. A guide for authors and other relevant information for submission of manuscripts is available on the Instructions for Authors page. Cells is an international peer-reviewed open access semimonthly journal published by MDPI.

Please visit the Instructions for Authors page before submitting a manuscript. The Article Processing Charge (APC) for publication in this open access journal is 2700 CHF (Swiss Francs). Submitted papers should be well formatted and use good English. Authors may use MDPI's English editing service prior to publication or during author revisions.

Keywords

  • channelopathy
  • voltage-gated ion channels
  • ligand-gated ion channels
  • brain disease
  • epilepsy
  • pain
  • headache
  • ataxia
  • tinnitus

Published Papers (2 papers)

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Research

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16 pages, 5559 KiB  
Article
Basket to Purkinje Cell Inhibitory Ephaptic Coupling Is Abolished in Episodic Ataxia Type 1
by Henry G. S. Martin and Dimitri M. Kullmann
Cells 2023, 12(10), 1382; https://doi.org/10.3390/cells12101382 - 13 May 2023
Viewed by 1546
Abstract
Dominantly inherited missense mutations of the KCNA1 gene, which encodes the KV1.1 potassium channel subunit, cause Episodic Ataxia type 1 (EA1). Although the cerebellar incoordination is thought to arise from abnormal Purkinje cell output, the underlying functional deficit remains unclear. Here [...] Read more.
Dominantly inherited missense mutations of the KCNA1 gene, which encodes the KV1.1 potassium channel subunit, cause Episodic Ataxia type 1 (EA1). Although the cerebellar incoordination is thought to arise from abnormal Purkinje cell output, the underlying functional deficit remains unclear. Here we examine synaptic and non-synaptic inhibition of Purkinje cells by cerebellar basket cells in an adult mouse model of EA1. The synaptic function of basket cell terminals was unaffected, despite their intense enrichment for KV1.1-containing channels. In turn, the phase response curve quantifying the influence of basket cell input on Purkine cell output was maintained. However, ultra-fast non-synaptic ephaptic coupling, which occurs in the cerebellar ‘pinceau’ formation surrounding the axon initial segment of Purkinje cells, was profoundly reduced in EA1 mice in comparison with their wild type littermates. The altered temporal profile of basket cell inhibition of Purkinje cells underlines the importance of Kv1.1 channels for this form of signalling, and may contribute to the clinical phenotype of EA1. Full article
(This article belongs to the Special Issue Brain Channelopathies: From Molecular Mechanisms to Therapeutic Approach)
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Review

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17 pages, 777 KiB  
Review
Contribution of Axon Initial Segment Structure and Channels to Brain Pathology
by Juan José Garrido
Cells 2023, 12(8), 1210; https://doi.org/10.3390/cells12081210 - 21 Apr 2023
Cited by 3 | Viewed by 1951
Abstract
Brain channelopathies are a group of neurological disorders that result from genetic mutations affecting ion channels in the brain. Ion channels are specialized proteins that play a crucial role in the electrical activity of nerve cells by controlling the flow of ions such [...] Read more.
Brain channelopathies are a group of neurological disorders that result from genetic mutations affecting ion channels in the brain. Ion channels are specialized proteins that play a crucial role in the electrical activity of nerve cells by controlling the flow of ions such as sodium, potassium, and calcium. When these channels are not functioning properly, they can cause a wide range of neurological symptoms such as seizures, movement disorders, and cognitive impairment. In this context, the axon initial segment (AIS) is the site of action potential initiation in most neurons. This region is characterized by a high density of voltage-gated sodium channels (VGSCs), which are responsible for the rapid depolarization that occurs when the neuron is stimulated. The AIS is also enriched in other ion channels, such as potassium channels, that play a role in shaping the action potential waveform and determining the firing frequency of the neuron. In addition to ion channels, the AIS contains a complex cytoskeletal structure that helps to anchor the channels in place and regulate their function. Therefore, alterations in this complex structure of ion channels, scaffold proteins, and specialized cytoskeleton may also cause brain channelopathies not necessarily associated with ion channel mutations. This review will focus on how the AISs structure, plasticity, and composition alterations may generate changes in action potentials and neuronal dysfunction leading to brain diseases. AIS function alterations may be the consequence of voltage-gated ion channel mutations, but also may be due to ligand-activated channels and receptors and AIS structural and membrane proteins that support the function of voltage-gated ion channels. Full article
(This article belongs to the Special Issue Brain Channelopathies: From Molecular Mechanisms to Therapeutic Approach)
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