Pathophysiology of ion channels in amyotrophic lateral sclerosis

0579977 - ÚOCHB 2024 RIV GB eng J - Journal Article
Stringer, Robin Nicholas - Weiss, N.
Pathophysiology of ion channels in amyotrophic lateral sclerosis.
Molecular Brain. Roč. 16, č. 1 (2023), č. článku 82. E-ISSN 1756-6606
R&D Projects: GA MŠMT(CZ) LX22NPO5104
Institutional support: RVO:61388963
Keywords : amyotrophic lateral sclerosis * motor neurons * ion channels * neuronal excitability * neurodegeneration
OECD category: Neurosciences (including psychophysiology
Impact factor: 3.6, year: 2022
Method of publishing: Open access
https://doi.org/10.1186/s13041-023-01070-6

Amyotrophic lateral sclerosis (ALS) stands as the most prevalent and severe form of motor neuron disease, affecting an estimated 2 in 100,000 individuals worldwide. It is characterized by the progressive loss of cortical, brainstem, and spinal motor neurons, ultimately resulting in muscle weakness and death. Although the etiology of ALS remains poorly understood in most cases, the remodelling of ion channels and alteration in neuronal excitability represent a hallmark of the disease, manifesting not only during the symptomatic period but also in the early pre-symptomatic stages. In this review, we delve into these alterations observed in ALS patients and preclinical disease models, and explore their consequences on neuronal activities. Furthermore, we discuss the potential of ion channels as therapeutic targets in the context of ALS.
Permanent Link: https://hdl.handle.net/11104/0348768