Elucidating the Contribution of Skeletal Muscle Ion Channels to Amyotrophic Lateral Sclerosis in search of new therapeutic options
Open accessAbstract
The discovery of pathogenetic mechanisms is essential to identify new therapeutic approaches in Amyotrophic Lateral Sclerosis (ALS). Here we investigated the role of the most important ion channels in skeletal muscle of an ALS animal model (MLC/SOD1 G93A) carrying a mutated SOD1 exclusively in this tissue, avoiding motor-neuron involvement. Ion channels are fundamental proteins for muscle function, and also to sustain neuromuscular junction and nerve integrity. By a multivariate statistical analysis, using machine learning algorithms, we identified the discriminant genes in MLC/SOD1 G93A mice. Surprisingly, the expression of ClC-1 chloride channel, present only in skeletal muscle, was reduced. Also, the expression of Protein Kinase-C, known to control ClC-1 activity, was increased, causing its inhibition. The functional characterization confirmed the reduction of ClC-1 activity, leading to hyperexcitability and impaired relaxation. The increased expression of ion channel coupled AMPA-receptor may contribute to sustained depolarization and functional impairment. Also, the decreased expression of irisin, a muscle-secreted peptide protecting brain function, may disturb muscle-nerve connection. Interestingly, the in-vitro application of chelerythrine or acetazolamide, restored ClC-1 activity and sarcolemma hyperexcitability in these mice. These findings show that ion channel function impairment in skeletal muscle may lead to motor-neuron increased vulnerability, and opens the possibility to investigate on new compounds as promising therapy.
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A recent in vitro study supported Acetazolamide as a potential treatment for amyotrophic lateral sclerosis.
“Moreover, a recent in vitro study40 supported this drug as a potential treatment for ALS.”
From Genomic atlas of the proteome from brain, CSF and plasma prioritizes proteins implicated in neurological disorders (2021) · cites this paper as [40]
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Myers, C. (2024) · Springer eBooks · doi:10.1007/978-3-031-47065-3_5
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Chloride Channels in Amyotrophic Lateral Sclerosis
Tourtourikov, I., Mitev, V., & Todorova, A. (2026) · Acta Medica Bulgarica · doi:10.2478/amb-2026-0029
- Authors
- Camerino, G. M., Fonzino, A., Conte, E., De Bellis, M., Mele, A., Liantonio, A., Tricarico, D., Tarantino, N., Dobrowolny, G., Musarò, A., Desaphy, J.-F., De Luca, A., & Pierno, S.
- Affiliations
- University of Bari Aldo Moro (IT)
- Italian Institute of Technology (IT)
- Corresponding author
- Pierno, Sabata
- ORCID
- Venue
- Scientific Reports
- Published
- 2019-02-28
- Volume
- 9
- Issue
- 1
- Pages
- 3185-3185
- ISSN
- 2045-2322 (ISSN-L)
- PMC
- PMC6395744
- OpenAlex
- W2917057660
- Cited by
- 38
- References
- 58
- Citations by year
- 2026: 4 · 2025: 2 · 2024: 5 · 2023: 3 · 2022: 7 · 2021: 7 · 2020: 7 · 2019: 3
- FWCI
- 4.06
- Type
- journal article
- Language
- en
- Open access
- gold
- doi.org
- Locations
- Scientific Reports (publishedVersion, cc-by)
- Europe PMC (PubMed Central) (publishedVersion, other-oa)
- PubMed Central (publishedVersion, cc-by)
- PubMed (publishedVersion)
- IRIS Research product catalog (Sapienza University of Rome) (submittedVersion, cc-by)
- DOAJ (DOAJ: Directory of Open Access Journals) (submittedVersion)
- CINECA IRIS Institutional Research Information System (University of Bari Aldo Moro) (submittedVersion, cc-by)
- Funders
- Universita degli Studi di Bari Aldo Moro (FondiAteneo 2015, Es. Fin. Fondi Ateneo 2012)
- Topics
- Health Sciences › Medicine › Neurology › Amyotrophic Lateral Sclerosis Research
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Muscle Physiology and Disorders
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Ion channel regulation and function
- Keywords
- amyotrophic lateral sclerosis, ClC-1 chloride channel, SOD1 G93A mice, protein kinase C, AMPA receptors, irisin, muscle hyperexcitability, muscle relaxation, neuromuscular junction, chelerythrine, acetazolamide, therapeutic target
- MeSH terms
- Superoxide Dismutase-1, Amyotrophic Lateral Sclerosis, Animals, Disease Models, Animal, Fibronectins, Male, Mice, Inbred C57BL, Mice, Transgenic, Receptors, AMPA, Chloride Channels, Muscle, Skeletal, Mice
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