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Amyotrophic Lateral Sclerosis as a Systemic Disease 2.0
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Amyotrophic Lateral Sclerosis as a Systemic Disease 2.0

A special issue of International Journal of Molecular Sciences (ISSN 1422-0067). This special issue belongs to the section "Molecular Neurobiology".

Deadline for manuscript submissions: 20 February 2025 | Viewed by 11800

Special Issue Editor

Dr. Viviana Moresi

E-Mail Website
Guest Editor
Unit of Histology and Medical Embryology, Department of Anatomy, Histology, Forensic Medicine and Orthopaedics, Sapienza University of Rome, 00185 Rome, Italy
Interests: molecular mechanisms underlying skeletal muscle homeostasis in pathophysiological conditions; epigenetics; cancer-induced cachexia; denervation; muscular dystrophy
Special Issues, Collections and Topics in MDPI journals

Special Issue Information

Dear Colleagues, 

Amyotrophic lateral sclerosis (ALS) is a lethal neurodegenerative disease affecting both the upper and lower motoneurons, which leads to a progressive paralysis of the voluntary muscles. Despite being studied for decades, ALS etiology, diagnosis and pathogenesis remain largely unclear. The only pharmacological option approved (i.e., riluzole) provides only a slight increase in life expectancy, despite presenting numerous side effects. Although the primary cause is the motoneuron’s death, ASL must be considered a multisystemic syndrome, in which multiple organs and tissues participate and contribute to disease progression. Cross-talk between different types of cells and retrograde signals, from peripheral tissues to the central nervous system, are emerging as essential contributors to the ALS process.

In this Special Issue, we aim to collect new findings underlying ALS etiology and diagnosis, including the search for reliable biomarkers. Special attention is pointed towards new frontiers of targeting and multidisciplinary approaches to counteract ALS progression, including cell-based research and therapy, nutritional intervention to counteract altered body and muscle metabolism, gastrointestinal status and microbiota. 

Dr. Viviana Moresi
Guest Editor

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Keywords

  • neurogenic muscle atrophy
  • retrograde signals
  • crosstalk
  • multi-system biomarkers
  • neuroinflammation
  • neurodegeneration
  • hypermetabolism

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Related Special Issue

Published Papers (4 papers)

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Research

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19 pages, 2725 KiB  
Article
Insight into Tetramolecular DNA G-Quadruplexes Associated with ALS and FTLD: Cation Interactions and Formation of Higher-Ordered Structure
by Matja Zalar, Baifan Wang, Janez Plavec and Primož Šket
Int. J. Mol. Sci. 2023, 24(17), 13437; https://doi.org/10.3390/ijms241713437 - 30 Aug 2023
Cited by 1 | Viewed by 2139
Abstract
The G4C2 hexanucleotide repeat expansion in the c9orf72 gene is a major genetic cause of familial amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD), with the formation of G-quadruplexes directly linked to the development of these diseases. Cations play [...] Read more.
The G4C2 hexanucleotide repeat expansion in the c9orf72 gene is a major genetic cause of familial amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration (FTLD), with the formation of G-quadruplexes directly linked to the development of these diseases. Cations play a crucial role in the formation and structure of G-quadruplexes. In this study, we investigated the impact of biologically relevant potassium ions on G-quadruplex structures and utilized 15N-labeled ammonium cations as a substitute for K+ ions to gain further insights into cation binding and exchange dynamics. Through nuclear magnetic resonance spectroscopy and molecular dynamics simulations, we demonstrate that the single d(G4C2) repeat, in the presence of 15NH4+ ions, adopts a tetramolecular G-quadruplex with an all-syn quartet at the 5′-end. The movement of 15NH4+ ions through the central channel of the G-quadruplex, as well as to the bulk solution, is governed by the vacant cation binding site, in addition to the all-syn quartet at the 5′-end. Furthermore, the addition of K+ ions to G-quadruplexes folded in the presence of 15NH4+ ions induces stacking of G-quadruplexes via their 5′-end G-quartets, leading to the formation of stable higher-ordered species. Full article
(This article belongs to the Special Issue Amyotrophic Lateral Sclerosis as a Systemic Disease 2.0)
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12 pages, 6549 KiB  
Article
Creatine Kinase MB Isoenzyme Is a Complementary Biomarker in Amyotrophic Lateral Sclerosis
by Natsinee Kittipeerapat, Rachel Fabian, Sarah Bernsen, Patrick Weydt and Sergio Castro-Gomez
Int. J. Mol. Sci. 2023, 24(14), 11682; https://doi.org/10.3390/ijms241411682 - 20 Jul 2023
Cited by 6 | Viewed by 3847
Abstract
Amyotrophic lateral sclerosis (ALS) is an invariably fatal neurodegenerative disease with limited therapeutic options. There is an urgent need for novel biomarkers to be used as surrogates for new therapeutic trials and disease monitoring. In this study, we sought to systematically study creatine [...] Read more.
Amyotrophic lateral sclerosis (ALS) is an invariably fatal neurodegenerative disease with limited therapeutic options. There is an urgent need for novel biomarkers to be used as surrogates for new therapeutic trials and disease monitoring. In this study, we sought to systematically study creatine kinase isoenzyme MB (CK-MB) in a real-world cohort of ALS patients, assess the diagnostic performance, and evaluate its association with other laboratory and clinical parameters. We reviewed data from 194 consecutive patients that included 130 ALS patients and 64 disease control patients (primary lateral sclerosis [PLS], benign fasciculations syndrome [BFS], Huntington’s disease [HD] and Alzheimer’s disease [AD]). CK-MB was elevated in the sera of more than half of all patients with ALS. In patients with spinal-onset ALS, CK-MB levels were significantly higher than in patients with other neurodegenerative diseases. Patients with slower rates of functional decline had a significantly higher baseline CK-MB. Furthermore, CK-MB elevations correlated with cardiac troponin T (cTnT) and with revised ALS Functional Rating Scale (ALSFRS-R) bulbar subcategory. We posit that measuring CK-MB in ALS patients in a complimentary fashion could potentially aid in the diagnostic workup of ALS and help discriminate the disease from some ALS mimics and other neurodegenerative diseases. CK-MB levels also may provide valuable prognostic information regarding disease aggressiveness as well as correlations with specific phenotypic presentations. Full article
(This article belongs to the Special Issue Amyotrophic Lateral Sclerosis as a Systemic Disease 2.0)
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Review

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14 pages, 612 KiB  
Review
Can Some Anticancer Drugs Be Repurposed to Treat Amyotrophic Lateral Sclerosis? A Brief Narrative Review
by Rosa Luisa Potenza, Monica Armida and Patrizia Popoli
Int. J. Mol. Sci. 2024, 25(3), 1751; https://doi.org/10.3390/ijms25031751 - 1 Feb 2024
Cited by 2 | Viewed by 1880
Abstract
Amyotrophic lateral sclerosis (ALS) is a rare progressive motor neuron disease that, due to its high complexity, still lacks effective treatments. Development of a new drug is a highly costly and time-consuming process, and the repositioning of approved drugs can represent an efficient [...] Read more.
Amyotrophic lateral sclerosis (ALS) is a rare progressive motor neuron disease that, due to its high complexity, still lacks effective treatments. Development of a new drug is a highly costly and time-consuming process, and the repositioning of approved drugs can represent an efficient strategy to provide therapeutic opportunities. This is particularly true for rare diseases, which are characterised by small patient populations and therefore attract little commercial interest. Based on the overlap between the biological background of cancer and neurodegeneration, the repurposing of antineoplastic drugs for ALS has been suggested. The objective of this narrative review was to summarise the current experimental evidence on the use of approved anticancer drugs in ALS. Specifically, anticancer drugs belonging to different classes were found to act on mechanisms involved in the ALS pathogenesis, and some of them proved to exert beneficial effects in ALS models. However, additional studies are necessary to confirm the real therapeutic potential of anticancer drugs for repositioning in ALS treatment. Full article
(This article belongs to the Special Issue Amyotrophic Lateral Sclerosis as a Systemic Disease 2.0)
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13 pages, 310 KiB  
Review
Dysautonomia in Amyotrophic Lateral Sclerosis
by Alexandra L. Oprisan and Bogdan Ovidiu Popescu
Int. J. Mol. Sci. 2023, 24(19), 14927; https://doi.org/10.3390/ijms241914927 - 5 Oct 2023
Cited by 6 | Viewed by 3137
Abstract
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease, characterized in its typical presentation by a combination of lower and upper motor neuron symptoms, with a progressive course and fatal outcome. Due to increased recognition of the non-motor symptoms, it is currently considered a [...] Read more.
Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease, characterized in its typical presentation by a combination of lower and upper motor neuron symptoms, with a progressive course and fatal outcome. Due to increased recognition of the non-motor symptoms, it is currently considered a multisystem disorder with great heterogeneity, regarding genetical, clinical, and neuropathological features. Often underestimated, autonomic signs and symptoms have been described in patients with ALS, and various method analyses have been used to assess autonomic nervous system involvement. The aim of this paper is to offer a narrative literature review on autonomic disturbances in ALS, based on the scarce data available to date. Full article
(This article belongs to the Special Issue Amyotrophic Lateral Sclerosis as a Systemic Disease 2.0)
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