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Mitochondrial Dysfunction in Aging and Diseases Affecting the Neuromuscular System
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Mitochondrial Dysfunction in Aging and Diseases Affecting the Neuromuscular System

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

Deadline for manuscript submissions: closed (30 April 2024) | Viewed by 2176

Special Issue Editor

Dr. Rosario Gulino

E-Mail Website
Guest Editor
Department of Biomedical and Biotechnological Sciences, University of Catania, Via Santa Sofia 97, 95123 Catania, Italy
Interests: neuroscience; neural plasticity; amyotrophic lateral sclerosis; motoneuron; regenerative medicine
Special Issues, Collections and Topics in MDPI journals

Special Issue Information

Dear Colleagues,

Mitochondria are fundamental organelles of eukaryotic cells. In addition to being classically considered as the energy centers producing ATP for cell activities, mitochondria are involved in a large plethora of cell functions and, consequently, also in a large number of diseases. Oxidation of metabolites and ATP production, as well as calcium buffering, production of reactive oxygen species, apoptosis, and proteostasis, are among the numerous processes involving mitochondria and affecting cellular homeostasis. Mitochondrial functions are regulated in a complex manner, by controlling the number, morphology, and size of mitochondria, as well as their subcellular localization. These organelles are characterized by high plasticity, but they are also highly sensitive to aging and environmental insults that can induce mitochondrial dysfunctions and ultimately lead to disruptions of cellular homeostasis and disease. Due to their high energy requirement, neurons and skeletal myocytes are among the cell types with higher vulnerability to mitochondrial dysregulation. A large amount of experimental evidence has shown that a number of muscular and neurodegenerative diseases are characterized by alterations of mitochondrial fitness, including amyotrophic lateral sclerosis, spinal muscular atrophy, myasthenia gravis, muscular dystrophy, sarcopenia, as well as other diseases affecting the neuromuscular system, with aging-related, inflammatory, genetic or vascular origin. In this Special Issue, we would like to collect recent evidence concerning the involvement of mitochondrial physiology in the neuromuscular system, as well as studies focusing to the role of mitochondrial dysfunctions as disease mechanisms or therapeutic targets. Original articles, comprehensive reviews, systematic reviews with meta-analysis, or perspective articles are welcome.

Dr. Rosario Gulino
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. International Journal of Molecular Sciences is an international peer-reviewed open access semimonthly journal published by MDPI.

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Keywords

  • mitochondrial physiology

  • mitochondria in ALS
  • mitochondria in SMA
  • mitochondria in neuroinflammation
  • mitochondria in muscular dystrophy
  • mitochondria in sarcopenia
  • mitochondria in myasthenia gravis
  • mitochondria in muscle physiology
  • mitochondria and motoneurons
  • mitochondria and therapy

Published Papers (3 papers)

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Research

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17 pages, 5095 KiB  
Article
Mitigating the Functional Deficit after Neurotoxic Motoneuronal Loss by an Inhibitor of Mitochondrial Fission
by Maria Ciuro, Maria Sangiorgio, Valeria Cacciato, Giuliano Cantone, Carlo Fichera, Lucia Salvatorelli, Gaetano Magro, Giampiero Leanza, Michele Vecchio, Maria Stella Valle and Rosario Gulino
Int. J. Mol. Sci. 2024, 25(13), 7059; https://doi.org/10.3390/ijms25137059 (registering DOI) - 27 Jun 2024
Viewed by 522
Abstract
Amyotrophic lateral sclerosis (ALS) is an extremely complex neurodegenerative disease involving different cell types, but motoneuronal loss represents its main pathological feature. Moreover, compensatory plastic changes taking place in parallel to neurodegeneration are likely to affect the timing of ALS onset and progression [...] Read more.
Amyotrophic lateral sclerosis (ALS) is an extremely complex neurodegenerative disease involving different cell types, but motoneuronal loss represents its main pathological feature. Moreover, compensatory plastic changes taking place in parallel to neurodegeneration are likely to affect the timing of ALS onset and progression and, interestingly, they might represent a promising target for disease-modifying treatments. Therefore, a simplified animal model mimicking motoneuronal loss without the other pathological aspects of ALS has been established by means of intramuscular injection of cholera toxin-B saporin (CTB-Sap), which is a targeted neurotoxin able to kill motoneurons by retrograde suicide transport. Previous studies employing the mouse CTB-Sap model have proven that spontaneous motor recovery is possible after a subtotal removal of a spinal motoneuronal pool. Although these kinds of plastic changes are not enough to counteract the functional effects of the progressive motoneuron degeneration, it would nevertheless represent a promising target for treatments aiming to postpone ALS onset and/or delay disease progression. Herein, the mouse CTB-Sap model has been used to test the efficacy of mitochondrial division inhibitor 1 (Mdivi-1) as a tool to counteract the CTB-Sap toxicity and/or to promote neuroplasticity. The homeostasis of mitochondrial fission/fusion dynamics is indeed important for cell integrity, and it could be affected during neurodegeneration. Lesioned mice were treated with Mdivi-1 and then examined by a series of behavioral test and histological analyses. The results have shown that the drug may be capable of reducing functional deficits after the lesion and promoting synaptic plasticity and neuroprotection, thus representing a putative translational approach for motoneuron disorders. Full article
(This article belongs to the Special Issue Mitochondrial Dysfunction in Aging and Diseases Affecting the Neuromuscular System)
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18 pages, 1977 KiB  
Article
Pathophysiology of Cerebellar Degeneration in Mitochondrial Disorders: Insights from the Harlequin Mouse
by Miguel Fernández de la Torre, Carmen Fiuza-Luces, Sara Laine-Menéndez, Aitor Delmiro, Joaquín Arenas, Miguel Ángel Martín, Alejandro Lucia and María Morán
Int. J. Mol. Sci. 2023, 24(13), 10973; https://doi.org/10.3390/ijms241310973 - 30 Jun 2023
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Abstract
By means of a proteomic approach, we assessed the pathways involved in cerebellar neurodegeneration in a mouse model (Harlequin, Hq) of mitochondrial disorder. A differential proteomic profile study (iTRAQ) was performed in cerebellum homogenates of male Hq and wild-type (WT) mice [...] Read more.
By means of a proteomic approach, we assessed the pathways involved in cerebellar neurodegeneration in a mouse model (Harlequin, Hq) of mitochondrial disorder. A differential proteomic profile study (iTRAQ) was performed in cerebellum homogenates of male Hq and wild-type (WT) mice 8 weeks after the onset of clear symptoms of ataxia in the Hq mice (aged 5.2 ± 0.2 and 5.3 ± 0.1 months for WT and Hq, respectively), followed by a biochemical validation of the most relevant changes. Additional groups of 2-, 3- and 6-month-old WT and Hq mice were analyzed to assess the disease progression on the proteins altered in the proteomic study. The proteomic analysis showed that beyond the expected deregulation of oxidative phosphorylation, the cerebellum of Hq mice showed a marked astroglial activation together with alterations in Ca2+ homeostasis and neurotransmission, with an up- and downregulation of GABAergic and glutamatergic neurotransmission, respectively, and the downregulation of cerebellar “long-term depression”, a synaptic plasticity phenomenon that is a major player in the error-driven learning that occurs in the cerebellar cortex. Our study provides novel insights into the mechanisms associated with cerebellar degeneration in the Hq mouse model, including a complex deregulation of neuroinflammation, oxidative phosphorylation and glutamate, GABA and amino acids’ metabolism Full article
(This article belongs to the Special Issue Mitochondrial Dysfunction in Aging and Diseases Affecting the Neuromuscular System)
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Review

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22 pages, 1960 KiB  
Review
Resveratrol and Vitamin D: Eclectic Molecules Promoting Mitochondrial Health in Sarcopenia
by Cristina Russo, Maria Stella Valle, Floriana D’Angeli, Sofia Surdo and Lucia Malaguarnera
Int. J. Mol. Sci. 2024, 25(14), 7503; https://doi.org/10.3390/ijms25147503 (registering DOI) - 9 Jul 2024
Abstract
Sarcopenia refers to the progressive loss and atrophy of skeletal muscle function, often associated with aging or secondary to conditions involving systemic inflammation, oxidative stress, and mitochondrial dysfunction. Recent evidence indicates that skeletal muscle function is not only influenced by physical, environmental, and [...] Read more.
Sarcopenia refers to the progressive loss and atrophy of skeletal muscle function, often associated with aging or secondary to conditions involving systemic inflammation, oxidative stress, and mitochondrial dysfunction. Recent evidence indicates that skeletal muscle function is not only influenced by physical, environmental, and genetic factors but is also significantly impacted by nutritional deficiencies. Natural compounds with antioxidant properties, such as resveratrol and vitamin D, have shown promise in preventing mitochondrial dysfunction in skeletal muscle cells. These antioxidants can slow down muscle atrophy by regulating mitochondrial functions and neuromuscular junctions. This review provides an overview of the molecular mechanisms leading to skeletal muscle atrophy and summarizes recent advances in using resveratrol and vitamin D supplementation for its prevention and treatment. Understanding these molecular mechanisms and implementing combined interventions can optimize treatment outcomes, ensure muscle function recovery, and improve the quality of life for patients. Full article
(This article belongs to the Special Issue Mitochondrial Dysfunction in Aging and Diseases Affecting the Neuromuscular System)
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