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Understanding the Mechanisms of Mitochondrial Stress and Its Association with Human Diseases

A special issue of International Journal of Molecular Sciences (ISSN 1422-0067). This special issue belongs to the section "Molecular Pathology, Diagnostics, and Therapeutics".

Deadline for manuscript submissions: 20 February 2025 | Viewed by 5710

Special Issue Editor

Dr. Jaeseok Han

E-Mail Website
Guest Editor
Soonchunhyang Institute of Medi-Bio Science (SIMS), Soonchunhyang University, Cheonan 31151, Republic of Korea
Interests: mitochondrial stress; integrated stress response; metabolic diseases; neurodegenerative diseases
Special Issues, Collections and Topics in MDPI journals

Special Issue Information

Dear Colleagues,

Mitochondria are essential organelles responsible for the production of energy in the form of ATP in eukaryotic cells. In addition to energy production, mitochondria play a crucial role in various cellular processes, such as calcium signalling, heme biosynthesis, the synthesis and breakdown of lipids and amino acids, and the regulation of programmed cell death. Therefore, the maintenance of mitochondrial integrity is important for optimal cellular function and the prevention of the development of diseases associated with mitochondrial dysfunction. Mitochondrial stress is the impairment of the normal function of mitochondria caused by various factors such as oxidative stress, mitochondrial DNA mutations, and impaired mitochondrial proteostasis. Mitochondrial stress can lead to mitochondrial dysfunction, which has been implicated in various human diseases, including neurodegenerative disorders, metabolic diseases, cardiovascular diseases, and cancers. Therefore, cells develop defensive systems to recover the mitochondrial function upon mitochondrial stress, which can mitigate the effects of mitochondrial dysfunction. Some of the key mitochondrial stress responses are mitochondrial biogenesis, the regulation of mitochondrial dynamics, mitophagy, and the mitochondrial unfolded protein response (UPRMT). These responses aim to restore normal mitochondrial function, clear damaged mitochondria, and prevent further damage. Understanding the underlying mechanisms of the mitochondrial stress response is essential for the development of therapeutic approaches to treat diseases associated with mitochondrial stress and dysfunction.

This Special Issue will cover a selection of recent research topics and current review articles related to the underlying mechanisms in mitochondrial stress and its association with human diseases. The topics of interest include, but are not limited to:

1. Mechanisms of mitochondrial stress and its effects on cellular processes.

2. Mitochondrial stress and its association with human diseases such as neurodegenerative disorders, metabolic diseases, cardiovascular diseases, and cancer.

3. Diagnostic tools and biomarkers for assessing mitochondrial stress and dysfunction.

4. Therapeutic approaches to mitigate the effects of mitochondrial stress and prevent the development of associated human diseases.

5. Mitochondrial stress and its association with ageing and age-related diseases

Dr. Jaeseok Han
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.

Please visit the Instructions for Authors page before submitting a manuscript. There is an Article Processing Charge (APC) for publication in this open access journal. For details about the APC please see here. 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

  • mitochondrial stress
  • mitochondrial quality control
  • mitophagy
  • mitochondrial unfolded protein response
  • mitochondrial fission and fusion
  • mitochondria-related diseases

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Published Papers (4 papers)

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Research

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17 pages, 4017 KiB  
Article
Exploring Mitochondrial Interactions with Pulsed Electromagnetic Fields: An Insightful Inquiry into Strategies for Addressing Neuroinflammation and Oxidative Stress in Diabetic Neuropathy
by Diego Chianese, Massimo Bonora, Maria Sambataro, Luisa Sambato, Luca Dalla Paola, Elena Tremoli, Ilenia Pia Cappucci, Marco Scatto, Paolo Pinton, Massimo Picari, Letizia Ferroni and Barbara Zavan
Int. J. Mol. Sci. 2024, 25(14), 7783; https://doi.org/10.3390/ijms25147783 - 16 Jul 2024
Viewed by 1144
Abstract
Pulsed electromagnetic fields (PEMFs) are recognized for their potential in regenerative medicine, offering a non-invasive avenue for tissue rejuvenation. While prior research has mainly focused on their effects on bone and dermo-epidermal tissues, the impact of PEMFs on nervous tissue, particularly in the [...] Read more.
Pulsed electromagnetic fields (PEMFs) are recognized for their potential in regenerative medicine, offering a non-invasive avenue for tissue rejuvenation. While prior research has mainly focused on their effects on bone and dermo-epidermal tissues, the impact of PEMFs on nervous tissue, particularly in the context of neuropathy associated with the diabetic foot, remains relatively unexplored. Addressing this gap, our preliminary in vitro study investigates the effects of complex magnetic fields (CMFs) on glial-like cells derived from mesenchymal cell differentiation, serving as a model for neuropathy of the diabetic foot. Through assessments of cellular proliferation, hemocompatibility, mutagenicity, and mitochondrial membrane potential, we have established the safety profile of the system. Furthermore, the analysis of microRNAs (miRNAs) suggests that CMFs may exert beneficial effects on cell cycle regulation, as evidenced by the upregulation of the miRNAs within the 121, 127, and 142 families, which are known to be associated with mitochondrial function and cell cycle control. This exploration holds promise for potential applications in mitigating neuropathic complications in diabetic foot conditions. Full article
(This article belongs to the Special Issue Understanding the Mechanisms of Mitochondrial Stress and Its Association with Human Diseases)
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20 pages, 4235 KiB  
Article
PKR Mediates the Mitochondrial Unfolded Protein Response through Double-Stranded RNA Accumulation under Mitochondrial Stress
by Fedho Kusuma, Soyoung Park, Kim Anh Nguyen, Rosalie Elvira, Duckgue Lee and Jaeseok Han
Int. J. Mol. Sci. 2024, 25(14), 7738; https://doi.org/10.3390/ijms25147738 - 15 Jul 2024
Viewed by 1151
Abstract
Mitochondrial stress, resulting from dysfunction and proteostasis disturbances, triggers the mitochondrial unfolded protein response (UPRMT), which activates gene encoding chaperones and proteases to restore mitochondrial function. Although ATFS-1 mediates mitochondrial stress UPRMT induction in C. elegans, the mechanisms [...] Read more.
Mitochondrial stress, resulting from dysfunction and proteostasis disturbances, triggers the mitochondrial unfolded protein response (UPRMT), which activates gene encoding chaperones and proteases to restore mitochondrial function. Although ATFS-1 mediates mitochondrial stress UPRMT induction in C. elegans, the mechanisms relaying mitochondrial stress signals to the nucleus in mammals remain poorly defined. Here, we explored the role of protein kinase R (PKR), an eIF2α kinase activated by double-stranded RNAs (dsRNAs), in mitochondrial stress signaling. We found that UPRMT does not occur in cells lacking PKR, indicating its crucial role in this process. Mechanistically, we observed that dsRNAs accumulate within mitochondria under stress conditions, along with unprocessed mitochondrial transcripts. Furthermore, we demonstrated that accumulated mitochondrial dsRNAs in mouse embryonic fibroblasts (MEFs) deficient in the Bax/Bak channels are not released into the cytosol and do not induce the UPRMT upon mitochondrial stress, suggesting a potential role of the Bax/Bak channels in mediating the mitochondrial stress response. These discoveries enhance our understanding of how cells maintain mitochondrial integrity, respond to mitochondrial dysfunction, and communicate stress signals to the nucleus through retrograde signaling. This knowledge provides valuable insights into prospective therapeutic targets for diseases associated with mitochondrial stress. Full article
(This article belongs to the Special Issue Understanding the Mechanisms of Mitochondrial Stress and Its Association with Human Diseases)
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30 pages, 10649 KiB  
Article
An In Silico Analysis of Genetic Variants and Structural Modeling of the Human Frataxin Protein in Friedreich’s Ataxia
by Loiane Mendonça Abrantes Da Conceição, Lucio Mendes Cabral, Gabriel Rodrigues Coutinho Pereira and Joelma Freire De Mesquita
Int. J. Mol. Sci. 2024, 25(11), 5796; https://doi.org/10.3390/ijms25115796 - 26 May 2024
Viewed by 1472
Abstract
Friedreich’s Ataxia (FRDA) stands out as the most prevalent form of hereditary ataxias, marked by progressive movement ataxia, loss of vibratory sensitivity, and skeletal deformities, severely affecting daily functioning. To date, the only medication available for treating FRDA is Omaveloxolone (Skyclarys®), [...] Read more.
Friedreich’s Ataxia (FRDA) stands out as the most prevalent form of hereditary ataxias, marked by progressive movement ataxia, loss of vibratory sensitivity, and skeletal deformities, severely affecting daily functioning. To date, the only medication available for treating FRDA is Omaveloxolone (Skyclarys®), recently approved by the FDA. Missense mutations within the human frataxin (FXN) gene, responsible for intracellular iron homeostasis regulation, are linked to FRDA development. These mutations induce FXN dysfunction, fostering mitochondrial iron accumulation and heightened oxidative stress, ultimately triggering neuronal cell death pathways. This study amalgamated 226 FXN genetic variants from the literature and database searches, with only 18 previously characterized. Predictive analyses revealed a notable prevalence of detrimental and destabilizing predictions for FXN mutations, predominantly impacting conserved residues crucial for protein function. Additionally, an accurate, comprehensive three-dimensional model of human FXN was constructed, serving as the basis for generating genetic variants I154F and W155R. These variants, selected for their severe clinical implications, underwent molecular dynamics (MD) simulations, unveiling flexibility and essential dynamic alterations in their N-terminal segments, encompassing FXN42, FXN56, and FXN78 domains pivotal for protein maturation. Thus, our findings indicate potential interaction profile disturbances in the FXN42, FXN56, and FXN78 domains induced by I154F and W155R mutations, aligning with the existing literature. Full article
(This article belongs to the Special Issue Understanding the Mechanisms of Mitochondrial Stress and Its Association with Human Diseases)
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Review

Jump to: Research

21 pages, 324 KiB  
Review
The Role of Mitochondrial Copy Number in Neurodegenerative Diseases: Present Insights and Future Directions
by Annamaria Cerantonio, Luigi Citrigno, Beatrice Maria Greco, Selene De Benedittis, Giuseppe Passarino, Raffaele Maletta, Antonio Qualtieri, Alberto Montesanto, Patrizia Spadafora and Francesca Cavalcanti
Int. J. Mol. Sci. 2024, 25(11), 6062; https://doi.org/10.3390/ijms25116062 - 31 May 2024
Viewed by 1411
Abstract
Neurodegenerative diseases are progressive disorders that affect the central nervous system (CNS) and represent the major cause of premature death in the elderly. One of the possible determinants of neurodegeneration is the change in mitochondrial function and content. Altered levels of mitochondrial DNA [...] Read more.
Neurodegenerative diseases are progressive disorders that affect the central nervous system (CNS) and represent the major cause of premature death in the elderly. One of the possible determinants of neurodegeneration is the change in mitochondrial function and content. Altered levels of mitochondrial DNA copy number (mtDNA-CN) in biological fluids have been reported during both the early stages and progression of the diseases. In patients affected by neurodegenerative diseases, changes in mtDNA-CN levels appear to correlate with mitochondrial dysfunction, cognitive decline, disease progression, and ultimately therapeutic interventions. In this review, we report the main results published up to April 2024, regarding the evaluation of mtDNA-CN levels in blood samples from patients affected by Alzheimer’s (AD), Parkinson’s (PD), and Huntington’s diseases (HD), amyotrophic lateral sclerosis (ALS), and multiple sclerosis (MS). The aim is to show a probable link between mtDNA-CN changes and neurodegenerative disorders. Understanding the causes underlying this association could provide useful information on the molecular mechanisms involved in neurodegeneration and offer the development of new diagnostic approaches and therapeutic interventions. Full article
(This article belongs to the Special Issue Understanding the Mechanisms of Mitochondrial Stress and Its Association with Human Diseases)
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