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Special Issue "Mitochondrial Bioenergetics in Different Pathophysiological Conditions 3.0"

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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 (28 February 2023) | Viewed by 6797

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Special Issue Editors

Dr. Anna Atlante

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Guest Editor

Institute of Biomembranes, Bioenergetics and Molecular Biotechnologies, National Council of Research, Bari, Italy
Interests: mitochondrial bioenergetics; oxidative phosphorylation; mitochondrial dysfunction; metabolic regulation; oxidative stress; apoptosis; Alzheimer’s disease; cystic fibrosis
Special Issues, Collections and Topics in MDPI journals

Dr. Daniela Valenti

E-Mail Website1 Website2
Guest Editor

Institute of Biomembranes, Bioenergetics and Molecular Biotechnologies, National Council of Research, Bari, Italy
Interests: mitochondrial bioenergetics; mitochondrial metabolism; mitochondrial transport; mitochondrial signaling pathways; mitochondrial dysfunction; neurodevelopmental diseases; oxidative stress; reactive oxygen species; programmed cell death
Special Issues, Collections and Topics in MDPI journals

Special Issue Information

Dear Colleagues,

Mitochondria are central actors in the bioenergetics of cellular life. They are maternally inherited, multifunctional organelles widely known for generating energy in the form of ATP through the inner membrane mitochondrial respiratory chain complexes that form the functional respirasome. Besides the oxidative phosphorylation process, mitochondrial transport, inter-organelle crosstalk, mitochondrial dynamics, biogenesis and degradation all play a critical role in the efficiency and homeostasis of mitochondrial bioenergetics. Damage to these highly energetic and redox-sensitive organelles can result in an increase in the autophagic removal of the mitochondria (mitophagy) and disruption to the mitochondrial network. Mitochondrial dysfunction is now emerging as a major contributor to the pathogenesis of a broad range of human diseases, directly or indirectly, through a wide spectrum of signaling pathways.

Contributions to this Special Issue will provide new insights into mitochondrial bioenergetics to deepen our understanding of its role in health and disease and reveal novel mitochondria-targeting therapeutic opportunities. Original research articles and topical reviews on these and related topics are welcome in this Special Issue.

Due to the success of the first and second editions, we would like to add more results and new insights from recent research projects.

https://www.mdpi.com/journal/ijms/special_issues/Mitochondrial_Bioenergetics

https://www.mdpi.com/journal/ijms/special_issues/Mitochondrial_Bioenergetics_2

Dr. Anna Atlante
Dr. Daniela Valenti
Guest Editors

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 ATP generation
oxidative phosphorylation machinery
mitochondrial quality control
mitochondrial dynamic network
mitogenesis/mitophagy
mitochondrial signaling
inter-organelle crosstalk
mitochondrial dysfunction
mitochondria-targeting therapeutic strategies

Related Special Issues

Mitochondrial Bioenergetics in Different Pathophysiological Conditions in International Journal of Molecular Sciences (10 articles)
Mitochondrial Bioenergetics in Different Pathophysiological Conditions 2.0 in International Journal of Molecular Sciences (16 articles)

Published Papers (7 papers)

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Research

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Open AccessArticle

Pathway and Network Analyses Identify Growth Factor Signaling and MMP9 as Potential Mediators of Mitochondrial Dysfunction in Severe COVID-19

, PREDICT-19 Consortium,

Benjamin Tang

Anthony McLean

and

Maryam Shojaei

Int. J. Mol. Sci. 2023, 24(3), 2524; https://doi.org/10.3390/ijms24032524 - 28 Jan 2023

Viewed by 645

Abstract

Patients with preexisting metabolic disorders such as diabetes are at a higher risk of developing severe coronavirus disease 2019 (COVID-19). Mitochondrion, the very organelle that controls cellular metabolism, holds the key to understanding disease progression at the cellular level. Our current study aimed to understand how cellular metabolism contributes to COVID-19 outcomes. Metacore pathway enrichment analyses on differentially expressed genes (encoded by both mitochondrial and nuclear deoxyribonucleic acid (DNA)) involved in cellular metabolism, regulation of mitochondrial respiration and organization, and apoptosis, was performed on RNA sequencing (RNASeq) data from blood samples collected from healthy controls and patients with mild/moderate or severe COVID-19. Genes from the enriched pathways were analyzed by network analysis to uncover interactions among them and up- or downstream genes within each pathway. Compared to the mild/moderate COVID-19, the upregulation of a myriad of growth factor and cell cycle signaling pathways, with concomitant downregulation of interferon signaling pathways, were observed in the severe group. Matrix metallopeptidase 9 (MMP9) was found in five of the top 10 upregulated pathways, indicating its potential as therapeutic target against COVID-19. In summary, our data demonstrates aberrant activation of endocrine signaling in severe COVID-19, and its implication in immune and metabolic dysfunction. Full article

(This article belongs to the Special Issue Mitochondrial Bioenergetics in Different Pathophysiological Conditions 3.0)

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Open AccessArticle

Effect of Quercetin on mitoBK_Ca Channel and Mitochondrial Function in Human Bronchial Epithelial Cells Exposed to Particulate Matter

and

Int. J. Mol. Sci. 2023, 24(1), 638; https://doi.org/10.3390/ijms24010638 - 30 Dec 2022

Viewed by 655

Abstract

Particulate matter (PM) exposure increases reactive oxygen species (ROS) levels. It can lead to inflammatory responses and damage of the mitochondria thus inducing cell death. Recently, it has been shown that potassium channels (mitoK) located in the inner mitochondrial membrane are involved in cytoprotection, and one of the mechanisms involves ROS. To verify the cytoprotective role of mitoBK_Ca, we performed a series of experiments using a patch-clamp, transepithelial electrical resistance assessment (TEER), mitochondrial respiration measurements, fluorescence methods for the ROS level and mitochondrial membrane potential assessment, and cell viability measurements. In the human bronchial epithelial cell model (16HBE14σ), PM < 4 μm in diameter (SRM-PM4.0) was used. We observed that PM decreased TEER of HBE cell monolayers. The effect was partially abolished by quercetin, a mitoBK_Ca opener. Consequently, quercetin decreased the mitochondrial membrane potential and increased mitochondrial respiration. The reduction of PM-induced ROS level occurs both on cellular and mitochondrial level. Additionally, quercetin restores HBE cell viability after PM administration. The incubation of cells with PM substantially reduced the mitochondrial function. Isorhamnetin had no effect on TEER, the mitoBK_Ca activity, respiratory rate, or mitochondrial membrane potential. Obtained results indicate that PM has an adverse effect on HBE cells at the cellular and mitochondrial level. Quercetin is able to limit the deleterious effect of PM on barrier function of airway epithelial cells. We show that the effect in HBE cells involves mitoBK_Ca channel-activation. However, quercetin’s mechanism of action is not exclusively determined by modulation of the channel activity. Full article

(This article belongs to the Special Issue Mitochondrial Bioenergetics in Different Pathophysiological Conditions 3.0)

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Open AccessArticle

ATP8B1 Deficiency Results in Elevated Mitochondrial Phosphatidylethanolamine Levels and Increased Mitochondrial Oxidative Phosphorylation in Human Hepatoma Cells

Valentina E. Gómez-Mellado

Jung-Chin Chang

Kam S. Ho-Mok

Carmen Bernardino Morcillo

Remco H. J. Kersten

Ronald P. J. Oude Elferink

Arthur J. Verhoeven

and

Coen C. Paulusma

Int. J. Mol. Sci. 2022, 23(20), 12344; https://doi.org/10.3390/ijms232012344 - 15 Oct 2022

Viewed by 621

Abstract

ATP8B1 is a phospholipid flippase that is deficient in patients with progressive familial intrahepatic cholestasis type 1 (PFIC1). PFIC1 patients suffer from severe liver disease but also present with dyslipidemia, including low plasma cholesterol, of yet unknown etiology. Here we show that ATP8B1 knockdown in HepG2 cells leads to a strong increase in the mitochondrial oxidative phosphorylation (OXPHOS) without a change in glycolysis. The enhanced OXPHOS coincides with elevated low-density lipoprotein receptor protein and increased mitochondrial fragmentation and phosphatidylethanolamine levels. Furthermore, expression of phosphatidylethanolamine N-methyltransferase, an enzyme that catalyzes the conversion of mitochondrial-derived phosphatidylethanolamine to phosphatidylcholine, was reduced in ATP8B1 knockdown cells. We conclude that ATP8B1 deficiency results in elevated mitochondrial PE levels that stimulate mitochondrial OXPHOS. The increased OXPHOS leads to elevated LDLR levels, which provides a possible explanation for the reduced plasma cholesterol levels in PFIC1 disease. Full article

(This article belongs to the Special Issue Mitochondrial Bioenergetics in Different Pathophysiological Conditions 3.0)

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Open AccessArticle

Sex as Biological Variable in Cardiac Mitochondrial Bioenergetic Responses to Acute Stress

and

Int. J. Mol. Sci. 2022, 23(16), 9312; https://doi.org/10.3390/ijms23169312 - 18 Aug 2022

Cited by 2 | Viewed by 946

Abstract

Cardiac dysfunction/damage following trauma, shock, sepsis, and ischemia impacts clinical outcomes. Acute inflammation and oxidative stress triggered by these injuries impair mitochondria, which are critical to maintaining cardiac function. Despite sex dimorphisms in consequences of these injuries, it is unclear whether mitochondrial bioenergetic responses to inflammation/oxidative stress are sex-dependent. We hypothesized that sex disparity in mitochondrial bioenergetics following TNFα or H₂O₂ exposure is responsible for reported sex differences in cardiac damage/dysfunction. Methods and Results: Cardiomyocytes isolated from age-matched adult male and female mice were subjected to 1 h TNFα or H₂O₂ challenge, followed by detection of mitochondrial respiration capacity using the Seahorse XF96 Cell Mito Stress Test. Mitochondrial membrane potential (ΔΨm) was analyzed using JC-1 in TNFα-challenged cardiomyocytes. We found that cardiomyocytes isolated from female mice displayed a better mitochondrial bioenergetic response to TNFα or H₂O₂ than those isolated from male mice did. TNFα decreased ΔΨm in cardiomyocytes isolated from males but not from females. 17β-estradiol (E2) treatment improved mitochondrial metabolic function in cardiomyocytes from male mice subjected to TNFα or H₂O₂ treatment. Conclusions: Cardiomyocyte mitochondria from female mice were more resistant to acute stress than those from males. The female sex hormone E2 treatment protected cardiac mitochondria against acute inflammatory and oxidative stress. Full article

(This article belongs to the Special Issue Mitochondrial Bioenergetics in Different Pathophysiological Conditions 3.0)

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Review

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Open AccessReview

Mitochondrial Transplantation in Mitochondrial Medicine: Current Challenges and Future Perspectives

and

Int. J. Mol. Sci. 2023, 24(3), 1969; https://doi.org/10.3390/ijms24031969 - 19 Jan 2023

Viewed by 882

Abstract

Mitochondrial diseases (MDs) are inherited genetic conditions characterized by pathogenic mutations in nuclear DNA (nDNA) or mitochondrial DNA (mtDNA). Current therapies are still far from being fully effective and from covering the broad spectrum of mutations in mtDNA. For example, unlike heteroplasmic conditions, MDs caused by homoplasmic mtDNA mutations do not yet benefit from advances in molecular approaches. An attractive method of providing dysfunctional cells and/or tissues with healthy mitochondria is mitochondrial transplantation. In this review, we discuss what is known about intercellular transfer of mitochondria and the methods used to transfer mitochondria both in vitro and in vivo, and we provide an outlook on future therapeutic applications. Overall, the transfer of healthy mitochondria containing wild-type mtDNA copies could induce a heteroplasmic shift even when homoplasmic mtDNA variants are present, with the aim of attenuating or preventing the progression of pathological clinical phenotypes. In summary, mitochondrial transplantation is a challenging but potentially ground-breaking option for the treatment of various mitochondrial pathologies, although several questions remain to be addressed before its application in mitochondrial medicine. Full article

(This article belongs to the Special Issue Mitochondrial Bioenergetics in Different Pathophysiological Conditions 3.0)

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Open AccessReview

Mitochondria and Cancer Recurrence after Liver Transplantation—What Is the Benefit of Machine Perfusion?

Alessandro Parente

Mauricio Flores Carvalho

Janina Eden

Philipp Dutkowski

and

Andrea Schlegel

Int. J. Mol. Sci. 2022, 23(17), 9747; https://doi.org/10.3390/ijms23179747 - 28 Aug 2022

Viewed by 1180

Abstract

Tumor recurrence after liver transplantation has been linked to multiple factors, including the recipient’s tumor burden, donor factors, and ischemia-reperfusion injury (IRI). The increasing number of livers accepted from extended criteria donors has forced the transplant community to push the development of dynamic perfusion strategies. The reason behind this progress is the urgent need to reduce the clinical consequences of IRI. Two concepts appear most beneficial and include either the avoidance of ischemia, e.g., the replacement of cold storage by machine perfusion, or secondly, an endischemic organ improvement through perfusion in the recipient center prior to implantation. While several concepts, including normothermic perfusion, were found to reduce recipient transaminase levels and early allograft dysfunction, hypothermic oxygenated perfusion also reduced IRI-associated post-transplant complications and costs. With the impact on mitochondrial injury and subsequent less IRI-inflammation, this endischemic perfusion was also found to reduce the recurrence of hepatocellular carcinoma after liver transplantation. Firstly, this article highlights the contributing factors to tumor recurrence, including the surgical and medical tissue trauma and underlying mechanisms of IRI-associated inflammation. Secondly, it focuses on the role of mitochondria and associated interventions to reduce cancer recurrence. Finally, the role of machine perfusion technology as a delivery tool and as an individual treatment is discussed together with the currently available clinical studies. Full article

(This article belongs to the Special Issue Mitochondrial Bioenergetics in Different Pathophysiological Conditions 3.0)

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Open AccessReview

Impairment of Mitochondrial Respiration in Metabolic Diseases: An Overview

Mariana Georgeta Rosca

and

Danina Mirela Muntean

Int. J. Mol. Sci. 2022, 23(16), 8852; https://doi.org/10.3390/ijms23168852 - 09 Aug 2022

Cited by 1 | Viewed by 1197

Abstract

Mitochondrial dysfunction has emerged as a central pathomechanism in the setting of obesity and diabetes mellitus, linking these intertwined pathologies that share insulin resistance as a common denominator. High-resolution respirometry (HRR) is a state-of-the-art research method currently used to study mitochondrial respiration and its impairment in health and disease. Tissue samples, cells or isolated mitochondria are exposed to various substrate-uncoupler-inhibitor-titration protocols, which allows the measurement and calculation of several parameters of mitochondrial respiration. In this review, we discuss the alterations of mitochondrial bioenergetics in the main dysfunctional organs that contribute to the development of the obese and diabetic phenotypes in both animal models and human subjects. Herein we review data regarding the impairment of oxidative phosphorylation as integrated mitochondrial function assessed by means of HRR. We acknowledge the critical role of this method in determining the alterations in oxidative phosphorylation occurring in the early stages of metabolic pathologies. We conclude that there is a mutual two-way relationship between mitochondrial dysfunction and insulin insensitivity that characterizes these diseases. Full article

(This article belongs to the Special Issue Mitochondrial Bioenergetics in Different Pathophysiological Conditions 3.0)

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