Search for Articles:
Title / Keyword
Author / Affiliation / Email
Journal
Article Type
 
 
Advanced Search
 
Section
Special Issue
Volume
Issue
Number
Page
 
4.3
3.2
Journals
Life
Special Issues
Advances in Mitochondrial Biology
share announcement

Advances in Mitochondrial Biology

A special issue of Life (ISSN 2075-1729). This special issue belongs to the section "Physiology and Pathology".

Deadline for manuscript submissions: closed (16 August 2023) | Viewed by 14865

Special Issue Editors

Dr. Francesco Bruni

E-Mail Website
Guest Editor
Department of Biosciences, Biotechnologies and Environment, University of Bari ‘Aldo Moro’, 70121 Bari, Italy
Interests: mitochondria; mitochondrial biogenesis; mtDNA gene expression; mitoribosome
Special Issues, Collections and Topics in MDPI journals
Dr. Paola Loguercio Polosa

E-Mail Website
Guest Editor
Department of Biosciences, Biotechnologies and Environment, University of Bari ‘Aldo Moro’, 70121 Bari, Italy
Interests: mitochondrial biogenesis; mtDNA replication; mtDNA transcription; OXPHOS
Special Issues, Collections and Topics in MDPI journals

Special Issue Information

Dear Colleagues,

Mitochondria play a fundamental role in regulating the life processes of eukaryotic cells. Although these organelles have always been defined as the 'powerhouse of the cell’, many mitochondrial functions have been discovered to not strictly be related to the bioenergetic metabolism. Mitochondria host several anabolic pathways, including the synthesis of amino acids, lipids, ubiquinone, heme, steroid hormones and Fe–S clusters. Furthermore, they are a part of a vast network of cellular processes, which include the exchange of metabolites, nucleotides, membrane lipids and ions with other subcellular compartments, as well as the import of macromolecules (e.g., proteins and RNA). In addition to the aforementioned functions, novel cellular activities dependent on mitochondria are constantly being reported. Intriguingly, as descendants of a bacterial endosymbiont, mitochondria have retained their own genome (mtDNA) and exhibit a complex gene expression system, dependent on proteins encoded by nuclear genes.

Given these premises, it is not surprising that the alteration of mitochondrial functions, as well as the presence of mutations in nuclear and mitochondrial genes involved in mitochondrial gene expression and its regulation, cause a wide range of dysfunctions and syndromes universally known as mitochondrial diseases. Finally, common metabolic and age-related diseases (obesity, diabetes, Parkinson’s, etc.) and ageing itself have been associated with mitochondrial dysfunction.

This Special Issue aims to collect and report recent advances in the field of mitochondrial biology. Articles and reviews focusing both on biochemical aspects and the molecular mechanisms underlying mitochondrial function in health and disease are welcome.

Dr. Francesco Bruni
Dr. Paola Loguercio Polosa
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. Life is an international peer-reviewed open access monthly journal published by MDPI.

Please visit the Instructions for Authors page before submitting a manuscript. The Article Processing Charge (APC) for publication in this open access journal is 2600 CHF (Swiss Francs). 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

  • mitochondria
  • bioenergetics
  • mitochondrial gene expression
  • physiology
  • pathology
  • mitochondriopathies

Benefits of Publishing in a Special Issue

  • Ease of navigation: Grouping papers by topic helps scholars navigate broad scope journals more efficiently.
  • Greater discoverability: Special Issues support the reach and impact of scientific research. Articles in Special Issues are more discoverable and cited more frequently.
  • Expansion of research network: Special Issues facilitate connections among authors, fostering scientific collaborations.
  • External promotion: Articles in Special Issues are often promoted through the journal's social media, increasing their visibility.
  • e-Book format: Special Issues with more than 10 articles can be published as dedicated e-books, ensuring wide and rapid dissemination.

Further information on MDPI's Special Issue polices can be found here.

Published Papers (6 papers)

Download All Papers
Order results
Result details
Show export options expand_more Show export options expand_less
Select all
Export citation of selected articles as:

Research

Jump to: Review

16 pages, 2689 KiB  
Article
Molecular Investigation of Mitochondrial RNA19 Role in the Pathogenesis of MELAS Disease
by Paola Loguercio Polosa, Francesco Capriglia and Francesco Bruni
Life 2023, 13(9), 1863; https://doi.org/10.3390/life13091863 - 3 Sep 2023
Cited by 2 | Viewed by 1498
Abstract
In mammalian mitochondria, the processing of primary RNA transcripts involves a coordinated series of cleavage and modification events, leading to the formation of processing intermediates and mature mt-RNAs. RNA19 is an unusually stable unprocessed precursor, physiologically polyadenylated, which includes the 16S mt-rRNA, the [...] Read more.
In mammalian mitochondria, the processing of primary RNA transcripts involves a coordinated series of cleavage and modification events, leading to the formation of processing intermediates and mature mt-RNAs. RNA19 is an unusually stable unprocessed precursor, physiologically polyadenylated, which includes the 16S mt-rRNA, the mt-tRNALeuUUR and the mt-ND1 mRNA. These peculiarities, together with the alteration of its steady-state levels in cellular models with defects in mitochondrial function, make RNA19 a potentially important molecule for the physiological regulation of mitochondrial molecular processes as well as for the pathogenesis of mitochondrial diseases. In this work, we quantitatively and qualitatively examined RNA19 in MELAS trans-mitochondrial cybrids carrying the mtDNA 3243A>G transition and displaying a profound mitochondrial translation defect. Through a combination of isokinetic sucrose gradient and RT-qPCR experiments, we found that RNA19 accumulated and co-sedimented with the mitoribosomal large subunit (mt-LSU) in mutant cells. Intriguingly, exogenous expression of the isolated LARS2 C-terminal domain (Cterm), which was shown to rescue defective translation in MELAS cybrids, decreased the levels of mt-LSU-associated RNA19 by relegating it to the pool of free unbound RNAs. Overall, the data reported here support a regulatory role for RNA19 in mitochondrial physiopathological processes, designating this RNA precursor as a possible molecular target in view of therapeutic strategy development. Full article
(This article belongs to the Special Issue Advances in Mitochondrial Biology)
►▼ Show Figures

Figure 1

19 pages, 3456 KiB  
Article
Analysis of Human Clinical Mutations of Mitochondrial ND1 in a Bacterial Model System for Complex I
by Hind A. Alkhaldi, Duong H. Phan and Steven B. Vik
Life 2022, 12(11), 1934; https://doi.org/10.3390/life12111934 - 20 Nov 2022
Cited by 2 | Viewed by 2262
Abstract
The most common causes of mitochondrial dysfunction and disease include mutations in subunits and assembly factors of Complex I. Numerous mutations in the mitochondrial gene ND1 have been identified in humans. Currently, a bacterial model system provides the only method for rapid construction [...] Read more.
The most common causes of mitochondrial dysfunction and disease include mutations in subunits and assembly factors of Complex I. Numerous mutations in the mitochondrial gene ND1 have been identified in humans. Currently, a bacterial model system provides the only method for rapid construction and analysis of mutations in homologs of human ND1. In this report, we have identified nine mutations in human ND1 that are reported to be pathogenic and are located at subunit interfaces. Our hypothesis was that these mutations would disrupt Complex I assembly. Seventeen mutations were constructed in the homologous nuoH gene in an E. coli model system. In addition to the clinical mutations, alanine substitutions were constructed in order to distinguish between a deleterious effect from the introduction of the mutant residue and the loss of the original residue. The mutations were moved to an expression vector containing all thirteen genes of the E. coli nuo operon coding for Complex I. Membrane vesicles were prepared and rates of deamino-NADH oxidase activity and proton translocation were measured. Samples were also tested for assembly by native gel electrophoresis and for expression of NuoH by immunoblotting. A range of outcomes was observed: Mutations at four of the sites allow normal assembly with moderate activity (50–76% of wild type). Mutations at the other sites disrupt assembly and/or activity, and in some cases the outcomes depend upon the amino acid introduced. In general, the outcomes are consistent with the proposed pathogenicity in humans. Full article
(This article belongs to the Special Issue Advances in Mitochondrial Biology)
►▼ Show Figures

Figure 1

10 pages, 1392 KiB  
Article
Middle-Aged Lpaatδ-Deficient Mice Have Altered Metabolic Measures
by Michelle Victoria Tomczewski, Maria Fernanda Fernandes, Rajan Singh Grewal and Robin Elaine Duncan
Life 2022, 12(11), 1717; https://doi.org/10.3390/life12111717 - 27 Oct 2022
Viewed by 1665
Abstract
Lysophosphatidic acid acyltransferases/acylglycerophosphate acyltransferases (LPAATs/AGPATs) are a group of homologous enzymes that catalyze the formation of phosphatidic acid (PA) from lysophosphatidic acid. We have previously reported that LPAATδ/AGPAT4 localizes to mitochondria, suggesting a potential role in energy metabolism. However, in prior studies of [...] Read more.
Lysophosphatidic acid acyltransferases/acylglycerophosphate acyltransferases (LPAATs/AGPATs) are a group of homologous enzymes that catalyze the formation of phosphatidic acid (PA) from lysophosphatidic acid. We have previously reported that LPAATδ/AGPAT4 localizes to mitochondria, suggesting a potential role in energy metabolism. However, in prior studies of young Lpaatδ-deficient mice (age 9–12 weeks old), we found no differences in body weights, food intakes, activity levels, respiratory gas exchange, or energy expenditure compared to their wildtype (Wt) littermates. To test whether Lpaatδ−/− mice may develop differences in metabolic measures with advancing age, we recorded body weights and food intakes, and used metabolic chambers to assess ambulatory and locomotor activity levels, oxygen consumption (VO2), carbon dioxide production (VCO2), respiratory exchange ratio (RER), and total energy expenditure (heat). Fourteen-month-old Lpaatδ−/− mice had significantly lower mean body weights compared to Wt littermate controls (44.6 ± 1.08 g vs. 53.5 ± 0.42 g, respectively), but no significant differences in food intake or activity levels. This phenotypic difference was accompanied by significantly elevated 24 h daily, and 12 h light and dark photoperiod average VO2 (~20% higher) and VCO2 (~30% higher) measures, as well as higher RER and total energy expenditure (heat) values compared to Wt control littermates. Thus, an age-related metabolic phenotype is evident in Lpaatδ−/− mice. Future studies should examine the role of the lipid-modifying enzyme LPAATδ across the lifespan for greater insight into its role in normal and pathophysiology. Full article
(This article belongs to the Special Issue Advances in Mitochondrial Biology)
►▼ Show Figures

Figure 1

Review

Jump to: Research

15 pages, 1000 KiB  
Review
Mitochondrial Adaptations in Aging Skeletal Muscle: Implications for Resistance Exercise Training to Treat Sarcopenia
by Ilyoung Jeong, Eun-Jeong Cho, Jang-Soo Yook, Youngju Choi, Dong-Ho Park, Ju-Hee Kang, Seok-Hun Lee, Dae-Yun Seo, Su-Jeen Jung and Hyo-Bum Kwak
Life 2024, 14(8), 962; https://doi.org/10.3390/life14080962 - 31 Jul 2024
Cited by 1 | Viewed by 1649
Abstract
Sarcopenia, the age-related decline in muscle mass and function, poses a significant health challenge as the global population ages. Mitochondrial dysfunction is a key factor in sarcopenia, as evidenced by the role of mitochondrial reactive oxygen species (mtROS) in mitochondrial biogenesis and dynamics, [...] Read more.
Sarcopenia, the age-related decline in muscle mass and function, poses a significant health challenge as the global population ages. Mitochondrial dysfunction is a key factor in sarcopenia, as evidenced by the role of mitochondrial reactive oxygen species (mtROS) in mitochondrial biogenesis and dynamics, as well as mitophagy. Resistance exercise training (RET) is a well-established intervention for sarcopenia; however, its effects on the mitochondria in aging skeletal muscles remain unclear. This review aims to elucidate the relationship between mitochondrial dynamics and sarcopenia, with a specific focus on the implications of RET. Although aerobic exercise training (AET) has traditionally been viewed as more effective for mitochondrial enhancement, emerging evidence suggests that RET may also confer beneficial effects. Here, we highlight the potential of RET to modulate mtROS, drive mitochondrial biogenesis, optimize mitochondrial dynamics, and promote mitophagy in aging skeletal muscles. Understanding this interplay offers insights for combating sarcopenia and preserving skeletal muscle health in aging individuals. Full article
(This article belongs to the Special Issue Advances in Mitochondrial Biology)
►▼ Show Figures

Figure 1

22 pages, 9108 KiB  
Review
Mitochondrial Melatonin: Beneficial Effects in Protecting against Heart Failure
by Russel J. Reiter, Ramaswamy Sharma, Luiz Gustavo de Almeida Chuffa, Fedor Simko and Alberto Dominguez-Rodriguez
Life 2024, 14(1), 88; https://doi.org/10.3390/life14010088 - 5 Jan 2024
Cited by 4 | Viewed by 3348
Abstract
Cardiovascular disease is the cause of physical infirmity and thousands of deaths annually. Typically, during heart failure, cardiomyocyte mitochondria falter in terms of energy production and metabolic processing. Additionally, inflammation and the accumulation of non-contractile fibrous tissue contribute to cardiac malfunction. Melatonin, an [...] Read more.
Cardiovascular disease is the cause of physical infirmity and thousands of deaths annually. Typically, during heart failure, cardiomyocyte mitochondria falter in terms of energy production and metabolic processing. Additionally, inflammation and the accumulation of non-contractile fibrous tissue contribute to cardiac malfunction. Melatonin, an endogenously produced molecule, experimentally reduces the initiation and progression of atherosclerotic lesions, which are often the basis of coronary artery disease. The current review critically analyzes published data related to the experimental use of melatonin to forestall coronary artery pathologies. Collectively, these studies document melatonin’s anti-atherosclerotic actions in reducing LDL oxidation and triglyceride levels, lowering endothelial malfunction, limiting adhesion molecule formation, preventing macrophage polarization to the M1 pro-inflammatory phenotype, changing cellular metabolism, scavenging destructive reactive oxygen species, preventing the proliferation and invasion of arterial smooth muscle cells into the lesioned area, restricting the ingrowth of blood vessels from the vasa vasorum, and solidifying the plaque cap to reduce the chance of its rupture. Diabetic hyperglycemia, which aggravates atherosclerotic plaque formation, is also inhibited by melatonin supplementation in experimental animals. The potential value of non-toxic melatonin as a possible inhibitor of cardiac pathology in humans should be seriously considered by performing clinical trials using this multifunctional molecule. Full article
(This article belongs to the Special Issue Advances in Mitochondrial Biology)
►▼ Show Figures

Figure 1

56 pages, 3471 KiB  
Review
The Role of Genetic Mutations in Mitochondrial-Driven Cancer Growth in Selected Tumors: Breast and Gynecological Malignancies
by Ibolya Czegle, Chelsea Huang, Priscilla Geraldine Soria, Dylan Wesley Purkiss, Andrea Shields and Edina Amalia Wappler-Guzzetta
Life 2023, 13(4), 996; https://doi.org/10.3390/life13040996 - 12 Apr 2023
Cited by 2 | Viewed by 3430
Abstract
There is an increasing understanding of the molecular and cytogenetic background of various tumors that helps us better conceptualize the pathogenesis of specific diseases. Additionally, in many cases, these molecular and cytogenetic alterations have diagnostic, prognostic, and/or therapeutic applications that are heavily used [...] Read more.
There is an increasing understanding of the molecular and cytogenetic background of various tumors that helps us better conceptualize the pathogenesis of specific diseases. Additionally, in many cases, these molecular and cytogenetic alterations have diagnostic, prognostic, and/or therapeutic applications that are heavily used in clinical practice. Given that there is always room for improvement in cancer treatments and in cancer patient management, it is important to discover new therapeutic targets for affected individuals. In this review, we discuss mitochondrial changes in breast and gynecological (endometrial and ovarian) cancers. In addition, we review how the frequently altered genes in these diseases (BRCA1/2, HER2, PTEN, PIK3CA, CTNNB1, RAS, CTNNB1, FGFR, TP53, ARID1A, and TERT) affect the mitochondria, highlighting the possible associated individual therapeutic targets. With this approach, drugs targeting mitochondrial glucose or fatty acid metabolism, reactive oxygen species production, mitochondrial biogenesis, mtDNA transcription, mitophagy, or cell death pathways could provide further tailored treatment. Full article
(This article belongs to the Special Issue Advances in Mitochondrial Biology)
►▼ Show Figures

Figure 1

Show export options expand_more Show export options expand_less
Select all
Export citation of selected articles as:
 
Displaying articles 1-6
Back to TopTop