Special Issue "Mitochondrial Biology in Health and Disease"

A special issue of Cells (ISSN 2073-4409). This special issue belongs to the section "Cell Signaling and Regulated Cell Death".

Deadline for manuscript submissions: 31 December 2018

Special Issue Editors

Guest Editor
Prof. Paul Fisher

Discipline of Microbiology, Department of Physiology Anatomy and Microbiology, School of Life Sciences, College of Science Health and Engineering, La Trobe University, Melbourne, Autralia
Website | E-Mail
Interests: mitochondrial biology and disease; neurodegenerative disease; AMPK; TOR complex I; cellular stress signalling
Assistant Guest Editor
Dr. Sarah Annesley

Discipline of Microbiology, Department of Physiology Anatomy and Microbiology, La Trobe University, Melbourne, Australia
Website | E-Mail
Interests: mitochondria; neurodegenerative disease; Dictyostelium discoideum

Special Issue Information

Dear Colleagues,

Best known as the site of respiratory ATP production, the mitochondria have been revealed over the past quarter-century as being deeply embedded in all aspects of the life of the eukaryotic cell. They are the product of two genomes whose expression must be coordinated by information shared in both directions between them and the nucleus. They participate in critical central metabolic pathways and they are fully integrated into the intracellular signalling networks that regulate diverse cellular functions. It is not surprising then that mitochondrial defects or dysregulation have emerged as having key roles in ageing and in the cytopathological mechanisms underlying cancer, neurodegenerative and other diseases.

This Special Issue of Cells is devoted to all aspects of mitochondrial function and dysfunction in healthy and diseased cells. It will contain articles that collectively provide a balanced, state-of-the-art view of mitochondrial biology. We seek submissions of high quality articles on including, but not limited to, mitochondrial biogenesis, metabolism, regulation, gene expression, signalling and roles in ageing and disease. Because of the vital and powerful contributions made by model organisms to understanding human biology and disease, we encourage manuscripts that describe or include the use of simple model systems to explore mitochondrial biology in health and disease.

Prof. Paul Robert Fisher
Dr. Sarah Annesley
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 papers will be 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. Cells 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 1000 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
  • mitochondrial biogenesis
  • mitochondrial metabolism
  • mitochondrial replication
  • mitochondrial gene expression
  • mitochondrial cell death
  • mitochondrial signalling
  • mitochondrial membrane transporters
  • mitochondrial respiration
  • OXPHOS
  • neurodegenerative disease
  • cellular stress responses
  • mitophagy
  • autophagy
  • MAMs
  • apoptosis
  • ER-mitochondria interactions
  • Parkinson’s disease
  • Alzheimer’s disease
  • ALS
  • Huntington’s disease
  • cancer

Published Papers (5 papers)

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Research

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Open AccessArticle Exploring the Effect of Rotenone—A Known Inducer of Parkinson’s Disease—On Mitochondrial Dynamics in Dictyostelium discoideum
Cells 2018, 7(11), 201; https://doi.org/10.3390/cells7110201
Received: 27 September 2018 / Revised: 31 October 2018 / Accepted: 6 November 2018 / Published: 8 November 2018
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Abstract
Current treatments for Parkinson’s disease (PD) only alleviate symptoms doing little to inhibit the onset and progression of the disease, thus we must research the mechanism of Parkinson’s. Rotenone is a known inducer of parkinsonian conditions in rats; we use rotenone to induce
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Current treatments for Parkinson’s disease (PD) only alleviate symptoms doing little to inhibit the onset and progression of the disease, thus we must research the mechanism of Parkinson’s. Rotenone is a known inducer of parkinsonian conditions in rats; we use rotenone to induce parkinsonian cellular conditions in Dictyostelium discoideum. In our model we primarily focus on mitochondrial dynamics. We found that rotenone disrupts the actin and microtubule cytoskeleton but mitochondrial morphology remains intact. Rotenone stimulates mitochondrial velocity while inhibiting mitochondrial fusion, increases reactive oxygen species (ROS) but has no effect on ATP levels. Antioxidants have been shown to decrease some PD symptoms thus we added ascorbic acid to our rotenone treated cells. Ascorbic acid administration suggests that rotenone effects may be specific to the disruption of the cytoskeleton rather than the increase in ROS. Our results imply that D. discoideum may be a valid cellular PD model and that the rotenone induced velocity increase and loss of fusion could prevent mitochondria from effectively providing energy and other mitochondrial products in high demand areas. The combination of these defects in mitochondrial dynamics and increased ROS could result in degeneration of neurons in PD. Full article
(This article belongs to the Special Issue Mitochondrial Biology in Health and Disease)
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Review

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Open AccessReview AAA Proteases: Guardians of Mitochondrial Function and Homeostasis
Cells 2018, 7(10), 163; https://doi.org/10.3390/cells7100163
Received: 18 September 2018 / Revised: 4 October 2018 / Accepted: 9 October 2018 / Published: 11 October 2018
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Abstract
Mitochondria are dynamic, semi-autonomous organelles that execute numerous life-sustaining tasks in eukaryotic cells. Functioning of mitochondria depends on the adequate action of versatile proteinaceous machineries. Fine-tuning of mitochondrial activity in response to cellular needs involves continuous remodeling of organellar proteome. This process not
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Mitochondria are dynamic, semi-autonomous organelles that execute numerous life-sustaining tasks in eukaryotic cells. Functioning of mitochondria depends on the adequate action of versatile proteinaceous machineries. Fine-tuning of mitochondrial activity in response to cellular needs involves continuous remodeling of organellar proteome. This process not only includes modulation of various biogenetic pathways, but also the removal of superfluous proteins by adenosine triphosphate (ATP)-driven proteolytic machineries. Accordingly, all mitochondrial sub-compartments are under persistent surveillance of ATP-dependent proteases. Particularly important are highly conserved two inner mitochondrial membrane-bound metalloproteases known as m-AAA and i-AAA (ATPases associated with diverse cellular activities), whose mis-functioning may lead to impaired organellar function and consequently to development of severe diseases. Herein, we discuss the current knowledge of yeast, mammalian, and plant AAA proteases and their implications in mitochondrial function and homeostasis maintenance. Full article
(This article belongs to the Special Issue Mitochondrial Biology in Health and Disease)
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Open AccessReview The Interplay among PINK1/PARKIN/Dj-1 Network during Mitochondrial Quality Control in Cancer Biology: Protein Interaction Analysis
Cells 2018, 7(10), 154; https://doi.org/10.3390/cells7100154
Received: 23 July 2018 / Revised: 14 September 2018 / Accepted: 25 September 2018 / Published: 29 September 2018
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Abstract
PARKIN (E3 ubiquitin ligase PARK2), PINK1 (PTEN induced kinase 1) and DJ-1 (PARK7) are proteins involved in autosomal recessive parkinsonism, and carcinogenic processes. In damaged mitochondria, PINK1’s importing into the inner mitochondrial membrane is prevented, PARKIN presents a partial mitochondrial
[...] Read more.
PARKIN (E3 ubiquitin ligase PARK2), PINK1 (PTEN induced kinase 1) and DJ-1 (PARK7) are proteins involved in autosomal recessive parkinsonism, and carcinogenic processes. In damaged mitochondria, PINK1’s importing into the inner mitochondrial membrane is prevented, PARKIN presents a partial mitochondrial localization at the outer mitochondrial membrane and DJ-1 relocates to mitochondria when oxidative stress increases. Depletion of these proteins result in abnormal mitochondrial morphology. PINK1, PARKIN, and DJ-1 participate in mitochondrial remodeling and actively regulate mitochondrial quality control. In this review, we highlight that PARKIN, PINK1, and DJ-1 should be regarded as having an important role in Cancer Biology. The STRING database and Gene Ontology (GO) enrichment analysis were performed to consolidate knowledge of well-known protein interactions for PINK1, PARKIN, and DJ-1 and envisage new ones. The enrichment analysis of KEGG pathways showed that the PINK1/PARKIN/DJ-1 network resulted in Parkinson disease as the main feature, while the protein DJ-1 showed enrichment in prostate cancer and p53 signaling pathway. Some predicted transcription factors regulating PINK1, PARK2 (PARKIN) and PARK7 (DJ-1) gene expression are related to cell cycle control. We can therefore suggest that the interplay among PINK1/PARKIN/DJ-1 network during mitochondrial quality control in cancer biology may occur at the transcriptional level. Further analysis, like a systems biology approach, will be helpful in the understanding of PINK1/PARKIN/DJ-1 network. Full article
(This article belongs to the Special Issue Mitochondrial Biology in Health and Disease)
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Open AccessReview Mitochondrial Quality Control in COPD and IPF
Received: 31 May 2018 / Revised: 6 July 2018 / Accepted: 24 July 2018 / Published: 24 July 2018
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Abstract
Mitochondria play important roles in the maintenance of intracellular homeostasis; hence, the quality control of mitochondria is crucial for cell fate determination. Mitochondria dynamics and mitochondria-specific autophagy, known as mitophagy, are two main quality control systems in cells. Mitochondria fuse to increase energy
[...] Read more.
Mitochondria play important roles in the maintenance of intracellular homeostasis; hence, the quality control of mitochondria is crucial for cell fate determination. Mitochondria dynamics and mitochondria-specific autophagy, known as mitophagy, are two main quality control systems in cells. Mitochondria fuse to increase energy production in response to stress, and damaged mitochondria are segregated by fission and degraded by mitophagy. Once these systems are disrupted, dysfunctional mitochondria with decreased adenosine triphosphate (ATP) production and increased reactive oxygen species (ROS) production accumulate, affecting cell fate. Recently, increasing evidence suggests that the dysregulation of mitochondria quality control is pathogenic in several age-related diseases. In this review, we outlined the role of mitochondria quality control systems in the pathogenesis of age-associated lung diseases, chronic obstructive pulmonary disease (COPD) and idiopathic pulmonary fibrosis (IPF). Full article
(This article belongs to the Special Issue Mitochondrial Biology in Health and Disease)
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Graphical abstract

Open AccessFeature PaperReview Mitochondrial Fatty Acid Oxidation Disorders Associated with Short-Chain Enoyl-CoA Hydratase (ECHS1) Deficiency
Received: 24 April 2018 / Revised: 15 May 2018 / Accepted: 16 May 2018 / Published: 23 May 2018
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Abstract
Mitochondrial fatty acid β-oxidation (FAO) is the primary pathway for fatty acid metabolism in humans, performing a key role in liver, heart and skeletal muscle energy homeostasis. FAO is particularly important during times of fasting when glucose supply is limited, providing energy for
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Mitochondrial fatty acid β-oxidation (FAO) is the primary pathway for fatty acid metabolism in humans, performing a key role in liver, heart and skeletal muscle energy homeostasis. FAO is particularly important during times of fasting when glucose supply is limited, providing energy for many organs and tissues, including the heart, liver and brain. Deficiencies in FAO can cause life-threatening metabolic disorders in early childhood that present with liver dysfunction, hypoglycemia, dilated hypertrophic cardiomyopathy and Reye-like Syndrome. Alternatively, FAO defects can also cause ‘milder’ adult-onset disease with exercise-induced myopathy and rhabdomyolysis. Short-chain enoyl-CoA hydratase (ECHS1) is a key FAO enzyme involved in the metabolism of fatty acyl-CoA esters. ECHS1 deficiency (ECHS1D) also causes human disease; however, the clinical manifestation is unlike most other FAO disorders. ECHS1D patients commonly present with Leigh syndrome, a lethal form of subacute necrotizing encephalomyelopathy traditionally associated with defects in oxidative phosphorylation (OXPHOS). In this article, we review the clinical, biochemical and genetic features of the ESHS1D patients described to date, and discuss the significance of the secondary OXPHOS defects associated with ECHS1D and their contribution to overall disease pathogenesis. Full article
(This article belongs to the Special Issue Mitochondrial Biology in Health and Disease)
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