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Medicina
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Mitochondrial Oxidative Phosphorylation Disorders
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Mitochondrial Oxidative Phosphorylation Disorders

A special issue of Medicina (ISSN 1648-9144).

Deadline for manuscript submissions: closed (31 August 2019) | Viewed by 5992

Special Issue Editor

Prof. Dr. Stavros J. Baloyannis

E-Mail Website
Guest Editor
Department of Neurology, Aristotelian University of Thessaloniki, 541 24 Thessaloniki, Greece
Interests: neurosciences; electron microscopy; Alzheimer’s disease; neuropathology; clinical neurology; neurophilosophy
Special Issues, Collections and Topics in MDPI journals

Special Issue Information

Dear Colleagues,

Mitochondrial oxidative phosphorylation disorders compose a wide spectrum of progressive clinical conditions, with a multisystem phenomenology at any age, affecting mostly organs requiring high energy supply, such as the brain, heart, muscles, retina, and kidneys. The diagnostic procedure usually includes the estimation of lactate, pyruvate, and alanine levels both in blood and cerebrospinal fluid (CSF). Imaging examination of the brain and heart and histological and biochemical analysis of skeletal muscles also offer substantial diagnostic documentations. From an etiopathological point of view, the majority of the affected patients have inhered a defective oxidative phosphorylation system (OXPHOS) of maternal origin, probably caused by a mutation in mitochondrial DNA (mtDNA) or nuclear DNA (nDNA), heteroplasmy, or mitotic segregation. The detailed investigation of oxidative phosphorylation (OX-PHOS) defects in mitochondrial disorders is based primarily on the muscle biopsy, which is an easily accessed resource for electron microscopy and biochemical studies. In addition, the ultrastructural, biochemical, and genetic analysis of lymphoblasts and transmitochondrial cell lines and cybrids would also be important, since the suspension enucleation of cells combined with electrofusion facilitates the use of any cell type for further research. The therapeutic approach of mitochondrial oxidative phosphorylation disorders remains a serious problem, since no specific treatment is available, in exception to coenzyme Q10 deficiency. Further research on mitochondrial disorders would be essential for a deeper understanding of the pathogenetic mechanisms of a wide range of neurodegenerative disorders, including Parkinson’s and Alzheimer’s diseases, which are presumably related to mitochondrial pathology.

Prof. Dr. Stavros J. Baloyannis
Guest Editor

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Keywords

  • Mitochondria
  • Oxidative stress
  • Neurodegerative disorders
  • Mitochondrial diseases
  • Mitochondrial myopathies
  • Histochemistry
  • Electron microscopy

Published Papers (2 papers)

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Research

11 pages, 1025 KiB  
Article
Nitric Oxide Donor NOC-18-Induced Changes of Mitochondrial Phosphoproteome in Rat Cardiac Ischemia Model
by Danielius Umbrasas, Ramūnas Jokubka, Algirdas Kaupinis, Mindaugas Valius, Odeta Arandarčikaitė and Vilmantė Borutaitė
Medicina 2019, 55(10), 631; https://doi.org/10.3390/medicina55100631 - 24 Sep 2019
Cited by 4 | Viewed by 2115
Abstract
Background and objective: Nitric oxide (NO) is known to exert cardioprotective effects against heart ischemic damage and may be involved in ischemic pre- and postconditioning. NO-triggered cardioprotective mechanisms are not well understood but may involve regulation of mitochondrial permeability transition pore (mPTP). [...] Read more.
Background and objective: Nitric oxide (NO) is known to exert cardioprotective effects against heart ischemic damage and may be involved in ischemic pre- and postconditioning. NO-triggered cardioprotective mechanisms are not well understood but may involve regulation of mitochondrial permeability transition pore (mPTP). In this study, we aimed to identify differentially phosphorylated mitochondrial proteins possibly involved in the NO/protein kinase G (PKG)/mPTP signaling pathway that can increase the resistance of cardiomyocytes to ischemic damage. Materials and methods: Isolated hearts from Wistar rats were perfused with NO donor NOC-18 prior to induction of stop–flow ischemia. To quantify and characterize the phosphoproteins, mitochondrial proteins were resolved and analyzed by two-dimensional gel electrophoresis followed by Pro-Q Diamond phosphoprotein gel staining, excision, trypsin digestions, and mass spectrometry. Quantitative proteomic analysis coupled with liquid chromatography–tandem mass spectrometry was also performed. Results: Mitochondrial protein phosphorylation patterns in NOC-18-pretreated ischemic hearts versus ischemic hearts were compared. Pretreatment of hearts with NOC-18 caused changes in mitochondrial phosphoproteome after ischemia which involved modifications of 10 mitochondrial membrane-bound and 10 matrix proteins. Among them, α-subunit of ATP synthase and adenine nucleotide (ADP/ATP) translocase 1, both of which are considered as potential structural components of mPTP, were identified. We also found that treatment of isolated non-ischemic mitochondria with recombinant PKG did not cause the same protein phosphorylation as pretreatment of hearts with NOC-18. Conclusions: Our study suggests that pretreatment of hearts with NOC-18 causes changes in mitochondrial phosphoproteome after ischemia which involves modifications of certain proteins thought to be involved in the regulation of mPTP opening and intracellular redox state. These proteins may be potential targets for pharmacological preconditioning of the heart. Full article
(This article belongs to the Special Issue Mitochondrial Oxidative Phosphorylation Disorders)
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18 pages, 19067 KiB  
Article
Morphological Stages of Mitochondrial Vacuolar Degeneration in Phenylephrine-Stressed Cardiac Myocytes and in Animal Models and Human Heart Failure
by Antoine H. Chaanine
Medicina 2019, 55(6), 239; https://doi.org/10.3390/medicina55060239 - 3 Jun 2019
Cited by 14 | Viewed by 3375
Abstract
Background and objectives: Derangements in mitochondrial integrity and function constitute an important pathophysiological feature in the pathogenesis of heart failure (HF) and play an important role in myocardial remodeling and systolic dysfunction. In systolic HF, we and others have shown an imbalance [...] Read more.
Background and objectives: Derangements in mitochondrial integrity and function constitute an important pathophysiological feature in the pathogenesis of heart failure (HF) and play an important role in myocardial remodeling and systolic dysfunction. In systolic HF, we and others have shown an imbalance in mitochondrial dynamics toward mitochondrial fission and fragmentation with evidence of mitophagy, mitochondrial vacuolar degeneration, and impairment in mitochondrial oxidative capacity. The morphological stages of mitochondrial vacuolar degeneration have not been defined. We sought to elucidate the progressive stages of mitochondrial vacuolar degeneration, which would serve as a measure to define, morphologically, the severity of mitochondrial damage. Materials and Methods: Transmission electron microscopy was used to study mitochondrial morphology and pathology in phenylephrine-stressed cardiac myocytes in vitro and in left ventricular myocardium from a rat model of pressure overload induced systolic dysfunction and from patients with systolic HF. Results: In phenylephrine-stressed cardiomyocytes for two hours, alterations in mitochondrial cristae morphology (Stage A) and loss and dissolution of mitochondrial cristae in one (Stage B) or multiple (early Stage B→C) mitochondrion area(s) were evident in the earliest stages of mitochondrial vacuolar degeneration. Mitochondrial swelling and progressive dissolution of mitochondrial cristae (advanced Stage B→C), followed by complete loss and dissolution of mitochondrial cristae and permeabilization and destruction of inner mitochondrial membrane (Stage C) then outer mitochondrial membrane rupture (Stage D) constituted advanced stages of mitochondrial vacuolar degeneration. Similar morphological changes in mitochondrial vacuolar degeneration were seen in vivo in animal models and in patients with systolic HF; where about 60–70% of the mitochondria are mainly observed in stages B→C and fewer in stages C and D. Conclusion: Mitochondrial vacuolar degeneration is a prominent mitochondrial morphological feature seen in HF. Defining the progressive stages of mitochondrial vacuolar degeneration would serve as a measure to assess morphologically the severity of mitochondrial damage. Full article
(This article belongs to the Special Issue Mitochondrial Oxidative Phosphorylation Disorders)
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