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Metabolites
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Mitochondrial Energy Metabolism in Health and Disease
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Mitochondrial Energy Metabolism in Health and Disease

A special issue of Metabolites (ISSN 2218-1989). This special issue belongs to the section "Cell Metabolism".

Deadline for manuscript submissions: closed (30 June 2022) | Viewed by 16624

Special Issue Editor

Dr. Markus Waldeck-Weiermair

E-Mail Website
Guest Editor
1. Gottfried Schatz Research Center, Chair of Molecular Biology and Biochemistry, Medical University of Graz, Graz, Austria
2. Division of Cardiovascular Medicine, Department of Medicine, Brigham and Women’s Hospital, Harvard Medical School, 75 Francis Street, Boston, MA 02115, USA
Interests: mitochondrial metabolism; mitochondrial bioenergetics; glycolysis; fatty acid oxidation; mitochondrial Ca2+ regulation; reactive oxygen biology; fluorescence live cell imaging

Special Issue Information

Dear Colleagues,

Mitochondria serve as unique organelles within almost all eukaryotic cells. Equipped with the tricarboxylic acid cycle and an electron transport chain, they metabolize nutrients to generate a large amount of energy, making them the so-called ‘powerhouse’ of the cell. However, beside ATP, mitochondria also communicate by providing other important metabolites, such as acetyl-CoA and alpha-ketoglutarate, and thus directly impact the fate of cells. Over the last few decades, disorders of mitochondrial metabolism have been intensively studied and linked to various diseases and aging. Several attempts to optimize specific mitochondrial functions have made them promising therapeutic and life-prolonging targets. This Special Issue, entitled “Mitochondrial Energy Metabolism in Health and Disease”, aims to present a collection of original research papers and review articles on this everlasting topic, which includes physiological effects of mitochondrial metabolites on cellular functions, mitochondrial metabolic disorders and mitochondrial metabolism in cancer progression, and pharmacological treatments to counteract such mitochondrial defects.

Dr. Markus Waldeck-Weiermair
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. Metabolites 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 2700 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

  • mitochondrial respiration
  • oxidative phosphorylation
  • mitochondrial Ca2+ signaling
  • mitochondrial DNA
  • trichloroacetic acid cycle
  • electron transport chain
  • mitochondrial membrane potential
  • redox signaling

Published Papers (5 papers)

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Research

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14 pages, 1358 KiB  
Article
Sigma-1 Receptor Promotes Mitochondrial Bioenergetics by Orchestrating ER Ca2+ Leak during Early ER Stress
by Zhanat Koshenov, Furkan E. Oflaz, Martin Hirtl, Johannes Pilic, Olaf A. Bachkoenig, Benjamin Gottschalk, Corina T. Madreiter-Sokolowski, Rene Rost, Roland Malli and Wolfgang F. Graier
Metabolites 2021, 11(7), 422; https://0-doi-org.brum.beds.ac.uk/10.3390/metabo11070422 - 26 Jun 2021
Cited by 16 | Viewed by 3473
Abstract
The endoplasmic reticulum (ER) is a complex, multifunctional organelle of eukaryotic cells and responsible for the trafficking and processing of nearly 30% of all human proteins. Any disturbance to these processes can cause ER stress, which initiates an adaptive mechanism called unfolded protein [...] Read more.
The endoplasmic reticulum (ER) is a complex, multifunctional organelle of eukaryotic cells and responsible for the trafficking and processing of nearly 30% of all human proteins. Any disturbance to these processes can cause ER stress, which initiates an adaptive mechanism called unfolded protein response (UPR) to restore ER functions and homeostasis. Mitochondrial ATP production is necessary to meet the high energy demand of the UPR, while the molecular mechanisms of ER to mitochondria crosstalk under such stress conditions remain mainly enigmatic. Thus, better understanding the regulation of mitochondrial bioenergetics during ER stress is essential to combat many pathologies involving ER stress, the UPR, and mitochondria. This article investigates the role of Sigma-1 Receptor (S1R), an ER chaperone, has in enhancing mitochondrial bioenergetics during early ER stress using human neuroblastoma cell lines. Our results show that inducing ER stress with tunicamycin, a known ER stressor, greatly enhances mitochondrial bioenergetics in a time- and S1R-dependent manner. This is achieved by enhanced ER Ca2+ leak directed towards mitochondria by S1R during the early phase of ER stress. Our data point to the importance of S1R in promoting mitochondrial bioenergetics and maintaining balanced H2O2 metabolism during early ER stress. Full article
(This article belongs to the Special Issue Mitochondrial Energy Metabolism in Health and Disease)
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18 pages, 5536 KiB  
Article
Dietary Macronutrient Composition Differentially Modulates the Remodeling of Mitochondrial Oxidative Metabolism during NAFLD
by Nathan Kattapuram, Christine Zhang, Muhammed S. Muyyarikkandy, Chaitra Surugihalli, Vaishna Muralidaran, Tabitha Gregory and Nishanth E. Sunny
Metabolites 2021, 11(5), 272; https://0-doi-org.brum.beds.ac.uk/10.3390/metabo11050272 - 26 Apr 2021
Cited by 6 | Viewed by 3403
Abstract
Diets rich in fats and carbohydrates aggravate non-alcoholic fatty liver disease (NAFLD), of which mitochondrial dysfunction is a central feature. It is not clear whether a high-carbohydrate driven ‘lipogenic’ diet differentially affects mitochondrial oxidative remodeling compared to a high-fat driven ‘oxidative’ environment. We [...] Read more.
Diets rich in fats and carbohydrates aggravate non-alcoholic fatty liver disease (NAFLD), of which mitochondrial dysfunction is a central feature. It is not clear whether a high-carbohydrate driven ‘lipogenic’ diet differentially affects mitochondrial oxidative remodeling compared to a high-fat driven ‘oxidative’ environment. We hypothesized that the high-fat driven ‘oxidative’ environment will chronically sustain mitochondrial oxidative function, hastening metabolic dysfunction during NAFLD. Mice (C57BL/6NJ) were reared on a low-fat (LF; 10% fat calories), high-fat (HF; 60% fat calories), or high-fructose/high-fat (HFr/HF; 25% fat and 34.9% fructose calories) diet for 10 weeks. De novo lipogenesis was determined by measuring the incorporation of deuterium from D2O into newly synthesized liver lipids using nuclear magnetic resonance (NMR) spectroscopy. Hepatic mitochondrial metabolism was profiled under fed and fasted states by the incubation of isolated mitochondria with [13C3]pyruvate, targeted metabolomics of tricarboxylic acid (TCA) cycle intermediates, estimates of oxidative phosphorylation (OXPHOS), and hepatic gene and protein expression. De novo lipogenesis was higher in the HFr/HF mice compared to their HF counterparts. Contrary to our expectations, hepatic oxidative function after fasting was induced in the HFr/HF group. This differential induction of mitochondrial oxidative function by the high fructose-driven ‘lipogenic’ environment could influence the progressive severity of hepatic insulin resistance. Full article
(This article belongs to the Special Issue Mitochondrial Energy Metabolism in Health and Disease)
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Review

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13 pages, 1346 KiB  
Review
Effects of Metabolic Disorders in Immune Cells and Synoviocytes on the Development of Rheumatoid Arthritis
by Alexander V. Blagov, Andrey V. Grechko, Nikita G. Nikiforov, Alexander D. Zhuravlev, Nikolay K. Sadykhov and Alexander N. Orekhov
Metabolites 2022, 12(7), 634; https://0-doi-org.brum.beds.ac.uk/10.3390/metabo12070634 - 11 Jul 2022
Cited by 4 | Viewed by 1502
Abstract
Rheumatoid arthritis (RA) is a progressive autoimmune disease that affects the joints. It has been proven that, with the development of RA, there are changes in the metabolism of cells located in the focus of inflammation. In this article, we describe the connection [...] Read more.
Rheumatoid arthritis (RA) is a progressive autoimmune disease that affects the joints. It has been proven that, with the development of RA, there are changes in the metabolism of cells located in the focus of inflammation. In this article, we describe the connection between metabolism and inflammation in the context of rheumatoid arthritis. We consider in detail the changes in metabolic processes and their subsequent immunomodulatory effects. In particular, we consider how changes in mitochondrial functioning lead to the modulation of metabolism in rheumatoid arthritis. We also describe the main features of the metabolism in cells present in the synovial membrane during inflammation, and we discuss possible targets for the therapy of rheumatoid arthritis. Full article
(This article belongs to the Special Issue Mitochondrial Energy Metabolism in Health and Disease)
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10 pages, 1254 KiB  
Review
Diversion of Acetyl CoA to 3-Methylglutaconic Acid Caused by Discrete Inborn Errors of Metabolism
by Dylan E. Jones, Elizabeth A. Jennings and Robert O. Ryan
Metabolites 2022, 12(5), 377; https://0-doi-org.brum.beds.ac.uk/10.3390/metabo12050377 - 21 Apr 2022
Cited by 3 | Viewed by 2253
Abstract
A growing number of inborn errors of metabolism (IEM) have been identified that manifest 3-methylglutaconic (3MGC) aciduria as a phenotypic feature. In primary 3MGC aciduria, IEM-dependent deficiencies in leucine pathway enzymes prevent catabolism of trans-3MGC CoA. Consequently, this metabolite is converted to [...] Read more.
A growing number of inborn errors of metabolism (IEM) have been identified that manifest 3-methylglutaconic (3MGC) aciduria as a phenotypic feature. In primary 3MGC aciduria, IEM-dependent deficiencies in leucine pathway enzymes prevent catabolism of trans-3MGC CoA. Consequently, this metabolite is converted to 3MGC acid and excreted in urine. In secondary 3MGC aciduria, however, no leucine metabolism pathway enzyme deficiencies exist. These IEMs affect mitochondrial membrane structure, electron transport chain function or ATP synthase subunits. As a result, acetyl CoA oxidation via the TCA cycle slows and acetyl CoA is diverted to trans-3MGC CoA, and then to 3MGC acid. Whereas the trans diastereomer of 3MGC CoA is the only biologically relevant diastereomer, the urine of affected subjects contains both cis- and trans-3MGC acids. Studies have revealed that trans-3MGC CoA is susceptible to isomerization to cis-3MGC CoA. Once formed, cis-3MGC CoA undergoes intramolecular cyclization, forming an anhydride that, upon hydrolysis, yields cis-3MGC acid. Alternatively, cis-3MGC anhydride can acylate protein lysine side chains. Once formed, cis-3MGCylated proteins can be deacylated by the NAD+-dependent enzyme, sirtuin 4. Taken together, the excretion of 3MGC acid in secondary 3MGC aciduria represents a barometer of defective mitochondrial function. Full article
(This article belongs to the Special Issue Mitochondrial Energy Metabolism in Health and Disease)
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14 pages, 871 KiB  
Review
Mitochondria in Myelinating Oligodendrocytes: Slow and Out of Breath?
by Niklas Meyer and Johanne Egge Rinholm
Metabolites 2021, 11(6), 359; https://0-doi-org.brum.beds.ac.uk/10.3390/metabo11060359 - 05 Jun 2021
Cited by 30 | Viewed by 4364
Abstract
Myelin is a lipid-rich membrane that wraps around axons and facilitates rapid action potential propagation. In the brain, myelin is synthesized and maintained by oligodendrocytes. These cells have a high metabolic demand that requires mitochondrial ATP production during the process of myelination, but [...] Read more.
Myelin is a lipid-rich membrane that wraps around axons and facilitates rapid action potential propagation. In the brain, myelin is synthesized and maintained by oligodendrocytes. These cells have a high metabolic demand that requires mitochondrial ATP production during the process of myelination, but they rely less on mitochondrial respiration after myelination is complete. Mitochondria change in morphology and distribution during oligodendrocyte development. Furthermore, the morphology and dynamic properties of mitochondria in mature oligodendrocytes seem different from any other brain cell. Here, we first give a brief introduction to oligodendrocyte biology and function. We then review the current knowledge on oligodendrocyte metabolism and discuss how the available data on mitochondrial morphology and mobility as well as transcriptome and proteome studies can shed light on the metabolic properties of oligodendrocytes. Full article
(This article belongs to the Special Issue Mitochondrial Energy Metabolism in Health and Disease)
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