Special Issue "Mitochondria and Metabolism in Disorders"

A special issue of Metabolites (ISSN 2218-1989).

Deadline for manuscript submissions: closed (31 January 2020).

Special Issue Editor

Dr. Simon-Pierre Gravel
E-Mail Website
Guest Editor
Laboratory of Metabolic Immunopharmacology, Faculté de Pharmacie, Université de Montréal, Montreal, QC, Canada
Interests: skin aging; inflammatory diseases; metabolic reprogramming in cancer; transcriptional regulation; high-throughput approaches for drug and target discovery

Special Issue Information

Dear Colleagues,

Mitochondria are bioenergetic organelles that support cell growth and functions through direct implication in cell signaling and metabolic pathways. These organelles are unique as they control both ATP production and apoptosis, and thus are key deciders of cellular fate. It is now appreciated that mitochondria exhibit structural and metabolic plasticity in response to cellular and microenvironmental contexts, indicating that they play a pivotal role in metabolic adaptation. While mitochondria have been classically isolated and tested under optimized conditions, their constant and dynamic communication with surrounding organelles suggests that their full complexity can only be appreciated in whole cells. Over the past few years, there has been an important progress in the development of new technologies to monitor changes in mitochondrial function in disease. Mitochondrial dysfunction regroups a spectrum of defects associated with altered bioenergetics, oxidative stress, metabolic rewiring, the production of new metabolites, inflammation, and cell death. Besides primary mitochondrial diseases caused by mutations in nuclear or mitochondrial DNA, mitochondrial dysfunction is increasingly recognized has a hallmark of an expanding number of human diseases referred to as secondary mitochondrial diseases. Since mitochondrial dysfunction is shared among a large spectrum of diseases, therapeutic targeting of mitochondria appears as a promising strategy to treat multiple disorders. This Special Issue proposes an exploration of the metabolic bases of disorders characterized by mitochondrial dysfunction. It will introduce state-of-the-art technologies in metabolomics and discuss emerging approaches to study mitochondrial dysfunction. Our objective is to bring together the work of leading researchers to shed light on the current state of mitochondrial research and the challenges associated with the study of mitochondria.

Dr. Simon-Pierre Gravel
Guest Editor

Manuscript Submission Information

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Keywords

  • mitochondrial dysfunction
  • mitochondrial metabolism
  • biomarkers
  • metabolomics
  • metabolic flux analysis

Published Papers (3 papers)

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Research

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Open AccessArticle
Serum Levels of Mitochondrial and Microbial Metabolites Reflect Mitochondrial Dysfunction in Different Stages of Sepsis
Metabolites 2019, 9(10), 196; https://doi.org/10.3390/metabo9100196 - 20 Sep 2019
Abstract
Mechanisms of mitochondrial dysfunction in sepsis are being extensively studied in recent years. During our study, concentrations of microbial phenolic acids and mitochondrial metabolites (succinic, α-ketoglutaric, fumaric, itaconic acids) as indicators of sepsis and mitochondrial dysfunction, respectively, are measured by gas chromatography–mass spectrometry [...] Read more.
Mechanisms of mitochondrial dysfunction in sepsis are being extensively studied in recent years. During our study, concentrations of microbial phenolic acids and mitochondrial metabolites (succinic, α-ketoglutaric, fumaric, itaconic acids) as indicators of sepsis and mitochondrial dysfunction, respectively, are measured by gas chromatography–mass spectrometry (GC–MS) in the blood of critically ill patients at the early and late stages of documented sepsis. The increase in levels of some phenylcarboxylic (phenyllactic (PhLA), p-hydroxyphenylacetic (p-HPhAA), p-hydroxyphenyllactic (p-HPhAA)) acids (PhCAs), simultaneously with a rise in levels of mitochondrial dicarboxylic acids, are mainly detected during the late stage of sepsis, especially succinic acid (up to 100–1000 µM). Itaconic acid is found in low concentrations (0.5–2.3 µM) only at early-stage sepsis. PhCAs in vitro inhibits succinate dehydrogenase (SDH) in isolated mitochondria but, unlike itaconic acid which acts as a competitive inhibitor of SDH, microbial metabolites most likely act on the ubiquinone binding site of the respiratory chain. A close correlation of the level of succinic acid in serum and sepsis-induced organ dysfunction is revealed, moreover the most significant correlation is observed at high concentrations of phenolic microbial metabolites (PhCAs) in late-stage sepsis. These data indicate the promise of such an approach for early detection, monitoring the progression of organ dysfunction and predicting the risk of non-survival in sepsis. Full article
(This article belongs to the Special Issue Mitochondria and Metabolism in Disorders)
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Review

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Open AccessReview
In Vivo Mitochondrial Function in Idiopathic and Genetic Parkinson’s Disease
Metabolites 2020, 10(1), 19; https://doi.org/10.3390/metabo10010019 - 28 Dec 2019
Abstract
Parkinson’s disease (PD) is associated with brain mitochondrial dysfunction. High-energy phosphates (HEPs), which rely on mitochondrial functioning, may be considered potential biomarkers for PD. Phosphorus magnetic resonance spectroscopy (31P-MRS) is a suitable tool to explore in vivo cerebral energetics. We considered [...] Read more.
Parkinson’s disease (PD) is associated with brain mitochondrial dysfunction. High-energy phosphates (HEPs), which rely on mitochondrial functioning, may be considered potential biomarkers for PD. Phosphorus magnetic resonance spectroscopy (31P-MRS) is a suitable tool to explore in vivo cerebral energetics. We considered 10 31P-MRS studies in order to highlight the main findings about brain energetic compounds in patients affected by idiopathic PD and genetic PD. The studies investigated several brain areas such as frontal lobes, occipital lobes, temporoparietal cortex, visual cortex, midbrain, and basal ganglia. Resting-state studies reported contrasting results showing decreased as well as normal or increased HEPs levels in PD patients. Functional studies revealed abnormal PCr + βATP levels in PD subjects during the recovery phase and abnormal values at rest, during activation and recovery in one PD subject with PINK1 gene mutation suggesting that mitochondrial machinery is more impaired in PD patients with PINK1 gene mutation. PD is characterized by energetics impairment both in idiopathic PD as well as in genetic PD, suggesting that mitochondrial dysfunction underlies the disease. Studies are still sparse and sometimes contrasting, maybe due to different methodological approaches. Further studies are needed to better assess the role of mitochondria in the PD development. Full article
(This article belongs to the Special Issue Mitochondria and Metabolism in Disorders)
Open AccessReview
Mitochondrial Dysfunction in the Transition from NASH to HCC
Metabolites 2019, 9(10), 233; https://doi.org/10.3390/metabo9100233 - 16 Oct 2019
Abstract
The liver constantly adapts to meet energy requirements of the whole body. Despite its remarkable adaptative capacity, prolonged exposure of liver cells to harmful environmental cues (such as diets rich in fat, sugar, and cholesterol) results in the development of chronic liver diseases [...] Read more.
The liver constantly adapts to meet energy requirements of the whole body. Despite its remarkable adaptative capacity, prolonged exposure of liver cells to harmful environmental cues (such as diets rich in fat, sugar, and cholesterol) results in the development of chronic liver diseases (including non-alcoholic fatty liver disease (NAFLD) and non-alcoholic steatohepatitis (NASH)) that can progress to hepatocellular carcinoma (HCC). The pathogenesis of these diseases is extremely complex, multifactorial, and poorly understood. Emerging evidence suggests that mitochondrial dysfunction or maladaptation contributes to detrimental effects on hepatocyte bioenergetics, reactive oxygen species (ROS) homeostasis, endoplasmic reticulum (ER) stress, inflammation, and cell death leading to NASH and HCC. The present review highlights the potential contribution of altered mitochondria function to NASH-related HCC and discusses how agents targeting this organelle could provide interesting treatment strategies for these diseases. Full article
(This article belongs to the Special Issue Mitochondria and Metabolism in Disorders)
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