Journal articles: 'Mitochondrial diseases'

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Tang, Xiaoqiang, Xiao-Feng Chen, Hou-Zao Chen, and De-Pei Liu. "Mitochondrial Sirtuins in cardiometabolic diseases." Clinical Science 131, no. 16 (July 24, 2017): 2063–78. http://dx.doi.org/10.1042/cs20160685.

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Mitochondria are heterogeneous and essentially contribute to cellular functions and tissue homeostasis. Mitochondrial dysfunction compromises overall cell functioning, tissue damage, and diseases. The advances in mitochondrion biology increase our understanding of mitochondrial dynamics, bioenergetics, and redox homeostasis, and subsequently, their functions in tissue homeostasis and diseases, including cardiometabolic diseases (CMDs). The functions of mitochondria mainly rely on the enzymes in their matrix. Sirtuins are a family of NAD+-dependent deacylases and ADP-ribosyltransferases. Three members of the Sirtuin family (SIRT3, SIRT4, and SIRT5) are located in the mitochondrion. These mitochondrial Sirtuins regulate energy and redox metabolism as well as mitochondrial dynamics in the mitochondrial matrix and are involved in cardiovascular homeostasis and CMDs. In this review, we discuss the advances in our understanding of mitochondrial Sirtuins in mitochondrion biology and CMDs, including cardiac remodeling, pulmonary artery hypertension, and vascular dysfunction. The potential therapeutic strategies by targetting mitochondrial Sirtuins to improve mitochondrial function in CMDs are also addressed.

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Fu, Ailing. "Mitotherapy as a Novel Therapeutic Strategy for Mitochondrial Diseases." Current Molecular Pharmacology 13, no. 1 (January 15, 2020): 41–49. http://dx.doi.org/10.2174/1874467212666190920144115.

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Background: The mitochondrion is a multi-functional organelle that is mainly responsible for energy supply in the mammalian cells. Over 100 human diseases are attributed to mitochondrial dysfunction. Mitochondrial therapy (mitotherapy) aims to transfer functional exogenous mitochondria into mitochondria-defective cells for recovery of the cell viability and consequently, prevention of the disease progress. Conclusion: Mitotherapy makes the of modulation of cell survival possible, and it would be a potential therapeutic strategy for mitochondrial diseases. Objective: The review summarizes the evidence on exogenous mitochondria that can directly enter mammalian cells for disease therapy following local and intravenous administration, and suggests that when healthy cells donate their mitochondria to damaged cells, the mitochondrial transfer between cells serve as a new mode of cell rescue. Then the transferred mitochondria play their roles in recipient cells, including energy production and maintenance of cell function.

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Macdonald, Ruby, Katy Barnes, Christopher Hastings, and Heather Mortiboys. "Mitochondrial abnormalities in Parkinson's disease and Alzheimer's disease: can mitochondria be targeted therapeutically?" Biochemical Society Transactions 46, no. 4 (July 19, 2018): 891–909. http://dx.doi.org/10.1042/bst20170501.

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Mitochondrial abnormalities have been identified as a central mechanism in multiple neurodegenerative diseases and, therefore, the mitochondria have been explored as a therapeutic target. This review will focus on the evidence for mitochondrial abnormalities in the two most common neurodegenerative diseases, Parkinson's disease and Alzheimer's disease. In addition, we discuss the main strategies which have been explored in these diseases to target the mitochondria for therapeutic purposes, focusing on mitochondrially targeted antioxidants, peptides, modulators of mitochondrial dynamics and phenotypic screening outcomes.

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Wang, Sheng-Fan, Shiuan Chen, Ling-Ming Tseng, and Hsin-Chen Lee. "Role of the mitochondrial stress response in human cancer progression." Experimental Biology and Medicine 245, no. 10 (April 23, 2020): 861–78. http://dx.doi.org/10.1177/1535370220920558.

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Mitochondria are important organelles that are responsible for cellular energy metabolism, cellular redox/calcium homeostasis, and cell death regulation in mammalian cells. Mitochondrial dysfunction is involved in various diseases, such as neurodegenerative diseases, cardiovascular diseases, immune disorders, and cancer. Defective mitochondria and metabolism remodeling are common characteristics in cancer cells. Several factors, such as mitochondrial DNA copy number changes, mitochondrial DNA mutations, mitochondrial enzyme defects, and mitochondrial dynamic changes, may contribute to mitochondrial dysfunction in cancer cells. Some lines of evidence have shown that mitochondrial dysfunction may promote cancer progression. Here, several mitochondrial stress responses, including the mitochondrial unfolded protein response and the integrated stress response, and several mitochondrion-derived molecules (reactive oxygen species, calcium, oncometabolites, and others) are reviewed; these pathways and molecules are considered to act as retrograde signaling regulators in the development and progression of cancer. Targeting these components of the mitochondrial stress response may be an important strategy for cancer treatment. Impact statement Dysregulated mitochondria often occurred in cancers. Mitochondrial dysfunction might contribute to cancer progression. We reviewed several mitochondrial stresses in cancers. Mitochondrial stress responses might contribute to cancer progression. Several mitochondrion-derived molecules (ROS, Ca2+, oncometabolites, exported mtDNA, mitochondrial double-stranded RNA, humanin, and MOTS-c), integrated stress response, and mitochondrial unfolded protein response act as retrograde signaling pathways and might be critical in the development and progression of cancer. Targeting these mitochondrial stress responses may be an important strategy for cancer treatment.

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Habbane, Mouna, Julio Montoya, Taha Rhouda, Yousra Sbaoui, Driss Radallah, and Sonia Emperador. "Human Mitochondrial DNA: Particularities and Diseases." Biomedicines 9, no. 10 (October 1, 2021): 1364. http://dx.doi.org/10.3390/biomedicines9101364.

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Mitochondria are the cell’s power site, transforming energy into a form that the cell can employ for necessary metabolic reactions. These organelles present their own DNA. Although it codes for a small number of genes, mutations in mtDNA are common. Molecular genetics diagnosis allows the analysis of DNA in several areas such as infectiology, oncology, human genetics and personalized medicine. Knowing that the mitochondrial DNA is subject to several mutations which have a direct impact on the metabolism of the mitochondrion leading to many diseases, it is therefore necessary to detect these mutations in the patients involved. To date numerous mitochondrial mutations have been described in humans, permitting confirmation of clinical diagnosis, in addition to a better management of the patients. Therefore, different techniques are employed to study the presence or absence of mitochondrial mutations. However, new mutations are discovered, and to determine if they are the cause of disease, different functional mitochondrial studies are undertaken using transmitochondrial cybrid cells that are constructed by fusion of platelets of the patient that presents the mutation, with rho osteosarcoma cell line. Moreover, the contribution of next generation sequencing allows sequencing of the entire human genome within a single day and should be considered in the diagnosis of mitochondrial mutations.

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Ngo, Jennifer, Corey Osto, Frankie Villalobos, and Orian S. Shirihai. "Mitochondrial Heterogeneity in Metabolic Diseases." Biology 10, no. 9 (September 17, 2021): 927. http://dx.doi.org/10.3390/biology10090927.

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Mitochondria have distinct architectural features and biochemical functions consistent with cell-specific bioenergetic needs. However, as imaging and isolation techniques advance, heterogeneity amongst mitochondria has been observed to occur within the same cell. Moreover, mitochondrial heterogeneity is associated with functional differences in metabolic signaling, fuel utilization, and triglyceride synthesis. These phenotypic associations suggest that mitochondrial subpopulations and heterogeneity influence the risk of metabolic diseases. This review examines the current literature regarding mitochondrial heterogeneity in the pancreatic beta-cell and renal proximal tubules as they exist in the pathological and physiological states; specifically, pathological states of glucolipotoxicity, progression of type 2 diabetes, and kidney diseases. Emphasis will be placed on the benefits of balancing mitochondrial heterogeneity and how the disruption of balancing heterogeneity leads to impaired tissue function and disease onset.

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Caffarra Malvezzi, Cristina, Aderville Cabassi, and Michele Miragoli. "Mitochondrial mechanosensor in cardiovascular diseases." Vascular Biology 2, no. 1 (July 22, 2020): R85—R92. http://dx.doi.org/10.1530/vb-20-0002.

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The role of mitochondria in cardiac tissue is of utmost importance due to the dynamic nature of the heart and its energetic demands, necessary to assure its proper beating function. Recently, other important mitochondrial roles have been discovered, namely its contribution to intracellular calcium handling in normal and pathological myocardium. Novel investigations support the fact that during the progression toward heart failure, mitochondrial calcium machinery is compromised due to its morphological, structural and biochemical modifications resulting in facilitated arrhythmogenesis and heart failure development. The interaction between mitochondria and sarcomere directly affect cardiomyocyte excitation-contraction and is also involved in mechano-transduction through the cytoskeletal proteins that tether together the mitochondria and the sarcoplasmic reticulum. The focus of this review is to briefly elucidate the role of mitochondria as (mechano) sensors in the heart.

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Che, Ruochen, Yanggang Yuan, Songming Huang, and Aihua Zhang. "Mitochondrial dysfunction in the pathophysiology of renal diseases." American Journal of Physiology-Renal Physiology 306, no. 4 (February 15, 2014): F367—F378. http://dx.doi.org/10.1152/ajprenal.00571.2013.

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Mitochondrial dysfunction has gained recognition as a contributing factor in many diseases. The kidney is a kind of organ with high energy demand, rich in mitochondria. As such, mitochondrial dysfunction in the kidney plays a critical role in the pathogenesis of kidney diseases. Despite the recognized importance mitochondria play in the pathogenesis of the diseases, there is limited understanding of various aspects of mitochondrial biology. This review examines the physiology and pathophysiology of mitochondria. It begins by discussing mitochondrial structure, mitochondrial DNA, mitochondrial reactive oxygen species production, mitochondrial dynamics, and mitophagy, before turning to inherited mitochondrial cytopathies in kidneys (inherited or sporadic mitochondrial DNA or nuclear DNA mutations in genes that affect mitochondrial function). Glomerular diseases, tubular defects, and other renal diseases are then discussed. Next, acquired mitochondrial dysfunction in kidney diseases is discussed, emphasizing the role of mitochondrial dysfunction in the pathogenesis of chronic kidney disease and acute kidney injury, as their prevalence is increasing. Finally, it summarizes the possible beneficial effects of mitochondrial-targeted therapeutic agents for treatment of mitochondrial dysfunction-mediated kidney injury-genetic therapies, antioxidants, thiazolidinediones, sirtuins, and resveratrol-as mitochondrial-based drugs may offer potential treatments for renal diseases.

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Sui, Guo-Yan, Feng Wang, Jin Lee, and Yoon Seok Roh. "Mitochondrial Control in Inflammatory Gastrointestinal Diseases." International Journal of Molecular Sciences 23, no. 23 (November 28, 2022): 14890. http://dx.doi.org/10.3390/ijms232314890.

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Mitochondria play a central role in the pathophysiology of inflammatory bowel disease (IBD) and colorectal cancer (CRC). The maintenance of mitochondrial function is necessary for a stable immune system. Mitochondrial dysfunction in the gastrointestinal system leads to the excessive activation of multiple inflammatory signaling pathways, leading to IBD and increased severity of CRC. In this review, we focus on the mitochondria and inflammatory signaling pathways and its related gastrointestinal diseases.

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Kochar Kaur, Kulvinder, Gautam Allahbadia, and Mandeep Singh. "A update on role of mitochondrial transport in etiopathogenesis & management of various CNS diseases, neurodegenerative diseases, immunometabolic diseases, cancer, viral infections inclusive of COVID 19 disease-a systematic review." Journal of Diabetes, Metabolic Disorders & Control 8, no. 2 (November 29, 2021): 91–103. http://dx.doi.org/10.15406/jdmdc.2021.08.00228.

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Mitochondria represent complicated intra cellular organelles which classically have been isolated as the powerhouse of eukaryotic cells secondary to their key part in the bioenergetic metabolism. In more recent decades, the escalation of mitochondrial research has got invoked in researchers that have illustrated that these organelles are much greater than just simple powerhouse of cell, possessing the capacity of other crucial parts like signaling platforms which control cell metabolism, proliferation, and demise besides immunological reactions. In the form of crucial controllers, mitochondria on impairment are implicated, in the etiopathogenesis of a wide variety of metabolic neurodegenerative, immune in addition to neoplastic conditions. Much more recently, greater importance has been given by the researchers with the capacity they possess with regards to. Intercellular transfer which might implicate whole mitochondria, mitochondrial Genome or other mitochondrial constituents. The Intercellular transfer of mitochondria that by definition as horizontal mitochondrial transport can take place in mammalian cells both in vivo, as well as in vitro in addition to both physiological along with pathological situations. Mitochondrial shifting can yield an external mitochondrial source that can restore the normal mitochondrial replacing the mitochondria having undergone impairment, thus resulting in the enhancement of mitochondrial quality, or in case of tumor, altering their functional properties, in addition to chemotherapy responsiveness. Here we have tried to provide a comprehensive review with regards to the biological significance, modes beneath the event, besides their implication in various pathophysiological disorders, emphasizing its treatment potential with regards to diseases where mitochondrial impairment is the primary etiopathogenetic cause of the disease.

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Basu, Urmimala, Alicia M. Bostwick, Kalyan Das, Kristin E. Dittenhafer-Reed, and Smita S. Patel. "Structure, mechanism, and regulation of mitochondrial DNA transcription initiation." Journal of Biological Chemistry 295, no. 52 (October 30, 2020): 18406–25. http://dx.doi.org/10.1074/jbc.rev120.011202.

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Mitochondria are specialized compartments that produce requisite ATP to fuel cellular functions and serve as centers of metabolite processing, cellular signaling, and apoptosis. To accomplish these roles, mitochondria rely on the genetic information in their small genome (mitochondrial DNA) and the nucleus. A growing appreciation for mitochondria's role in a myriad of human diseases, including inherited genetic disorders, degenerative diseases, inflammation, and cancer, has fueled the study of biochemical mechanisms that control mitochondrial function. The mitochondrial transcriptional machinery is different from nuclear machinery. The in vitro re-constituted transcriptional complexes of Saccharomyces cerevisiae (yeast) and humans, aided with high-resolution structures and biochemical characterizations, have provided a deeper understanding of the mechanism and regulation of mitochondrial DNA transcription. In this review, we will discuss recent advances in the structure and mechanism of mitochondrial transcription initiation. We will follow up with recent discoveries and formative findings regarding the regulatory events that control mitochondrial DNA transcription, focusing on those involved in cross-talk between the mitochondria and nucleus.

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Khotina, Victoria A., Andrey Y. Vinokurov, Mariam Bagheri Ekta, Vasily N. Sukhorukov, and Alexander N. Orekhov. "Creation of Mitochondrial Disease Models Using Mitochondrial DNA Editing." Biomedicines 11, no. 2 (February 12, 2023): 532. http://dx.doi.org/10.3390/biomedicines11020532.

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Mitochondrial diseases are a large class of human hereditary diseases, accompanied by the dysfunction of mitochondria and the disruption of cellular energy synthesis, that affect various tissues and organ systems. Mitochondrial DNA mutation-caused disorders are difficult to study because of the insufficient number of clinical cases and the challenges of creating appropriate models. There are many cellular models of mitochondrial diseases, but their application has a number of limitations. The most proper and promising models of mitochondrial diseases are animal models, which, unfortunately, are quite rare and more difficult to develop. The challenges mainly arise from the structural features of mitochondria, which complicate the genetic editing of mitochondrial DNA. This review is devoted to discussing animal models of human mitochondrial diseases and recently developed approaches used to create them. Furthermore, this review discusses mitochondrial diseases and studies of metabolic disorders caused by the mitochondrial DNA mutations underlying these diseases.

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Brunetti, Dario, Werner Dykstra, Stephanie Le, Annika Zink, and Alessandro Prigione. "Mitochondria in Neurogenesis: Implications for Mitochondrial Diseases." Stem Cells 39, no. 10 (June 5, 2021): 1289–97. http://dx.doi.org/10.1002/stem.3425.

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Abstract Mitochondria are organelles with recognized key roles in cellular homeostasis, including bioenergetics, redox, calcium signaling, and cell death. Mitochondria are essential for neuronal function, given the high energy demands of the human brain. Consequently, mitochondrial diseases affecting oxidative phosphorylation (OXPHOS) commonly exhibit neurological impairment. Emerging evidence suggests that mitochondria are important not only for mature postmitotic neurons but also for the regulation of neural progenitor cells (NPCs) during the process of neurogenesis. These recent findings put mitochondria as central regulator of cell fate decisions during brain development. OXPHOS mutations may disrupt the function of NPCs and thereby impair the metabolic programming required for neural fate commitment. Promoting the mitochondrial function of NPCs could therefore represent a novel interventional approach against incurable mitochondrial diseases.

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Lin, Tsu-Kung, Shang-Der Chen, Yao-Chung Chuang, Min-Yu Lan, Jiin-Haur Chuang, Pei-Wen Wang, Te-Yao Hsu, et al. "Mitochondrial Transfer of Wharton’s Jelly Mesenchymal Stem Cells Eliminates Mutation Burden and Rescues Mitochondrial Bioenergetics in Rotenone-Stressed MELAS Fibroblasts." Oxidative Medicine and Cellular Longevity 2019 (May 22, 2019): 1–17. http://dx.doi.org/10.1155/2019/9537504.

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Wharton’s jelly mesenchymal stem cells (WJMSCs) transfer healthy mitochondria to cells harboring a mitochondrial DNA (mtDNA) defect. Mitochondrial myopathy, encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) is one of the major subgroups of mitochondrial diseases, caused by the mt.3243A>G point mutation in the mitochondrial tRNALeu(UUR) gene. The specific aim of the study is to investigate whether WJMSCs exert therapeutic effect for mitochondrial dysfunction in cells of MELAS patient through donating healthy mitochondria. We herein demonstrate that WJMSCs transfer healthy mitochondria into rotenone-stressed fibroblasts of a MELAS patient, thereby eliminating mutation burden and rescuing mitochondrial functions. In the coculture system in vitro study, WJMSCs transferred healthy mitochondria to rotenone-stressed MELAS fibroblasts. By inhibiting actin polymerization to block tunneling nanotubes (TNTs), the WJMSC-conducted mitochondrial transfer was abrogated. After mitochondrial transfer, the mt.3243A>G mutation burden of MELAS fibroblasts was reduced to an undetectable level, with long-term retention. Sequencing results confirmed that the transferred mitochondria were donated from WJMSCs. Furthermore, mitochondrial transfer of WJMSCs to MELAS fibroblasts improves mitochondrial functions and cellular performance, including protein translation of respiratory complexes, ROS overexpression, mitochondrial membrane potential, mitochondrial morphology and bioenergetics, cell proliferation, mitochondrion-dependent viability, and apoptotic resistance. This study demonstrates that WJMSCs exert bioenergetic therapeutic effects through mitochondrial transfer. This finding paves the way for the development of innovative treatments for MELAS and other mitochondrial diseases.

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Park, Anna, Mihee Oh, Su Jeong Lee, Kyoung-Jin Oh, Eun-Woo Lee, Sang Chul Lee, Kwang-Hee Bae, Baek Soo Han, and Won Kon Kim. "Mitochondrial Transplantation as a Novel Therapeutic Strategy for Mitochondrial Diseases." International Journal of Molecular Sciences 22, no. 9 (April 30, 2021): 4793. http://dx.doi.org/10.3390/ijms22094793.

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Mitochondria are the major source of intercellular bioenergy in the form of ATP. They are necessary for cell survival and play many essential roles such as maintaining calcium homeostasis, body temperature, regulation of metabolism and apoptosis. Mitochondrial dysfunction has been observed in variety of diseases such as cardiovascular disease, aging, type 2 diabetes, cancer and degenerative brain disease. In other words, the interpretation and regulation of mitochondrial signals has the potential to be applied as a treatment for various diseases caused by mitochondrial disorders. In recent years, mitochondrial transplantation has increasingly been a topic of interest as an innovative strategy for the treatment of mitochondrial diseases by augmentation and replacement of mitochondria. In this review, we focus on diseases that are associated with mitochondrial dysfunction and highlight studies related to the rescue of tissue-specific mitochondrial disorders. We firmly believe that mitochondrial transplantation is an optimistic therapeutic approach in finding a potentially valuable treatment for a variety of mitochondrial diseases.

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Jang, Yoon-ha, Sae Ryun Ahn, Ji-yeon Shim, and Kwang-il Lim. "Engineering Genetic Systems for Treating Mitochondrial Diseases." Pharmaceutics 13, no. 6 (May 28, 2021): 810. http://dx.doi.org/10.3390/pharmaceutics13060810.

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Mitochondria are intracellular energy generators involved in various cellular processes. Therefore, mitochondrial dysfunction often leads to multiple serious diseases, including neurodegenerative and cardiovascular diseases. A better understanding of the underlying mitochondrial dysfunctions of the molecular mechanism will provide important hints on how to mitigate the symptoms of mitochondrial diseases and eventually cure them. In this review, we first summarize the key parts of the genetic processes that control the physiology and functions of mitochondria and discuss how alterations of the processes cause mitochondrial diseases. We then list up the relevant core genetic components involved in these processes and explore the mutations of the components that link to the diseases. Lastly, we discuss recent attempts to apply multiple genetic methods to alleviate and further reverse the adverse effects of the core component mutations on the physiology and functions of mitochondria.

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Mu, Jian-Kang, Yan-Qin Li, Ting-Ting Shi, Li-Ping Yu, Ya-Qin Yang, Wen Gu, Jing-Ping Li, Jie Yu, and Xing-Xin Yang. "Remedying the Mitochondria to Cure Human Diseases by Natural Products." Oxidative Medicine and Cellular Longevity 2020 (July 14, 2020): 1–18. http://dx.doi.org/10.1155/2020/5232614.

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Mitochondria are the ‘engine’ of cells. Mitochondrial dysfunction is an important mechanism in many human diseases. Many natural products could remedy the mitochondria to alleviate mitochondria-involved diseases. In this review, we summarized the current knowledge of the relationship between the mitochondria and human diseases and the regulation of natural products to the mitochondria. We proposed that the development of mitochondrial regulators/nutrients from natural products to remedy mitochondrial dysfunction represents an attractive strategy for a mitochondria-involved disorder therapy. Moreover, investigating the mitochondrial regulation of natural products can potentiate the in-depth comprehension of the mechanism of action of natural products.

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Zhou, Zhengqiu, Grant Austin, Lyndsay Young, Lance Johnson, and Ramon Sun. "Mitochondrial Metabolism in Major Neurological Diseases." Cells 7, no. 12 (November 23, 2018): 229. http://dx.doi.org/10.3390/cells7120229.

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Mitochondria are bilayer sub-cellular organelles that are an integral part of normal cellular physiology. They are responsible for producing the majority of a cell’s ATP, thus supplying energy for a variety of key cellular processes, especially in the brain. Although energy production is a key aspect of mitochondrial metabolism, its role extends far beyond energy production to cell signaling and epigenetic regulation–functions that contribute to cellular proliferation, differentiation, apoptosis, migration, and autophagy. Recent research on neurological disorders suggest a major metabolic component in disease pathophysiology, and mitochondria have been shown to be in the center of metabolic dysregulation and possibly disease manifestation. This review will discuss the basic functions of mitochondria and how alterations in mitochondrial activity lead to neurological disease progression.

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Shibata, Tatsuya, Toshinari Takahashi, Eio Yamada, Akiko Kimura, Hiroshi Nishikawa, Hiroyoshi Hayakawa, Nobuhiko Nomura, and Junichi Mitsuyama. "T-2307 Causes Collapse of Mitochondrial Membrane Potential in Yeast." Antimicrobial Agents and Chemotherapy 56, no. 11 (September 4, 2012): 5892–97. http://dx.doi.org/10.1128/aac.05954-11.

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ABSTRACTT-2307, an arylamidine compound, has been previously reported to have broad-spectrumin vitroandin vivoantifungal activities against clinically significant pathogens, includingCandidaspecies,Cryptococcus neoformans, andAspergillusspecies, and is now undergoing clinical trials. Here we investigated the mechanism of action of T-2307 using yeast cells and mitochondria isolated from yeast and rat liver. Nonfermentative growth ofCandida albicansandSaccharomyces cerevisiaein glycerol medium, in which yeasts relied on mitochondrial respiratory function, was inhibited at 0.001 to 0.002 μg/ml (0.002 to 0.004 μM) of T-2307. However, fermentative growth in dextrose medium was not inhibited by T-2307. Microscopic examination using Mitotracker fluorescent dye, a cell-permeant mitochondrion-specific probe, demonstrated that T-2307 impaired the mitochondrial function ofC. albicansandS. cerevisiaeat concentrations near the MIC in glycerol medium. T-2307 collapsed the mitochondrial membrane potential in mitochondria isolated fromS. cerevisiaeat 20 μM. On the other hand, in isolated rat liver mitochondria, T-2307 did not have any effect on the mitochondrial membrane potential at 10 mM. Moreover, T-2307 had little inhibitory and stimulatory effect on mitochondrial respiration in rat liver mitochondria. In conclusion, T-2307 selectively disrupted yeast mitochondrial function, and it was also demonstrated that the fungal mitochondrion is an attractive antifungal target.

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Wang, Li, Qiang Wu, Zhijia Fan, Rufeng Xie, Zhicheng Wang, and Yuan Lu. "Platelet mitochondrial dysfunction and the correlation with human diseases." Biochemical Society Transactions 45, no. 6 (October 20, 2017): 1213–23. http://dx.doi.org/10.1042/bst20170291.

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The platelet is considered as an accessible and valuable tool to study mitochondrial function, owing to its greater content of fully functional mitochondria compared with other metabolically active organelles. Different lines of studies have demonstrated that mitochondria in platelets have function far more than thrombogenesis regulation, and beyond hemostasis, platelet mitochondrial dysfunction has also been used for studying mitochondrial-related diseases. In this review, the interplay between platelet mitochondrial dysfunction and oxidative stress, mitochondrial DNA lesions, electron transfer chain impairments, mitochondrial apoptosis and mitophagy has been outlined. Meanwhile, considerable efforts have been made towards understanding the role of platelet mitochondrial dysfunction in human diseases, such as diabetes mellitus, sepsis and neurodegenerative disorders. Alongside this, we have also articulated our perspectives on the development of potential biomarkers of platelet mitochondrial dysfunction in mitochondrial-related diseases.

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Jackson, Thomas Daniel, Catherine Sarah Palmer, and Diana Stojanovski. "Mitochondrial diseases caused by dysfunctional mitochondrial protein import." Biochemical Society Transactions 46, no. 5 (October 4, 2018): 1225–38. http://dx.doi.org/10.1042/bst20180239.

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Mitochondria are essential organelles which perform complex and varied functions within eukaryotic cells. Maintenance of mitochondrial health and functionality is thus a key cellular priority and relies on the organelle's extensive proteome. The mitochondrial proteome is largely encoded by nuclear genes, and mitochondrial proteins must be sorted to the correct mitochondrial sub-compartment post-translationally. This essential process is carried out by multimeric and dynamic translocation and sorting machineries, which can be found in all four mitochondrial compartments. Interestingly, advances in the diagnosis of genetic disease have revealed that mutations in various components of the human import machinery can cause mitochondrial disease, a heterogenous and often severe collection of disorders associated with energy generation defects and a multisystem presentation often affecting the cardiovascular and nervous systems. Here, we review our current understanding of mitochondrial protein import systems in human cells and the molecular basis of mitochondrial diseases caused by defects in these pathways.

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Singh, Gyanesh, U. C. Pachouri, Devika Chanu Khaidem, Aman Kundu, Chirag Chopra, and Pushplata Singh. "Mitochondrial DNA Damage and Diseases." F1000Research 4 (July 1, 2015): 176. http://dx.doi.org/10.12688/f1000research.6665.1.

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Various endogenous and environmental factors can cause mitochondrial DNA (mtDNA) damage. One of the reasons for enhanced mtDNA damage could be its proximity to the source of oxidants, and lack of histone-like protective proteins. Moreover, mitochondria contain inadequate DNA repair pathways, and, diminished DNA repair capacity may be one of the factors responsible for high mutation frequency of the mtDNA. mtDNA damage might cause impaired mitochondrial function, and, unrepaired mtDNA damage has been frequently linked with several diseases. Exploration of mitochondrial perspective of diseases might lead to a better understanding of several diseases, and will certainly open new avenues for detection, cure, and prevention of ailments.

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Patergnani, Simone, Esmaa Bouhamida, Sara Leo, Paolo Pinton, and Alessandro Rimessi. "Mitochondrial Oxidative Stress and “Mito-Inflammation”: Actors in the Diseases." Biomedicines 9, no. 2 (February 20, 2021): 216. http://dx.doi.org/10.3390/biomedicines9020216.

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A decline in mitochondrial redox homeostasis has been associated with the development of a wide range of inflammatory-related diseases. Continue discoveries demonstrate that mitochondria are pivotal elements to trigger inflammation and stimulate innate immune signaling cascades to intensify the inflammatory response at front of different stimuli. Here, we review the evidence that an exacerbation in the levels of mitochondrial-derived reactive oxygen species (ROS) contribute to mito-inflammation, a new concept that identifies the compartmentalization of the inflammatory process, in which the mitochondrion acts as central regulator, checkpoint, and arbitrator. In particular, we discuss how ROS contribute to specific aspects of mito-inflammation in different inflammatory-related diseases, such as neurodegenerative disorders, cancer, pulmonary diseases, diabetes, and cardiovascular diseases. Taken together, these observations indicate that mitochondrial ROS influence and regulate a number of key aspects of mito-inflammation and that strategies directed to reduce or neutralize mitochondrial ROS levels might have broad beneficial effects on inflammatory-related diseases.

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Corkery-Hayward, Madeleine, and Louise A. Metherell. "Adrenal Dysfunction in Mitochondrial Diseases." International Journal of Molecular Sciences 24, no. 2 (January 6, 2023): 1126. http://dx.doi.org/10.3390/ijms24021126.

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Cortisol is central to several homeostatic mechanisms including the stress and immune response. Adrenal insufficiency and impaired cortisol production leads to severe, potentially fatal disorders. Several fundamental stages of steroidogenesis occur within the mitochondria. These dynamic organelles not only contribute ATP for steroidogenesis, but also detoxify harmful by-products generated during cortisol synthesis (reactive oxygen species). Mutations in nuclear or mitochondrial DNA that impair mitochondrial function lead to debilitating multi-system diseases. Recently, genetic variants that impair mitochondrial function have been identified in people with isolated cortisol insufficiency. This review aimed to clarify the association between mitochondrial diseases and adrenal insufficiency to produce cortisol. Mitochondrial diseases are rare and mitochondrial diseases that feature adrenal insufficiency are even rarer. We identified only 14 cases of adrenal insufficiency in people with confirmed mitochondrial diseases globally. In line with previous reviews, adrenal dysfunction was most prevalent in mitochondrial deletion syndromes (particularly Pearson syndrome and Kearns–Sayre syndrome) and with point mutations that compromised oxidative phosphorylation. Although adrenal insufficiency has been reported with mitochondrial diseases, the incidence reflects that expected in the general population. Thus, it is unlikely that mitochondrial mutations alone are responsible for an insufficiency to produce cortisol. More research is needed into the pathogenesis of adrenal disease in these individuals.

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Kondratyeva, E. V., and T. I. Vitkina. "Functional state of mitochondria in chronic respiratory diseases." Bulletin Physiology and Pathology of Respiration 1, no. 84 (July 9, 2022): 116–26. http://dx.doi.org/10.36604/1998-5029-2022-84-116-126.

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Introduction. Chronic respiratory diseases are one of the most common types of non-communicable diseases and are an important problem of our time. The induction of oxidative stress, chronic inflammation and hypoxia, which underlie the pathogenesis of chronic diseases of the bronchopulmonary system, can be determined at the cellular and molecular level by impaired mitochondrial functioning.Aim. This review is devoted to the prospects for assessing the functional state of mitochondria as a fine indicator of the course of chronic respiratory diseases.Results. The data of domestic and foreign sources on the most important parameters of mitochondrial functioning in normal and chronic bronchopulmonary pathology were analyzed. It has been shown that mitochondria are highly sensitive to changes in both exogenous and endogenous homeostasis. Functional parameters of mitochondria, the level of mitochondrial reactive oxygen species, mitochondrial membrane potential, and fatty acid composition of mitochondrial membranes can be used as diagnostic and prognostic criteria for chronic respiratory diseases. The data presented in the review indicate the need for further studies of the functional state of mitochondria in chronic bronchopulmonary pathology.

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Goto, Yu-ichi. "Mitochondrial diseases." Equilibrium Research 75, no. 1 (2016): 1–6. http://dx.doi.org/10.3757/jser.75.1.

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Loekken, N., S. Vinther Skriver, T. Khawajazada, J. Storgaard, and J. Vissing. "MITOCHONDRIAL DISEASES." Neuromuscular Disorders 31 (October 2021): S114. http://dx.doi.org/10.1016/j.nmd.2021.07.238.

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Ishii, A., S. Ouchi, R. Matsuoka, A. Tamaoka, and M. Noguchi. "MITOCHONDRIAL DISEASES." Neuromuscular Disorders 31 (October 2021): S115. http://dx.doi.org/10.1016/j.nmd.2021.07.242.

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Carrera, L., L. Cantarero, G. Nolasco, J. Pijuan, D. Natera, B. Estevez, J. Expósito, et al. "MITOCHONDRIAL DISEASES." Neuromuscular Disorders 31 (October 2021): S114. http://dx.doi.org/10.1016/j.nmd.2021.07.239.

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Lee, M., J. Lee, and H. Lee. "MITOCHONDRIAL DISEASES." Neuromuscular Disorders 31 (October 2021): S115—S116. http://dx.doi.org/10.1016/j.nmd.2021.07.243.

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Burns, A., M. Leffler, A. Sapp, A. Allred, E. Kelly, S. Doyle, T. Uz, and A. Karaa. "MITOCHONDRIAL DISEASES." Neuromuscular Disorders 31 (October 2021): S114—S115. http://dx.doi.org/10.1016/j.nmd.2021.07.240.

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Lee, H., and Y. Lee. "MITOCHONDRIAL DISEASES." Neuromuscular Disorders 31 (October 2021): S115. http://dx.doi.org/10.1016/j.nmd.2021.07.241.

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Kopishinskaia, S., P. Pchelin, M. Korotysh, T. Kovaleva, S. Svetozarskiy, S. Nikitin, and I. Mukhina. "MITOCHONDRIAL DISEASES." Neuromuscular Disorders 31 (October 2021): S113—S114. http://dx.doi.org/10.1016/j.nmd.2021.07.236.

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Domínguez-González, C., S. Laine-Menéndez, A. Delmiro, I. García-Consuegra, M. Fernández-de la Torre, A. Hernández-Laín, J. Sayas, C. de Fuenmayor, M. Martin, and M. Morán. "MITOCHONDRIAL DISEASES." Neuromuscular Disorders 31 (October 2021): S114. http://dx.doi.org/10.1016/j.nmd.2021.07.237.

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35

Vu, Tuan H., Michio Hirano, and Salvatore DiMauro. "Mitochondrial diseases." Neurologic Clinics 20, no. 3 (August 2002): 809–39. http://dx.doi.org/10.1016/s0733-8619(01)00017-2.

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Zeviani, Massimo, Eduardo Bonilla, Darryl C. DeVivo, and Salvatore DiMauro. "Mitochondrial Diseases." Neurologic Clinics 7, no. 1 (February 1989): 123–56. http://dx.doi.org/10.1016/s0733-8619(18)30832-6.

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Wegberg van, A., H. Zweers-van Essen, J. Smeitink, C. Saris, and M. Janssen. "MITOCHONDRIAL DISEASES." Neuromuscular Disorders 29 (October 2019): S56. http://dx.doi.org/10.1016/j.nmd.2019.06.081.

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Reiner, Gail, and Jan Panyard-Davis. "Mitochondrial diseases." Nursing 42, no. 6 (June 2012): 51–56. http://dx.doi.org/10.1097/01.nurse.0000413616.59485.cf.

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39

Morgan-Hughes, J. A. "Mitochondrial diseases." Trends in Neurosciences 9 (January 1986): 15–19. http://dx.doi.org/10.1016/0166-2236(86)90006-8.

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40

Schapira, Anthony HV. "Mitochondrial diseases." Lancet 379, no. 9828 (May 2012): 1825–34. http://dx.doi.org/10.1016/s0140-6736(11)61305-6.

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41

Graff, Caroline, The-Hung Bui, and Nils-Göran Larsson. "Mitochondrial diseases." Best Practice & Research Clinical Obstetrics & Gynaecology 16, no. 5 (October 2002): 715–28. http://dx.doi.org/10.1053/beog.2002.0315.

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DiMauro, Salvatore. "Mitochondrial diseases." Biochimica et Biophysica Acta (BBA) - Bioenergetics 1658, no. 1-2 (July 2004): 80–88. http://dx.doi.org/10.1016/j.bbabio.2004.03.014.

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43

Zheng, Yifan, Jing Zhang, Xiaohong Zhu, Yuanjuan Wei, Wuli Zhao, Shuyi Si, and Yan Li. "A Mitochondrial Perspective on Noncommunicable Diseases." Biomedicines 11, no. 3 (February 21, 2023): 647. http://dx.doi.org/10.3390/biomedicines11030647.

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Mitochondria are the center of energy metabolism in eukaryotic cells and play a central role in the metabolism of living organisms. Mitochondrial diseases characterized by defects in oxidative phosphorylation are the most common congenital diseases. Meanwhile, mitochondrial dysfunction caused by secondary factors such as non-inherited genetic mutations can affect normal physiological functions of human cells, induce apoptosis, and lead to the development of various diseases. This paper reviewed several major factors and mechanisms that contribute to mitochondrial dysfunction and discussed the development of diseases closely related to mitochondrial dysfunction and drug treatment strategies discovered in recent years.

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Ma, Xiaowen, Tara McKeen, Jianhua Zhang, and Wen-Xing Ding. "Role and Mechanisms of Mitophagy in Liver Diseases." Cells 9, no. 4 (March 31, 2020): 837. http://dx.doi.org/10.3390/cells9040837.

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The mitochondrion is an organelle that plays a vital role in the regulation of hepatic cellular redox, lipid metabolism, and cell death. Mitochondrial dysfunction is associated with both acute and chronic liver diseases with emerging evidence indicating that mitophagy, a selective form of autophagy for damaged/excessive mitochondria, plays a key role in the liver’s physiology and pathophysiology. This review will focus on mitochondrial dynamics, mitophagy regulation, and their roles in various liver diseases (alcoholic liver disease, non-alcoholic fatty liver disease, drug-induced liver injury, hepatic ischemia-reperfusion injury, viral hepatitis, and cancer) with the hope that a better understanding of the molecular events and signaling pathways in mitophagy regulation will help identify promising targets for the future treatment of liver diseases.

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Lee, Seo-Eun, Young Cheol Kang, Yujin Kim, Soomin Kim, Shin-Hye Yu, Jong Hyeok Park, In-Hyeon Kim, et al. "Preferred Migration of Mitochondria toward Cells and Tissues with Mitochondrial Damage." International Journal of Molecular Sciences 23, no. 24 (December 12, 2022): 15734. http://dx.doi.org/10.3390/ijms232415734.

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Mitochondria are organelles that play a vital role in cellular survival by supplying ATP and metabolic substrates via oxidative phosphorylation and the Krebs cycle. Hence, mitochondrial dysfunction contributes to many human diseases, including metabolic syndromes, neurodegenerative diseases, cancer, and aging. Mitochondrial transfer between cells has been shown to occur naturally, and mitochondrial transplantation is beneficial for treating mitochondrial dysfunction. In this study, the migration of mitochondria was tracked in vitro and in vivo using mitochondria conjugated with green fluorescent protein (MTGFP). When MTGFP were used in a coculture model, they were selectively internalized into lung fibroblasts, and this selectivity depended on the mitochondrial functional states of the receiving fibroblasts. Compared with MTGFP injected intravenously into normal mice, MTGFP injected into bleomycin-induced idiopathic pulmonary fibrosis model mice localized more abundantly in the lung tissue, indicating that mitochondrial homing to injured tissue occurred. This study shows for the first time that exogenous mitochondria are preferentially trafficked to cells and tissues in which mitochondria are damaged, which has implications for the delivery of therapeutic agents to injured or diseased sites.

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Матіюк, В. В. "Diseases caused by mitochondrial DNA mutations." Вісник Полтавської державної аграрної академії, no. 4 (December 30, 2022): 86–92. http://dx.doi.org/10.31210/visnyk2022.04.10.

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This article highlights data on diseases caused by mitochondrial DNA mutations. The purpose of the review was to reveal existing diseases that arise as a result of mtDNA mutations. Mitochondrial diseases are diseases that are most often caused by genetically determined structural and functional disorders of mitochondria, and as a result, the energy supply of cells is disrupted. All mitochondrial diseases are transmitted through the maternal line, so if mutations are detected in time, they can be blocked and the further inheritance will be stopped. It is suggested that the role of mitochondrial DNA in certain diseases began to develop rapidly in 1988 when the first mutations in mitochondrial DNA were discovered. To understand the course and development of mitochondrial DNA, it is necessary to understand the structure and functional properties of the mitochondrial cell. MtDNA is a circular DNA molecule and is localized in mitochondria. Such organelles can replicate, transcribe, and translate their own DNA independently of nuclear DNA. Mitochondrial DNA can mutate more than 10 times more often than nuclear DNA. MtDNA has no protective functions against the phenomena of mutations. A mitochondrial cell can contain both mutant DNA and normal DNA. In genetics, such a condition is called heteroplasmy, which allows the survival of a lethal mutation. Single deletions, large deletions, and multiple deletions that are transmitted autosomally and have different phenotypic manifestations are the primary cause of the development of mitochondrial diseases. Scientists also identify systemic manifestations of mitochondrial DNA mutations. they include endocrine manifestations (diabetes), neurological diseases, gastrointestinal manifestations (acid-alkaline imbalance), and pulmonary manifestations (myoclonic epilepsy, hypoventilation abnormalities). Several main principles of treatment of mitochondropathies are distinguished: following a diet; additional introduction of cofactors involved in enzymatic reactions of energy metabolism (thiamine, riboflavin, nicotinamide, lipoic acid, biotin, carnitine); prescription of drugs, capable of carrying out the function of transferring electrons in the respiratory chain (vitamins K1 and K3, ascorbic acid).

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Stanga, Serena, Anna Caretto, Marina Boido, and Alessandro Vercelli. "Mitochondrial Dysfunctions: A Red Thread across Neurodegenerative Diseases." International Journal of Molecular Sciences 21, no. 10 (May 25, 2020): 3719. http://dx.doi.org/10.3390/ijms21103719.

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Mitochondria play a central role in a plethora of processes related to the maintenance of cellular homeostasis and genomic integrity. They contribute to preserving the optimal functioning of cells and protecting them from potential DNA damage which could result in mutations and disease. However, perturbations of the system due to senescence or environmental factors induce alterations of the physiological balance and lead to the impairment of mitochondrial functions. After the description of the crucial roles of mitochondria for cell survival and activity, the core of this review focuses on the “mitochondrial switch” which occurs at the onset of neuronal degeneration. We dissect the pathways related to mitochondrial dysfunctions which are shared among the most frequent or disabling neurodegenerative diseases such as Alzheimer’s, Parkinson’s, and Huntington’s, Amyotrophic Lateral Sclerosis, and Spinal Muscular Atrophy. Can mitochondrial dysfunctions (affecting their morphology and activities) represent the early event eliciting the shift towards pathological neurobiological processes? Can mitochondria represent a common target against neurodegeneration? We also review here the drugs that target mitochondria in neurodegenerative diseases.

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Zhou, Wen-cheng, Jiao Qu, Sheng-yang Xie, Yang Sun, and Hong-wei Yao. "Mitochondrial Dysfunction in Chronic Respiratory Diseases: Implications for the Pathogenesis and Potential Therapeutics." Oxidative Medicine and Cellular Longevity 2021 (July 27, 2021): 1–20. http://dx.doi.org/10.1155/2021/5188306.

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Mitochondria are indispensable for energy metabolism and cell signaling. Mitochondrial homeostasis is sustained with stabilization of mitochondrial membrane potential, balance of mitochondrial calcium, integrity of mitochondrial DNA, and timely clearance of damaged mitochondria via mitophagy. Mitochondrial dysfunction is featured by increased generation of mitochondrial reactive oxygen species, reduced mitochondrial membrane potential, mitochondrial calcium imbalance, mitochondrial DNA damage, and abnormal mitophagy. Accumulating evidence indicates that mitochondrial dysregulation causes oxidative stress, inflammasome activation, apoptosis, senescence, and metabolic reprogramming. All these cellular processes participate in the pathogenesis and progression of chronic respiratory diseases, including chronic obstructive pulmonary disease, pulmonary fibrosis, and asthma. In this review, we provide a comprehensive and updated overview of the impact of mitochondrial dysfunction on cellular processes involved in the development of these respiratory diseases. This not only implicates mechanisms of mitochondrial dysfunction for the pathogenesis of chronic lung diseases but also provides potential therapeutic approaches for these diseases by targeting dysfunctional mitochondria.

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Hroudová, Jana, Namrata Singh, and Zdeněk Fišar. "Mitochondrial Dysfunctions in Neurodegenerative Diseases: Relevance to Alzheimer’s Disease." BioMed Research International 2014 (2014): 1–9. http://dx.doi.org/10.1155/2014/175062.

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Mitochondrial dysfunctions are supposed to be responsible for many neurodegenerative diseases dominating in Alzheimer’s disease (AD), Parkinson’s disease (PD), and Huntington’s disease (HD). A growing body of evidence suggests that defects in mitochondrial metabolism and particularly of electron transport chain may play a role in pathogenesis of AD. Structurally and functionally damaged mitochondria do not produce sufficient ATP and are more prominent in producing proapoptotic factors and reactive oxygen species (ROS), and this can be an early stage of several mitochondrial disorders, including neurodegenerative diseases. Mitochondrial dysfunctions may be caused by both mutations in mitochondrial or nuclear DNA that code mitochondrial components and by environmental causes. In the following review, common aspects of mitochondrial impairment concerned about neurodegenerative diseases are summarized including ROS production, impaired mitochondrial dynamics, and apoptosis. Also, damaged function of electron transport chain complexes and interactions between pathological proteins and mitochondria are described for AD particularly and marginally for PD and HD.

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Kiseljaković, Emina, Radivoj Jadrić, Sabaheta Hasić, Lorenka Ljuboja, Jovo Radovanović, Husein Kulenović, and Mira Winterhalter-Jadrić. "Mitochondrial medicine - a key to solve pathophysiology of 21 century diseases." Bosnian Journal of Basic Medical Sciences 2, no. 1-2 (February 20, 2008): 46–48. http://dx.doi.org/10.17305/bjbms.2002.3580.

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Over the past 13 years mitochondrial defects have been involved in wide variety of degenerative diseases - Parkinson disease, Alzheimer dementia, arteriosclerosis, ageing and cancer. Mitochondria are believed to control apoptosis or programmed cell death. Disturbance in mitochondrial metabolism has also been implicated in many common diseases such as congestive hart failure, diabetes and migraine. Scientific investigations have showed complexities in mitochondrial genetics, but at the same time, pathophysiology of mitochondrial diseases is still enigma. Mitochondria and their DNAs are opening the era of "mitochondrial medicine". What we today call "a mitochondrial medicine" is only a part of the whole panorama of diseases based on disordered mitochondrial function.

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Journal articles on the topic 'Mitochondrial diseases'

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