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Journal articles on the topic 'Mitochondrial diseases'

Author: Grafiati

Published: 4 June 2021

Last updated: 25 July 2025

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1

Tang, Xiaoqiang, Xiao-Feng Chen, Hou-Zao Chen, and De-Pei Liu. "Mitochondrial Sirtuins in cardiometabolic diseases." Clinical Science 131, no. 16 (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

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Hong, Seongho, Sanghun Kim, Kyoungmi Kim, and Hyunji Lee. "Clinical Approaches for Mitochondrial Diseases." Cells 12, no. 20 (2023): 2494. http://dx.doi.org/10.3390/cells12202494.

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Mitochondria are subcontractors dedicated to energy production within cells. In human mitochondria, almost all mitochondrial proteins originate from the nucleus, except for 13 subunit proteins that make up the crucial system required to perform ‘oxidative phosphorylation (OX PHOS)’, which are expressed by the mitochondria’s self-contained DNA. Mitochondrial DNA (mtDNA) also encodes 2 rRNA and 22 tRNA species. Mitochondrial DNA replicates almost autonomously, independent of the nucleus, and its heredity follows a non-Mendelian pattern, exclusively passing from mother to children. Numerous studi

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Fu, Ailing. "Mitotherapy as a Novel Therapeutic Strategy for Mitochondrial Diseases." Current Molecular Pharmacology 13, no. 1 (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 e

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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 (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 phe

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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 (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 mitochon

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Hu, Cuilan, Zheng Shi, Xiongxiong Liu, and Chao Sun. "The Research Progress of Mitochondrial Transplantation in the Treatment of Mitochondrial Defective Diseases." International Journal of Molecular Sciences 25, no. 2 (2024): 1175. http://dx.doi.org/10.3390/ijms25021175.

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Mitochondria are double-membrane organelles that are involved in energy production, apoptosis, and signaling in eukaryotic cells. Several studies conducted over the past decades have correlated mitochondrial dysfunction with various diseases, including cerebral ischemia, myocardial ischemia-reperfusion, and cancer. Mitochondrial transplantation entails importing intact mitochondria from healthy tissues into diseased tissues with damaged mitochondria to rescue the injured cells. In this review, the different mitochondrial transplantation techniques and their clinical applications have been disc

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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 (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 mu

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8

AkL, Ahmed, Iman Afsah, and Ramadan A Saad. "Mitochondrial diseases: past, present, and future." Urology & Nephrology Open Access Journal 10, no. 1 (2022): 9–13. http://dx.doi.org/10.15406/unoaj.2022.10.00316.

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Mitochondria are membrane-bound organelles found in almost all eukaryotic cells. Mitochondria are in charge of mastering and organizing cellular energy production in order to sustain life. Two genomic systems are responsible for mitochondrial biogenesis: nucli-genomes and another set of mitochondrial genes (mtDNA). MtDNA encodes 13 proteins that are required for respiratory chain function. Mitochondria play a role in a variety of cellular processes, including calcium signaling, cell cycle, differentiation, and cell death. Mitochondrial shape indicates good health, and their location in the cel

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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 (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

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Cilleros-Holgado, Paula, David Gómez-Fernández, Rocío Piñero-Pérez, et al. "Mitochondrial Quality Control via Mitochondrial Unfolded Protein Response (mtUPR) in Ageing and Neurodegenerative Diseases." Biomolecules 13, no. 12 (2023): 1789. http://dx.doi.org/10.3390/biom13121789.

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Mitochondria play a key role in cellular functions, including energy production and oxidative stress regulation. For this reason, maintaining mitochondrial homeostasis and proteostasis (homeostasis of the proteome) is essential for cellular health. Therefore, there are different mitochondrial quality control mechanisms, such as mitochondrial biogenesis, mitochondrial dynamics, mitochondrial-derived vesicles (MDVs), mitophagy, or mitochondrial unfolded protein response (mtUPR). The last item is a stress response that occurs when stress is present within mitochondria and, especially, when the ac

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11

Yang, Han-Mo. "Mitochondrial Dysfunction in Cardiovascular Diseases." International Journal of Molecular Sciences 26, no. 5 (2025): 1917. https://doi.org/10.3390/ijms26051917.

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Mitochondrial dysfunction is increasingly recognized as a central contributor to the pathogenesis of cardiovascular diseases (CVDs), including heart failure, ischemic heart disease, hypertension, and cardiomyopathy. Mitochondria, known as the powerhouses of the cell, play a vital role in maintaining cardiac energy homeostasis, regulating reactive oxygen species (ROS) production and controlling cell death pathways. Dysregulated mitochondrial function results in impaired adenosine triphosphate (ATP) production, excessive ROS generation, and activation of apoptotic and necrotic pathways, collecti

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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 (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 machine

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Nagel, Stefan. "Is Mitochondrial Dysfunction the Underlying Causal Reason for Human Diseases?" Science Insights 46, no. 6 (2025): 1843–46. https://doi.org/10.15354/si.25.op301.

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Mitochondria, the cellular powerhouses, have emerged as pivotal players in human health and disease. Recent advances suggest mitochondrial dysfunction is implicated in a broad spectrum of disorders, ranging from neurodegenerative diseases, metabolic syndromes, cardiovascular conditions, to aging-related decline. This article explores the hypothesis that mitochondrial dysfunction may be a central underlying cause driving many, if not all, human diseases. By integrating evidence from molecular biology, clinical studies, and evolutionary theory, I argue that mitochondrial health is fundamental to

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Ngo, Jennifer, Corey Osto, Frankie Villalobos, and Orian S. Shirihai. "Mitochondrial Heterogeneity in Metabolic Diseases." Biology 10, no. 9 (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

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15

Snyder, Ryan J., and Steven R. Kleeberger. "Role of Mitochondrial DNA in Inflammatory Airway Diseases." Comprehensive Physiology 11, no. 2 (2021): 1485–99. https://doi.org/10.1002/j.2040-4603.2021.tb00159.x.

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AbstractThe mitochondrial genome is a small, circular, and highly conserved piece of DNA which encodes only 13 protein subunits yet is vital for electron transport in the mitochondrion and, therefore, vital for the existence of multicellular life on Earth. Despite this importance, mitochondrial DNA (mtDNA) is located in one of the least‐protected areas of the cell, exposing it to high concentrations of intracellular reactive oxygen species (ROS) and threat from exogenous substances and pathogens. Until recently, the quality control mechanisms which ensured the stability of the nuclear genome w

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16

Lin, Tsu-Kung, Shang-Der Chen, Yao-Chung Chuang, 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

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Caffarra Malvezzi, Cristina, Aderville Cabassi, and Michele Miragoli. "Mitochondrial mechanosensor in cardiovascular diseases." Vascular Biology 2, no. 1 (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

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18

Huang, Yafei, and Bingying Zhou. "Mitochondrial Dysfunction in Cardiac Diseases and Therapeutic Strategies." Biomedicines 11, no. 5 (2023): 1500. http://dx.doi.org/10.3390/biomedicines11051500.

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Mitochondria are the main site of intracellular synthesis of ATP, which provides energy for various physiological activities of the cell. Cardiomyocytes have a high density of mitochondria and mitochondrial damage is present in a variety of cardiovascular diseases. In this paper, we describe mitochondrial damage in mitochondrial cardiomyopathy, congenital heart disease, coronary heart disease, myocardial ischemia–reperfusion injury, heart failure, and drug-induced cardiotoxicity, in the context of the key roles of mitochondria in cardiac development and homeostasis. Finally, we discuss the mai

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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 (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

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20

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 (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, ar

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21

Yashooa, Raya Kh, Elisa Duranti, Donatella Conconi, Marialuisa Lavitrano, Suhad A. Mustafa, and Chiara Villa. "Mitochondrial microRNAs: Key Drivers in Unraveling Neurodegenerative Diseases." International Journal of Molecular Sciences 26, no. 2 (2025): 626. https://doi.org/10.3390/ijms26020626.

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MicroRNAs (miRNAs) are a class of small non-coding RNAs (ncRNAs) crucial for regulating gene expression at the post-transcriptional level. Recent evidence has shown that miRNAs are also found in mitochondria, organelles that produce energy in the cell. These mitochondrial miRNAs, also known as mitomiRs, are essential for regulating mitochondrial function and metabolism. MitomiRs can originate from the nucleus, following traditional miRNA biogenesis pathways, or potentially from mitochondrial DNA, allowing them to directly affect gene expression and cellular energy dynamics within the mitochond

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22

Tokuyama, Takeshi, and Shigeru Yanagi. "Role of Mitochondrial Dynamics in Heart Diseases." Genes 14, no. 10 (2023): 1876. http://dx.doi.org/10.3390/genes14101876.

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Mitochondrial dynamics, including fission and fusion processes, are essential for heart health. Mitochondria, the powerhouses of cells, maintain their integrity through continuous cycles of biogenesis, fission, fusion, and degradation. Mitochondria are relatively immobile in the adult heart, but their morphological changes due to mitochondrial morphology factors are critical for cellular functions such as energy production, organelle integrity, and stress response. Mitochondrial fusion proteins, particularly Mfn1/2 and Opa1, play multiple roles beyond their pro-fusion effects, such as endoplas

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Mu, Jian-Kang, Yan-Qin Li, Ting-Ting Shi, et al. "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. Mo

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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 (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 mi

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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 (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 transf

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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 (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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Shibata, Tatsuya, Toshinari Takahashi, Eio Yamada, et al. "T-2307 Causes Collapse of Mitochondrial Membrane Potential in Yeast." Antimicrobial Agents and Chemotherapy 56, no. 11 (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

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Park, Anna, Mihee Oh, Su Jeong Lee, et al. "Mitochondrial Transplantation as a Novel Therapeutic Strategy for Mitochondrial Diseases." International Journal of Molecular Sciences 22, no. 9 (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 yea

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Lee, Seo-Eun, Young Cheol Kang, Yujin Kim, et al. "Preferred Migration of Mitochondria toward Cells and Tissues with Mitochondrial Damage." International Journal of Molecular Sciences 23, no. 24 (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 fluoresce

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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 oxida

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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 (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 pu

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Huang, Michael L. H., Shannon Chiang, Danuta S. Kalinowski, Dong-Hun Bae, Sumit Sahni, and Des R. Richardson. "The Role of the Antioxidant Response in Mitochondrial Dysfunction in Degenerative Diseases: Cross-Talk between Antioxidant Defense, Autophagy, and Apoptosis." Oxidative Medicine and Cellular Longevity 2019 (April 7, 2019): 1–26. http://dx.doi.org/10.1155/2019/6392763.

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The mitochondrion is an essential organelle important for the generation of ATP for cellular function. This is especially critical for cells with high energy demands, such as neurons for signal transmission and cardiomyocytes for the continuous mechanical work of the heart. However, deleterious reactive oxygen species are generated as a result of mitochondrial electron transport, requiring a rigorous activation of antioxidative defense in order to maintain homeostatic mitochondrial function. Indeed, recent studies have demonstrated that the dysregulation of antioxidant response leads to mitoch

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Kiseljaković, Emina, Radivoj Jadrić, Sabaheta Hasić, et al. "Mitochondrial medicine - a key to solve pathophysiology of 21 century diseases." Bosnian Journal of Basic Medical Sciences 2, no. 1-2 (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

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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 (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

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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 (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 sour

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Lee, Yun Haeng, Myeong Uk Kuk, Moon Kyoung So, et al. "Targeting Mitochondrial Oxidative Stress as a Strategy to Treat Aging and Age-Related Diseases." Antioxidants 12, no. 4 (2023): 934. http://dx.doi.org/10.3390/antiox12040934.

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Mitochondria are one of the organelles undergoing rapid alteration during the senescence process. Senescent cells show an increase in mitochondrial size, which is attributed to the accumulation of defective mitochondria, which causes mitochondrial oxidative stress. Defective mitochondria are also targets of mitochondrial oxidative stress, and the vicious cycle between defective mitochondria and mitochondrial oxidative stress contributes to the onset and development of aging and age-related diseases. Based on the findings, strategies to reduce mitochondrial oxidative stress have been suggested

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Liesa, Marc, Manuel Palacín, and Antonio Zorzano. "Mitochondrial Dynamics in Mammalian Health and Disease." Physiological Reviews 89, no. 3 (2009): 799–845. http://dx.doi.org/10.1152/physrev.00030.2008.

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The meaning of the word mitochondrion (from the Greek mitos, meaning thread, and chondros, grain) illustrates that the heterogeneity of mitochondrial morphology has been known since the first descriptions of this organelle. Such a heterogeneous morphology is explained by the dynamic nature of mitochondria. Mitochondrial dynamics is a concept that includes the movement of mitochondria along the cytoskeleton, the regulation of mitochondrial architecture (morphology and distribution), and connectivity mediated by tethering and fusion/fission events. The relevance of these events in mitochondrial

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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 (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

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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 (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 comp

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Corkery-Hayward, Madeleine, and Louise A. Metherell. "Adrenal Dysfunction in Mitochondrial Diseases." International Journal of Molecular Sciences 24, no. 2 (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 im

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Portz, Philipp, та Michael K. Lee. "Changes in Drp1 Function and Mitochondrial Morphology Are Associated with the α-Synuclein Pathology in a Transgenic Mouse Model of Parkinson’s Disease". Cells 10, № 4 (2021): 885. http://dx.doi.org/10.3390/cells10040885.

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Alterations in mitochondrial function and morphology are associated with many human diseases, including cancer and neurodegenerative diseases. Mitochondrial impairment is linked to Parkinson’s disease (PD) pathogenesis, and alterations in mitochondrial dynamics are seen in PD models. In particular, α-synuclein (αS) abnormalities are often associated with pathological changes to mitochondria. However, the relationship between αS pathology and mitochondrial dynamics remains poorly defined. Herein, we examined a mouse model of α-synucleinopathy for αS pathology-linked alterations in mitochondrial

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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 (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

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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, includin

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Gumeniuk, O. I., I. A. Glushakov, Yu V. Chernenkov, I. A. Bochkarev, G. V. Gafurova, and E. E. Sukhushina. "Digestive system and mitochondrial diseases in children." Experimental and Clinical Gastroenterology, no. 6 (October 22, 2024): 43–46. http://dx.doi.org/10.31146/1682-8658-ecg-226-6-43-46.

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The article discusses the features of lesions of the digestive system in children with mitochondrial diseases. Mitochondria play an important role in cellular metabolism as they are responsible for producing the majority of cellular energy in the form of adenosine triphosphate. Mutations in mitochondrial genes responsible for the functioning of mitochondria can lead to various forms of mitochondrial diseases. These diseases may present with the following clinical symptoms: muscle weakness, movement disorders, neurological symptoms, impaired motility and absorption from the gastrointestinal tra

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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 m

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Choi, Eui-Hwan, Mi-Hye Kim, and Sun-Ji Park. "Targeting Mitochondrial Dysfunction and Reactive Oxygen Species for Neurodegenerative Disease Treatment." International Journal of Molecular Sciences 25, no. 14 (2024): 7952. http://dx.doi.org/10.3390/ijms25147952.

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Alzheimer’s disease (AD) and Parkinson’s disease (PD) are the most common neurodegenerative diseases, and they affect millions of people worldwide, particularly older individuals. Therefore, there is a clear need to develop novel drug targets for the treatment of age-related neurodegenerative diseases. Emerging evidence suggests that mitochondrial dysfunction and reactive oxygen species (ROS) generation play central roles in the onset and progression of neurodegenerative diseases. Mitochondria are key regulators of respiratory function, cellular energy adenosine triphosphate production, and th

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Zerihun, Mulate, Surya Sukumaran, and Nir Qvit. "The Drp1-Mediated Mitochondrial Fission Protein Interactome as an Emerging Core Player in Mitochondrial Dynamics and Cardiovascular Disease Therapy." International Journal of Molecular Sciences 24, no. 6 (2023): 5785. http://dx.doi.org/10.3390/ijms24065785.

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Mitochondria, the membrane-bound cell organelles that supply most of the energy needed for cell function, are highly regulated, dynamic organelles bearing the ability to alter both form and functionality rapidly to maintain normal physiological events and challenge stress to the cell. This amazingly vibrant movement and distribution of mitochondria within cells is controlled by the highly coordinated interplay between mitochondrial dynamic processes and fission and fusion events, as well as mitochondrial quality-control processes, mainly mitochondrial autophagy (also known as mitophagy). Fusio

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Liu, Ruibo. "The Impact of Mitochondria on Neurodegenerative Diseases." Theoretical and Natural Science 90, no. 1 (2025): 61–67. https://doi.org/10.54254/2753-8818/2025.gu20452.

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Neurodegenerative diseases (NDDs) include Alzheimer's disease (AD), Parkinson's disease (PD) and Huntington's disease (HD), which are defined by the progressive deterioration of neurons in the central nervous system and functional decline. The pathogenesis of these diseases is complex and there is currently no effective treatment. For example, the failure rate of clinical trials for AD treatment strategies is as high as 99.5%. Research indicates that elements such mitochondrial malfunction, oxidative stress, excitotoxicity, inflammation, and apoptosis are closely related to the onset of NDDs,

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Duan, Geyan, Jianjun Li, Yehui Duan, et al. "Mitochondrial Iron Metabolism: The Crucial Actors in Diseases." Molecules 28, no. 1 (2022): 29. http://dx.doi.org/10.3390/molecules28010029.

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Iron is a trace element necessary for cell growth, development, and cellular homeostasis, but insufficient or excessive level of iron is toxic. Intracellularly, sufficient amounts of iron are required for mitochondria (the center of iron utilization) to maintain their normal physiologic function. Iron deficiency impairs mitochondrial metabolism and respiratory activity, while mitochondrial iron overload promotes ROS production during mitochondrial electron transport, thus promoting potential disease development. This review provides an overview of iron homeostasis, mitochondrial iron metabolis

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Yang, Xuan, Ruoyu Zhang, Kiichi Nakahira, and Zhenglong Gu. "Mitochondrial DNA Mutation, Diseases, and Nutrient-Regulated Mitophagy." Annual Review of Nutrition 39, no. 1 (2019): 201–26. http://dx.doi.org/10.1146/annurev-nutr-082018-124643.

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A wide spectrum of human diseases, including cancer, neurodegenerative diseases, and metabolic disorders, have been shown to be associated with mitochondrial dysfunction through multiple molecular mechanisms. Mitochondria are particularly susceptible to nutrient deficiencies, and nutritional intervention is an essential way to maintain mitochondrial homeostasis. Recent advances in genetic manipulation and next-generation sequencing reveal the crucial roles of mitochondrial DNA (mtDNA) in various pathophysiological conditions. Mitophagy, a term coined to describe autophagy that targets dysfunct

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