Academic literature on the topic 'Mitochondria, mitochondrial diseases, Opa1, therapies'
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Journal articles on the topic "Mitochondria, mitochondrial diseases, Opa1, therapies"
1
Xia, Yi, Xu Zhang, Peng An, Junjie Luo, and Yongting Luo. "Mitochondrial Homeostasis in VSMCs as a Central Hub in Vascular Remodeling." International Journal of Molecular Sciences 24, no. 4 (February 9, 2023): 3483. http://dx.doi.org/10.3390/ijms24043483.
Full textAbstract:
Vascular remodeling is a common pathological hallmark of many cardiovascular diseases. Vascular smooth muscle cells (VSMCs) are the predominant cell type lining the tunica media and play a crucial role in maintaining aortic morphology, integrity, contraction and elasticity. Their abnormal proliferation, migration, apoptosis and other activities are tightly associated with a spectrum of structural and functional alterations in blood vessels. Emerging evidence suggests that mitochondria, the energy center of VSMCs, participate in vascular remodeling through multiple mechanisms. For example, peroxisome proliferator-activated receptor-γ coactivator-1α (PGC-1α)-mediated mitochondrial biogenesis prevents VSMCs from proliferation and senescence. The imbalance between mitochondrial fusion and fission controls the abnormal proliferation, migration and phenotypic transformation of VSMCs. Guanosine triphosphate-hydrolyzing enzymes, including mitofusin 1 (MFN1), mitofusin 2 (MFN2), optic atrophy protein 1 (OPA1) and dynamin-related protein 1 (DRP1), are crucial for mitochondrial fusion and fission. In addition, abnormal mitophagy accelerates the senescence and apoptosis of VSMCs. PINK/Parkin and NIX/BINP3 pathways alleviate vascular remodeling by awakening mitophagy in VSMCs. Mitochondrial DNA (mtDNA) damage destroys the respiratory chain of VSMCs, resulting in excessive ROS production and decreased ATP levels, which are related to the proliferation, migration and apoptosis of VSMCs. Thus, maintaining mitochondrial homeostasis in VSMCs is a possible way to relieve pathologic vascular remodeling. This review aims to provide an overview of the role of mitochondria homeostasis in VSMCs during vascular remodeling and potential mitochondria-targeted therapies.
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Samant, Sadhana A., Hannah J. Zhang, Zhigang Hong, Vinodkumar B. Pillai, Nagalingam R. Sundaresan, Donald Wolfgeher, Stephen L. Archer, David C. Chan, and Mahesh P. Gupta. "SIRT3 Deacetylates and Activates OPA1 To Regulate Mitochondrial Dynamics during Stress." Molecular and Cellular Biology 34, no. 5 (December 16, 2013): 807–19. http://dx.doi.org/10.1128/mcb.01483-13.
Full textAbstract:
Mitochondrial morphology is regulated by the balance between two counteracting mitochondrial processes of fusion and fission. There is significant evidence suggesting a stringent association between morphology and bioenergetics of mitochondria. Morphological alterations in mitochondria are linked to several pathological disorders, including cardiovascular diseases. The consequences of stress-induced acetylation of mitochondrial proteins on the organelle morphology remain largely unexplored. Here we report that OPA1, a mitochondrial fusion protein, was hyperacetylated in hearts under pathological stress and this posttranslational modification reduced the GTPase activity of the protein. The mitochondrial deacetylase SIRT3 was capable of deacetylating OPA1 and elevating its GTPase activity. Mass spectrometry and mutagenesis analyses indicated that in SIRT3-deficient cells OPA1 was acetylated at lysine 926 and 931 residues. Overexpression of a deacetylation-mimetic version of OPA1 recovered the mitochondrial functions of OPA1-null cells, thus demonstrating the functional significance of K926/931 acetylation in regulating OPA1 activity. Moreover, SIRT3-dependent activation of OPA1 contributed to the preservation of mitochondrial networking and protection of cardiomyocytes from doxorubicin-mediated cell death. In summary, these data indicated that SIRT3 promotes mitochondrial function not only by regulating activity of metabolic enzymes, as previously reported, but also by regulating mitochondrial dynamics by targeting OPA1.
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Ranieri, Michela, Simona Brajkovic, Giulietta Riboldi, Dario Ronchi, Federica Rizzo, Nereo Bresolin, Stefania Corti, and Giacomo P. Comi. "Mitochondrial Fusion Proteins and Human Diseases." Neurology Research International 2013 (2013): 1–11. http://dx.doi.org/10.1155/2013/293893.
Full textAbstract:
Mitochondria are highly dynamic, complex organelles that continuously alter their shape, ranging between two opposite processes, fission and fusion, in response to several stimuli and the metabolic demands of the cell. Alterations in mitochondrial dynamics due to mutations in proteins involved in the fusion-fission machinery represent an important pathogenic mechanism of human diseases. The most relevant proteins involved in the mitochondrial fusion process are three GTPase dynamin-like proteins: mitofusin 1 (MFN1) and 2 (MFN2), located in the outer mitochondrial membrane, and optic atrophy protein 1 (OPA1), in the inner membrane. An expanding number of degenerative disorders are associated with mutations in the genes encoding MFN2 and OPA1, including Charcot-Marie-Tooth disease type 2A and autosomal dominant optic atrophy. While these disorders can still be considered rare, defective mitochondrial dynamics seem to play a significant role in the molecular and cellular pathogenesis of more common neurodegenerative diseases, for example, Alzheimer’s and Parkinson’s diseases. This review provides an overview of the basic molecular mechanisms involved in mitochondrial fusion and focuses on the alteration in mitochondrial DNA amount resulting from impairment of mitochondrial dynamics. We also review the literature describing the main disorders associated with the disruption of mitochondrial fusion.
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Wu, Bangwei, Jian Li, Huanchun Ni, Xinyu Zhuang, Zhiyong Qi, Qiying Chen, Zhichao Wen, Haiming Shi, Xinping Luo, and Bo Jin. "TLR4 Activation Promotes the Progression of Experimental Autoimmune Myocarditis to Dilated Cardiomyopathy by Inducing Mitochondrial Dynamic Imbalance." Oxidative Medicine and Cellular Longevity 2018 (June 26, 2018): 1–15. http://dx.doi.org/10.1155/2018/3181278.
Full textAbstract:
Mitochondrial dynamic imbalance associates with several cardiovascular diseases. However, the role of mitochondrial dynamics in TLR4 activation-mediated dilated cardiomyopathy (DCM) progress remains unknown. A model of experimental autoimmune myocarditis (EAM) was established in BALB/c mice on which TLR4 activation by LPS-EB or TLR4 inhibition by LPS-RS was performed to induce chronic inflammation for 5 weeks. TLR4 activation promoted the transition of EAM to DCM as demonstrated by increased cardiomyocyte apoptosis, myocardial fibrosis, ventricular dilatation, and declined heart function. TLR4 inhibition mitigated the above DCM changes. Transmission electron microscope study showed that mitochondria became fragmented, also with damaged crista in ultrastructure in EAM mice. TLR4 activation aggravated the above mitochondrial aberration, and TLR4 inhibition alleviated it. The mitochondrial dynamic imbalance and damage in DCM development were mainly associated with OPA1 downregulation, which may be caused by elevated TNF-α level and ROS stress after TLR4 activation. Furthermore, OMA1/YME1L abnormal degradation was involved in the OPA1 dysfunction, and intervening OMA1/YME1L in H9C2 significantly alleviated mitochondrial fission, ultrastructure damage, and cell apoptosis induced by TNF-α and ROS. These data indicate that TLR4 activation resulted in OPA1 dysfunction, promoting mitochondrial dynamic imbalance and damage, which may involve in the progress of EAM to DCM.
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Markin, Alexander M., Viktoria A. Khotina, Xenia G. Zabudskaya, Anastasia I. Bogatyreva, Antonina V. Starodubova, Ekaterina Ivanova, Nikita G. Nikiforov, and Alexander N. Orekhov. "Disturbance of Mitochondrial Dynamics and Mitochondrial Therapies in Atherosclerosis." Life 11, no. 2 (February 20, 2021): 165. http://dx.doi.org/10.3390/life11020165.
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Mitochondrial dysfunction is associated with a wide range of chronic human disorders, including atherosclerosis and diabetes mellitus. Mitochondria are dynamic organelles that undergo constant turnover in living cells. Through the processes of mitochondrial fission and fusion, a functional population of mitochondria is maintained, that responds to the energy needs of the cell. Damaged or excessive mitochondria are degraded by mitophagy, a specialized type of autophagy. These processes are orchestrated by a number of proteins and genes, and are tightly regulated. When one or several of these processes are affected, it can lead to the accumulation of dysfunctional mitochondria, deficient energy production, increased oxidative stress and cell death—features that are described in many human disorders. While severe mitochondrial dysfunction is known to cause specific and mitochondrial disorders in humans, progressing damage of the mitochondria is also observed in a wide range of other chronic diseases, including cancer and atherosclerosis, and appears to play an important role in disease development. Therefore, correction of mitochondrial dynamics can help in developing new therapies for the treatment of these conditions. In this review, we summarize the recent knowledge on the processes of mitochondrial turnover and the proteins and genes involved in it. We provide a list of known mutations that affect mitochondrial function, and discuss the emerging therapeutic approaches.
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Carelli, Valerio, Chiara La Morgia, Luisa Iommarini, Rosanna Carroccia, Marina Mattiazzi, Simonetta Sangiorgi, Sabrina Farne', et al. "Mitochondrial Optic Neuropathies: How Two Genomes may Kill the Same Cell Type?" Bioscience Reports 27, no. 1-3 (June 13, 2007): 173–84. http://dx.doi.org/10.1007/s10540-007-9045-0.
Full textAbstract:
Ocular involvement is a prevalent feature in mitochondrial diseases. Leber's hereditary optic neuropathy (LHON) and dominant optic atrophy (DOA) are both non-syndromic optic neuropathies with a mitochondrial etiology. LHON is associated with point mutations in the mitochondrial DNA (mtDNA), which affect subunit genes of complex I. The majority of DOA patients harbor mutations in the nuclear-encoded protein OPA1, which is targeted to mitochondria and participates to cristae organization and mitochondrial network dynamics. In both disorders the retinal ganglion cells (RGCs) are specific cellular targets of the degenerative process. We here review the clinical features and the genetic bases, and delineate the possible common pathomechanism for both these disorders.
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Liesa, Marc, Manuel Palacín, and Antonio Zorzano. "Mitochondrial Dynamics in Mammalian Health and Disease." Physiological Reviews 89, no. 3 (July 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 and cell physiology has been partially unraveled after the identification of the genes responsible for mitochondrial fusion and fission. Furthermore, during the last decade, it has been identified that mutations in two mitochondrial fusion genes ( MFN2 and OPA1) cause prevalent neurodegenerative diseases (Charcot-Marie Tooth type 2A and Kjer disease/autosomal dominant optic atrophy). In addition, other diseases such as type 2 diabetes or vascular proliferative disorders show impaired MFN2 expression. Altogether, these findings have established mitochondrial dynamics as a consolidated area in cellular physiology. Here we review the most significant findings in the field of mitochondrial dynamics in mammalian cells and their implication in human pathologies.
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Zhong, Gang, Jagadeesh K. Venkatesan, Henning Madry, and Magali Cucchiarini. "Advances in Human Mitochondria-Based Therapies." International Journal of Molecular Sciences 24, no. 1 (December 29, 2022): 608. http://dx.doi.org/10.3390/ijms24010608.
Full textAbstract:
Mitochondria are the key biological generators of eukaryotic cells, controlling the energy supply while providing many important biosynthetic intermediates. Mitochondria act as a dynamic, functionally and structurally interconnected network hub closely integrated with other cellular compartments via biomembrane systems, transmitting biological information by shuttling between cells and tissues. Defects and dysregulation of mitochondrial functions are critically involved in pathological mechanisms contributing to aging, cancer, inflammation, neurodegenerative diseases, and other severe human diseases. Mediating and rejuvenating the mitochondria may therefore be of significant benefit to prevent, reverse, and even treat such pathological conditions in patients. The goal of this review is to present the most advanced strategies using mitochondria to manage such disorders and to further explore innovative approaches in the field of human mitochondria-based therapies.
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Yang, Yanyan, Min Li, Yan Liu, Zhibin Wang, Xiuxiu Fu, Xingqiang He, Qi Wang, et al. "The lncRNA Punisher Regulates Apoptosis and Mitochondrial Homeostasis of Vascular Smooth Muscle Cells via Targeting miR-664a-5p and OPA1." Oxidative Medicine and Cellular Longevity 2022 (May 25, 2022): 1–21. http://dx.doi.org/10.1155/2022/5477024.
Full textAbstract:
Long noncoding RNAs (lncRNAs) are important regulators of various cellular functions. Recent studies have shown that a novel lncRNA termed Punisher is highly expressed in cardiovascular progenitors and has potential role in cardiovascular diseases. However, its role, especially in molecular mechanism, is unclear. In our present study, we observed that Punisher was obviously downregulated in atherosclerotic plaques. Further research proved that it can suppress the apoptosis of VSMCs potentially contributing to the progression of atherosclerosis. Intriguingly, Punisher revealed to regulate mitochondria fission as well as mitochondrial functions induced by hydrogen peroxide (H2O2) in VSMCs. Mechanistically, Punisher was further proved to serve as a ceRNA which directly binds to miR-664a-5p and consequently regulates its target OPA1, and finally contributes to the biological function of VSMCs. Particularly, Punisher overexpression distinctly suppressed neointima formation and VSMC apoptosis in vivo. Encouragingly, these results were in accordance with findings obtained with the clinical evaluation of patients with atherosclerosis. Our data provides the significant relationship among OPA1, mitochondrial homeostasis, VSMC apoptosis, and atherosclerosis. And lncRNA Punisher and miR-664a-5p could serve as the novel and potential targets in the diagnosis and treatment of cardiovascular diseases.
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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.
Full textAbstract:
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.
Dissertations / Theses on the topic "Mitochondria, mitochondrial diseases, Opa1, therapies"
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CIVILETTO, GABRIELE. "Opa1 overexpression as potential therapy in mitochondrial diseases." Doctoral thesis, Università degli Studi di Milano-Bicocca, 2014. http://hdl.handle.net/10281/55460.
Full textAbstract:
Mitochondrial disorders are a group of highly invalidating human conditions due to defective oxidative phosphorylation, for which no effective treatment is nowadays available. In order to develop effective therapies for these disorders, I focused on an experimental approach based on the manipulation of mitochondrial morphology.
Opa1 is a GTPase of the inner mitochondrial membrane involved in both mitochondrial fusion and cristae shaping. The role of OPA1 in mitodynamics has also a documented impact on controlling the assembly of the respiratory supercomplexes and respiratory proficiency. Based on these considerations, I tested whether Opa1 overexpression could mitigate the effects of a severe mitochondrial respiratory chain deficit in vivo. In this thesis, the effects of mild transgenic overexpression of Opa1 on two mouse models of defective mitochondrial bioenergetics, a constitutive knockout for Ndufs4 (Ndufs4-/-), encoding a structural component of complex I, and a muscle-specific conditional knockout for Cox15, (Cox15sm/sm), encoding a heme-a biosynthetic enzyme involved in complex IV hemylation and assembly are shown. Crossing of both models with an Opa1 transgenic mouse line (Opa1tg) was associated with clinical and biochemical improvement, but whilst the effect was limited in Ndufs4-/-::Opa1tg mice, the Cox15sm/sm::Opa1tg mice showed relevant amelioration of motor performance, remarkable prolongation of survival, and significant correction of mitochondrial respiration. This effect was associated with the increased amount of active cIV holocomplex and cIV-containing supercomplexes.
Book chapters on the topic "Mitochondria, mitochondrial diseases, Opa1, therapies"
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Malhotra, Ashim, and Shivani Soni. "Examining the Effect of Mitochondrial Fission and Fusion Events on the Heart." In Emerging Applications, Perspectives, and Discoveries in Cardiovascular Research, 73–92. IGI Global, 2017. http://dx.doi.org/10.4018/978-1-5225-2092-4.ch005.
Full textAbstract:
Mitochondria constitute an integral structural and functional part of the cardiac muscle. The heart muscle relies on the mitochondrial production of fatty acids and ATP as sources of energy during different stages of human growth and development. New mitochondria are created from existing ones by a process called mitochondrial biogenesis which involves both fusion and fission events controlled by a bevy of proteins such as Drp1, OPA1, Mfn1, and Mfn2. In this chapter, we examine the role of these mitochondrial fission and fusion proteins in regulating various heart diseases, particularly, reperfusion injury, dilated cardiomyopathy, and heart failure. It is our intent to examine whether any of these proteins may serve as future candidates for cardiovascular therapy.