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Journal articles on the topic 'Mitochondria, mitochondrial diseases, Opa1, therapies'

Author: Grafiati

Published: 9 March 2023

Last updated: 28 July 2025

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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 (2023): 3483. http://dx.doi.org/10.3390/ijms24043483.

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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, pero

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2

Samant, Sadhana A., Hannah J. Zhang, Zhigang Hong, et al. "SIRT3 Deacetylates and Activates OPA1 To Regulate Mitochondrial Dynamics during Stress." Molecular and Cellular Biology 34, no. 5 (2013): 807–19. http://dx.doi.org/10.1128/mcb.01483-13.

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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 pathologic

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Ranieri, Michela, Simona Brajkovic, Giulietta Riboldi, et al. "Mitochondrial Fusion Proteins and Human Diseases." Neurology Research International 2013 (2013): 1–11. http://dx.doi.org/10.1155/2013/293893.

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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 pr

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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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Toda, Yuki, Sang-Bing Ong, Toshiyuki Yano, et al. "Downregulation of Mitochondrial Fusion Protein Expression Affords Protection from Canonical Necroptosis in H9c2 Cardiomyoblasts." International Journal of Molecular Sciences 25, no. 5 (2024): 2905. http://dx.doi.org/10.3390/ijms25052905.

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Necroptosis, a form of necrosis, and alterations in mitochondrial dynamics, a coordinated process of mitochondrial fission and fusion, have been implicated in the pathogenesis of cardiovascular diseases. This study aimed to determine the role of mitochondrial morphology in canonical necroptosis induced by a combination of TNFα and zVAD (TNF/zVAD) in H9c2 cells, rat cardiomyoblasts. Time-course analyses of mitochondrial morphology showed that mitochondria were initially shortened after the addition of TNF/zVAD and then their length was restored, and the proportion of cells with elongated mitoch

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Wu, Bangwei, Jian Li, Huanchun Ni, et al. "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.

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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

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Schuettpelz, Jana, Alexandre Janer, Hana Antonicka, and Eric A. Shoubridge. "The role of the mitochondrial outer membrane protein SLC25A46 in mitochondrial fission and fusion." Life Science Alliance 6, no. 6 (2023): e202301914. http://dx.doi.org/10.26508/lsa.202301914.

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Mutations inSLC25A46underlie a wide spectrum of neurodegenerative diseases associated with alterations in mitochondrial morphology. We established an SLC25A46 knock-out cell line in human fibroblasts and studied the pathogenicity of three variants (p.T142I, p.R257Q, and p.E335D). Mitochondria were fragmented in the knock-out cell line and hyperfused in all pathogenic variants. The loss of SLC25A46 led to abnormalities in the mitochondrial cristae ultrastructure that were not rescued by the expression of the variants. SLC25A46 was present in discrete puncta at mitochondrial branch points and ti

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Suárez-Rivero, Juan M., Juan López-Pérez, Inés Muela-Zarzuela, et al. "Neurodegeneration, Mitochondria, and Antibiotics." Metabolites 13, no. 3 (2023): 416. http://dx.doi.org/10.3390/metabo13030416.

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Neurodegenerative diseases are characterized by the progressive loss of neurons, synapses, dendrites, and myelin in the central and/or peripheral nervous system. Actual therapeutic options for patients are scarce and merely palliative. Although they affect millions of patients worldwide, the molecular mechanisms underlying these conditions remain unclear. Mitochondrial dysfunction is generally found in neurodegenerative diseases and is believed to be involved in the pathomechanisms of these disorders. Therefore, therapies aiming to improve mitochondrial function are promising approaches for ne

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9

Carelli, Valerio, Chiara La Morgia, Luisa Iommarini, et al. "Mitochondrial Optic Neuropathies: How Two Genomes may Kill the Same Cell Type?" Bioscience Reports 27, no. 1-3 (2007): 173–84. http://dx.doi.org/10.1007/s10540-007-9045-0.

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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

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10

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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11

Grel, Hubert, Damian Woznica, Katarzyna Ratajczak, et al. "Mitochondrial Dynamics in Neurodegenerative Diseases: Unraveling the Role of Fusion and Fission Processes." International Journal of Molecular Sciences 24, no. 17 (2023): 13033. http://dx.doi.org/10.3390/ijms241713033.

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Neurodegenerative diseases (NDs) are a diverse group of disorders characterized by the progressive degeneration and death of neurons, leading to a range of neurological symptoms. Despite the heterogeneity of these conditions, a common denominator is the implication of mitochondrial dysfunction in their pathogenesis. Mitochondria play a crucial role in creating biomolecules, providing energy through adenosine triphosphate (ATP) generated by oxidative phosphorylation (OXPHOS), and producing reactive oxygen species (ROS). When they’re not functioning correctly, becoming fragmented and losing thei

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Markin, Alexander M., Viktoria A. Khotina, Xenia G. Zabudskaya, et al. "Disturbance of Mitochondrial Dynamics and Mitochondrial Therapies in Atherosclerosis." Life 11, no. 2 (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 pr

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Ore, Adaleiz, James M. Angelastro, and Cecilia Giulivi. "Integrating Mitochondrial Biology into Innovative Cell Therapies for Neurodegenerative Diseases." Brain Sciences 14, no. 9 (2024): 899. http://dx.doi.org/10.3390/brainsci14090899.

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The role of mitochondria in neurodegenerative diseases is crucial, and recent developments have highlighted its significance in cell therapy. Mitochondrial dysfunction has been implicated in various neurodegenerative disorders, including Alzheimer’s, Parkinson’s, amyotrophic lateral sclerosis, and Huntington’s diseases. Understanding the impact of mitochondrial biology on these conditions can provide valuable insights for developing targeted cell therapies. This mini-review refocuses on mitochondria and emphasizes the potential of therapies leveraging mesenchymal stem cells, embryonic stem cel

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Yang, Yanyan, Min Li, Yan Liu, 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.

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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 mitoc

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15

Al Ojaimi, Mode, Azza Salah, and Ayman W. El-Hattab. "Mitochondrial Fission and Fusion: Molecular Mechanisms, Biological Functions, and Related Disorders." Membranes 12, no. 9 (2022): 893. http://dx.doi.org/10.3390/membranes12090893.

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Mitochondria are dynamic organelles that undergo fusion and fission. These active processes occur continuously and simultaneously and are mediated by nuclear-DNA-encoded proteins that act on mitochondrial membranes. The balance between fusion and fission determines the mitochondrial morphology and adapts it to the metabolic needs of the cells. Therefore, these two processes are crucial to optimize mitochondrial function and its bioenergetics abilities. Defects in mitochondrial proteins involved in fission and fusion due to pathogenic variants in the genes encoding them result in disruption of

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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 (2022): 608. http://dx.doi.org/10.3390/ijms24010608.

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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 di

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Méndez-López, Iago, Francisco J. Sancho-Bielsa, Tobias Engel, Antonio G. G. García, and Juan Fernando Padín. "Progressive Mitochondrial SOD1G93A Accumulation Causes Severe Structural, Metabolic and Functional Aberrations through OPA1 Down-Regulation in a Mouse Model of Amyotrophic Lateral Sclerosis." International Journal of Molecular Sciences 22, no. 15 (2021): 8194. http://dx.doi.org/10.3390/ijms22158194.

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In recent years, the “non-autonomous motor neuron death” hypothesis has become more consolidated behind amyotrophic lateral sclerosis (ALS). It postulates that cells other than motor neurons participate in the pathology. In fact, the involvement of the autonomic nervous system is fundamental since patients die of sudden death when they become unable to compensate for cardiorespiratory arrest. Mitochondria are thought to play a fundamental role in the physiopathology of ALS, as they are compromised in multiple ALS models in different cell types, and it also occurs in other neurodegenerative dis

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Borysova, Olga. "MITOCHONDRIAL THERAPIES IN AGING AND AGE-RELATED DISEASES." Anti-Aging Eastern Europe 4, no. 2 (2025): 78–92. https://doi.org/10.56543/aaeeu.2025.4.2.02.

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Mitochondria, essential for cellular energy production and metabolic homeostasis, undergo progressive dysfunction with aging, contributing to a range of age-related diseases. Key mechanisms underlying mitochondrial deterioration include impaired mitophagy, cardiolipin remodeling, genetic instability, and excessive reactive oxygen species (ROS) accumulation. These dysfunctions are critical in the aging process, affecting brain function, cardiovascular health, metabolic health, fertility, and stem cell maintenance. This article explores the molecular basis of mitochondrial aging and its impact o

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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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20

Pires Da Silva, Julie, Mariana Casa de Vito, Carissa Miyano, and Carmen C. Sucharov. "Mitochondrial Dysfunction in Congenital Heart Disease." Journal of Cardiovascular Development and Disease 12, no. 2 (2025): 42. https://doi.org/10.3390/jcdd12020042.

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Mitochondria play a crucial role in multiple cellular processes such as energy metabolism, generation of reactive oxygen species, excitation–contraction coupling, cell survival and death. Dysfunction of mitochondria contributes to the development of cancer; neuromuscular, cardiovascular/congenital heart disease; and metabolic diseases, including diabetes. Mitochondrial dysfunction can result in excessive reactive oxygen species, a decrease in energy production, mitophagy and apoptosis. All these processes are known to be dysregulated in cardiovascular diseases. The focus of this review is to s

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Mohamud, Yasir, Boaz Li, Amirhossein Bahreyni, and Honglin Luo. "Mitochondria Dysfunction at the Heart of Viral Myocarditis: Mechanistic Insights and Therapeutic Implications." Viruses 15, no. 2 (2023): 351. http://dx.doi.org/10.3390/v15020351.

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The myocardium/heart is the most mitochondria-rich tissue in the human body with mitochondria comprising approximately 30% of total cardiomyocyte volume. As the resident “powerhouse” of cells, mitochondria help to fuel the high energy demands of a continuously beating myocardium. It is no surprise that mitochondrial dysfunction underscores the pathogenesis of many cardiovascular ailments, including those of viral origin such as virus-induced myocarditis. Enteroviruses have been especially linked to injuries of the myocardium and its sequelae dilated cardiomyopathy for which no effective therap

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22

Prasuhn, Jannik, and Norbert Brüggemann. "Gene Therapeutic Approaches for the Treatment of Mitochondrial Dysfunction in Parkinson’s Disease." Genes 12, no. 11 (2021): 1840. http://dx.doi.org/10.3390/genes12111840.

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Background: Mitochondrial dysfunction has been identified as a pathophysiological hallmark of disease onset and progression in patients with Parkinsonian disorders. Besides the overall emergence of gene therapies in treating these patients, this highly relevant molecular concept has not yet been defined as a target for gene therapeutic approaches. Methods: This narrative review will discuss the experimental evidence suggesting mitochondrial dysfunction as a viable treatment target in patients with monogenic and idiopathic Parkinson’s disease. In addition, we will focus on general treatment str

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Yang, Tsai-Hsuan, Eugene Yu-Chuan Kang, Pei-Hsuan Lin, et al. "Mitochondria in Retinal Ganglion Cells: Unraveling the Metabolic Nexus and Oxidative Stress." International Journal of Molecular Sciences 25, no. 16 (2024): 8626. http://dx.doi.org/10.3390/ijms25168626.

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This review explored the role of mitochondria in retinal ganglion cells (RGCs), which are essential for visual processing. Mitochondrial dysfunction is a key factor in the pathogenesis of various vision-related disorders, including glaucoma, hereditary optic neuropathy, and age-related macular degeneration. This review highlighted the critical role of mitochondria in RGCs, which provide metabolic support, regulate cellular health, and respond to cellular stress while also producing reactive oxygen species (ROS) that can damage cellular components. Maintaining mitochondrial function is essentia

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Hinton, Antentor O., Alhaji U. N’jai, Zer Vue, and Celestine Wanjalla. "Connection Between HIV and Mitochondria in Cardiovascular Disease and Implications for Treatments." Circulation Research 134, no. 11 (2024): 1581–606. http://dx.doi.org/10.1161/circresaha.124.324296.

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HIV infection and antiretroviral therapy alter mitochondrial function, which can progressively lead to mitochondrial damage and accelerated aging. The interaction between persistent HIV reservoirs and mitochondria may provide insight into the relatively high rates of cardiovascular disease and mortality in persons living with HIV. In this review, we explore the intricate relationship between HIV and mitochondrial function, highlighting the potential for novel therapeutic strategies in the context of cardiovascular diseases. We reflect on mitochondrial dynamics, mitochondrial DNA, and mitochond

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Mokhtari, Behnaz, Rana Yavari, Reza Badalzadeh, and Ata Mahmoodpoor. "An Overview on Mitochondrial-Based Therapies in Sepsis-Related Myocardial Dysfunction: Mitochondrial Transplantation as a Promising Approach." Canadian Journal of Infectious Diseases and Medical Microbiology 2022 (June 6, 2022): 1–17. http://dx.doi.org/10.1155/2022/3277274.

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Sepsis is defined as a life-threatening organ failure due to dysregulated host response to infection. Despite current advances in our knowledge about sepsis, it is still considered as a major global health challenge. Myocardial dysfunction is a well-defined manifestation of sepsis which is related to worse outcomes in septic patients. Given that the heart is a mitochondria-rich organ and the normal function of mitochondria is essential for successful modulation of septic response, the contribution of mitochondrial damage in sepsis-related myocardial dysfunction has attracted the attention of m

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Martel, Cécile, Le Ha Huynh, Anne Garnier, Renée Ventura-Clapier, and Catherine Brenner. "Inhibition of the Mitochondrial Permeability Transition for Cytoprotection: DirectversusIndirect Mechanisms." Biochemistry Research International 2012 (2012): 1–13. http://dx.doi.org/10.1155/2012/213403.

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Mitochondria are fascinating organelles, which fulfill multiple cellular functions, as diverse as energy production, fatty acidβoxidation, reactive oxygen species (ROS) production and detoxification, and cell death regulation. The coordination of these functions relies on autonomous mitochondrial processes as well as on sustained cross-talk with other organelles and/or the cytosol. Therefore, this implies a tight regulation of mitochondrial functions to ensure cell homeostasis. In many diseases (e.g., cancer, cardiopathies, nonalcoholic fatty liver diseases, and neurodegenerative diseases), mi

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Sita, Giulia, Patrizia Hrelia, Agnese Graziosi, and Fabiana Morroni. "Back to The Fusion: Mitofusin-2 in Alzheimer’s Disease." Journal of Clinical Medicine 9, no. 1 (2020): 126. http://dx.doi.org/10.3390/jcm9010126.

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Mitochondria are dynamic organelles that undergo constant fission and fusion. Mitochondria dysfunction underlies several human disorders, including Alzheimer’s disease (AD). Preservation of mitochondrial dynamics is fundamental for regulating the organelle’s functions. Several proteins participate in the regulation of mitochondrial morphology and networks, and among these, Mitofusin 2 (Mfn2) has been extensively studied. This review focuses on the role of Mfn2 in mitochondrial dynamics and in the crosstalk between mitochondria and the endoplasmic reticulum, in particular in AD. Understanding h

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Shilovsky, G. A., E. V. Sorokina, and D. N. Akhayev. "Anti-Aging Medicine: Mitochondrial-Directed Antioxidants and Physical Activity." Успехи современной биологии 144, no. 2 (2024): 155–64. http://dx.doi.org/10.31857/s0042132424020035.

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Mitochondria are an important source of reactive oxygen species in skeletal muscle. Mitochondrial dysfunction accompanies the development of age-related human diseases. Increased production of reactive oxygen species contributes to muscle atrophy caused, for example, by physical inactivity. Many regulatory pathways involved in mitochondrial biogenesis are targets of anti-aging therapies. Active lifestyle and exercise prevent age-related damage to skeletal muscle mitochondria. Another way to correct the action of reactive oxygen species is the use of antioxidants directly targeted to the mitoch

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D’Amato, Marco, Francesca Morra, Ivano Di Di Meo, and Valeria Tiranti. "Mitochondrial Transplantation in Mitochondrial Medicine: Current Challenges and Future Perspectives." International Journal of Molecular Sciences 24, no. 3 (2023): 1969. http://dx.doi.org/10.3390/ijms24031969.

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Mitochondrial diseases (MDs) are inherited genetic conditions characterized by pathogenic mutations in nuclear DNA (nDNA) or mitochondrial DNA (mtDNA). Current therapies are still far from being fully effective and from covering the broad spectrum of mutations in mtDNA. For example, unlike heteroplasmic conditions, MDs caused by homoplasmic mtDNA mutations do not yet benefit from advances in molecular approaches. An attractive method of providing dysfunctional cells and/or tissues with healthy mitochondria is mitochondrial transplantation. In this review, we discuss what is known about interce

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Middleton, Paul, and Nikhil Vergis. "Mitochondrial dysfunction and liver disease: role, relevance, and potential for therapeutic modulation." Therapeutic Advances in Gastroenterology 14 (January 2021): 175628482110313. http://dx.doi.org/10.1177/17562848211031394.

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Mitochondria are key organelles involved in energy production as well as numerous metabolic processes. There is a growing interest in the role of mitochondrial dysfunction in the pathogenesis of common chronic diseases as well as in cancer development. This review will examine the role mitochondria play in the pathophysiology of common liver diseases, including alcohol-related liver disease, non-alcoholic fatty liver disease, chronic hepatitis B and hepatocellular carcinoma. Mitochondrial dysfunction is described widely in the literature in studies examining patient tissue and in disease model

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James, Rachel, Helena Chaytow, Leire M. Ledahawsky, and Thomas H. Gillingwater. "Revisiting the role of mitochondria in spinal muscular atrophy." Cellular and Molecular Life Sciences 78, no. 10 (2021): 4785–804. http://dx.doi.org/10.1007/s00018-021-03819-5.

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AbstractSpinal muscular atrophy (SMA) is an autosomal recessive motor neuron disease of variable clinical severity that is caused by mutations in the survival motor neuron 1 (SMN1) gene. Despite its name, SMN is a ubiquitous protein that functions within and outside the nervous system and has multiple cellular roles in transcription, translation, and proteostatic mechanisms. Encouragingly, several SMN-directed therapies have recently reached the clinic, albeit this has highlighted the increasing need to develop combinatorial therapies for SMA to achieve full clinical efficacy. As a subcellular

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Blagov, Alexander V., Sergey Kozlov, Tatiana Blokhina, Vasily N. Sukhorukov, and Aleksander N. Orekhov. "Targeting Mitochondrial Dynamics Proteins for the Development of Therapies for Cardiovascular Diseases." International Journal of Molecular Sciences 23, no. 23 (2022): 14741. http://dx.doi.org/10.3390/ijms232314741.

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Cardiovascular diseases are one of the leading causes of death worldwide. The identification of new pathogenetic targets contributes to more efficient development of new types of drugs for the treatment of cardiovascular diseases. This review highlights the problem of mitochondrial dynamics disorders, in the context of cardiovascular diseases. A change in the normal function of mitochondrial dynamics proteins is one of the reasons for the development of the pathological state of cardiomyocytes. Based on this, therapeutic targeting of these proteins may be a promising strategy in the developmen

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Agrawal, Anurag, and Ulaganathan Mabalirajan. "Rejuvenating cellular respiration for optimizing respiratory function: targeting mitochondria." American Journal of Physiology-Lung Cellular and Molecular Physiology 310, no. 2 (2016): L103—L113. http://dx.doi.org/10.1152/ajplung.00320.2015.

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Altered bioenergetics with increased mitochondrial reactive oxygen species production and degradation of epithelial function are key aspects of pathogenesis in asthma and chronic obstructive pulmonary disease (COPD). This motif is not unique to obstructive airway disease, reported in related airway diseases such as bronchopulmonary dysplasia and parenchymal diseases such as pulmonary fibrosis. Similarly, mitochondrial dysfunction in vascular endothelium or skeletal muscles contributes to the development of pulmonary hypertension and systemic manifestations of lung disease. In experimental mode

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Maycotte, Paola, Alvaro Marín-Hernández, Miriam Goyri-Aguirre, Maricruz Anaya-Ruiz, Julio Reyes-Leyva, and Paulina Cortés-Hernández. "Mitochondrial dynamics and cancer." Tumor Biology 39, no. 5 (2017): 101042831769839. http://dx.doi.org/10.1177/1010428317698391.

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Cancer is among the leading causes of death worldwide, and the number of new cases continues to rise. Despite recent advances in diagnosis and therapeutic strategies, millions of cancer-related deaths occur, indicating the need for better therapies and diagnostic strategies. Mitochondria and metabolic alterations have been recognized as important for cancer progression. However, a more precise understanding of how to manipulate mitochondria-related processes for cancer therapy remains to be established. Mitochondria are highly dynamic organelles which continually fuse and divide in response to

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Tian, Rong, Wilson S. Colucci, Zoltan Arany, et al. "Unlocking the Secrets of Mitochondria in the Cardiovascular System." Circulation 140, no. 14 (2019): 1205–16. http://dx.doi.org/10.1161/circulationaha.119.040551.

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Mitochondria have emerged as a central factor in the pathogenesis and progression of heart failure, and other cardiovascular diseases, as well, but no therapies are available to treat mitochondrial dysfunction. The National Heart, Lung, and Blood Institute convened a group of leading experts in heart failure, cardiovascular diseases, and mitochondria research in August 2018. These experts reviewed the current state of science and identified key gaps and opportunities in basic, translational, and clinical research focusing on the potential of mitochondria-based therapeutic strategies in heart f

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Del Giudice, Luigi, Paola Pontieri, Mariarosaria Aletta, and Matteo Calcagnile. "Mitochondrial Neurodegenerative Diseases: Three Mitochondrial Ribosomal Proteins as Intermediate Stage in the Pathway That Associates Damaged Genes with Alzheimer’s and Parkinson’s." Biology 12, no. 7 (2023): 972. http://dx.doi.org/10.3390/biology12070972.

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Currently, numerous research endeavors are dedicated to unraveling the intricate nature of neurodegenerative diseases. These conditions are characterized by the gradual and progressive impairment of specific neuronal systems that exhibit anatomical or physiological connections. In particular, in the last twenty years, remarkable efforts have been made to elucidate neurodegenerative disorders such as Alzheimer's disease and Parkinson's disease. However, despite extensive research endeavors, no cure or effective treatment has been discovered thus far. With the emergence of studies shedding light

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Protasoni, Margherita, and Manuel Serrano. "Targeting Mitochondria to Control Ageing and Senescence." Pharmaceutics 15, no. 2 (2023): 352. http://dx.doi.org/10.3390/pharmaceutics15020352.

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Ageing is accompanied by a progressive impairment of cellular function and a systemic deterioration of tissues and organs, resulting in increased vulnerability to multiple diseases. Here, we review the interplay between two hallmarks of ageing, namely, mitochondrial dysfunction and cellular senescence. The targeting of specific mitochondrial features in senescent cells has the potential of delaying or even reverting the ageing process. A deeper and more comprehensive understanding of mitochondrial biology in senescent cells is necessary to effectively face this challenge. Here, we discuss the

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Li, Danni, Fenghua Tao, and Lin Jin. "Mitochondrial Dysfunction in Intervertebral Disc Degeneration: From Pathogenesis to Therapeutic Target." Oxidative Medicine and Cellular Longevity 2020 (November 27, 2020): 1–13. http://dx.doi.org/10.1155/2020/8880320.

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Mitochondria are cytosolic organelles essential for cellular function and survival. The function of mitochondria is maintained by mitochondrial quality control systems including mitochondrial fission and fusion to adapt the altered environment and mitophagy for removal of damaged mitochondria. Mitochondrial dysfunction is closely involved in aging-related diseases. Intervertebral disc (IVD) degeneration, an aging-associated process, is the major contributor to low back pain. Growing evidence has suggested that the mitochondrial function in IVD cells is severely compromised during the degenerat

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Ahuja, Abhimanyu S. "Understanding mitochondrial myopathies: a review." PeerJ 6 (May 21, 2018): e4790. http://dx.doi.org/10.7717/peerj.4790.

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Mitochondria are small, energy-producing structures vital to the energy needs of the body. Genetic mutations cause mitochondria to fail to produce the energy needed by cells and organs which can cause severe disease and death. These genetic mutations are likely to be in the mitochondrial DNA (mtDNA), or possibly in the nuclear DNA (nDNA). The goal of this review is to assess the current understanding of mitochondrial diseases. This review focuses on the pathology, causes, risk factors, symptoms, prevalence data, symptomatic treatments, and new research aimed at possible preventions and/or trea

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Protasoni, Margherita, and Massimo Zeviani. "Mitochondrial Structure and Bioenergetics in Normal and Disease Conditions." International Journal of Molecular Sciences 22, no. 2 (2021): 586. http://dx.doi.org/10.3390/ijms22020586.

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Mitochondria are ubiquitous intracellular organelles found in almost all eukaryotes and involved in various aspects of cellular life, with a primary role in energy production. The interest in this organelle has grown stronger with the discovery of their link to various pathologies, including cancer, aging and neurodegenerative diseases. Indeed, dysfunctional mitochondria cannot provide the required energy to tissues with a high-energy demand, such as heart, brain and muscles, leading to a large spectrum of clinical phenotypes. Mitochondrial defects are at the origin of a group of clinically he

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Protasoni, Margherita, and Massimo Zeviani. "Mitochondrial Structure and Bioenergetics in Normal and Disease Conditions." International Journal of Molecular Sciences 22, no. 2 (2021): 586. http://dx.doi.org/10.3390/ijms22020586.

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Mitochondria are ubiquitous intracellular organelles found in almost all eukaryotes and involved in various aspects of cellular life, with a primary role in energy production. The interest in this organelle has grown stronger with the discovery of their link to various pathologies, including cancer, aging and neurodegenerative diseases. Indeed, dysfunctional mitochondria cannot provide the required energy to tissues with a high-energy demand, such as heart, brain and muscles, leading to a large spectrum of clinical phenotypes. Mitochondrial defects are at the origin of a group of clinically he

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Bartman, Sydney, Giuseppe Coppotelli, and Jaime M. Ross. "Mitochondrial Dysfunction: A Key Player in Brain Aging and Diseases." Current Issues in Molecular Biology 46, no. 3 (2024): 1987–2026. http://dx.doi.org/10.3390/cimb46030130.

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Mitochondria are thought to have become incorporated within the eukaryotic cell approximately 2 billion years ago and play a role in a variety of cellular processes, such as energy production, calcium buffering and homeostasis, steroid synthesis, cell growth, and apoptosis, as well as inflammation and ROS production. Considering that mitochondria are involved in a multitude of cellular processes, mitochondrial dysfunction has been shown to play a role within several age-related diseases, including cancers, diabetes (type 2), and neurodegenerative diseases, although the underlying mechanisms ar

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Kar, Bibekananda, Santiago R. Castillo, Ankit Sabharwal, Karl J. Clark, and Stephen C. Ekker. "Mitochondrial Base Editing: Recent Advances towards Therapeutic Opportunities." International Journal of Molecular Sciences 24, no. 6 (2023): 5798. http://dx.doi.org/10.3390/ijms24065798.

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Mitochondria are critical organelles that form networks within our cells, generate energy dynamically, contribute to diverse cell and organ function, and produce a variety of critical signaling molecules, such as cortisol. This intracellular microbiome can differ between cells, tissues, and organs. Mitochondria can change with disease, age, and in response to the environment. Single nucleotide variants in the circular genomes of human mitochondrial DNA are associated with many different life-threatening diseases. Mitochondrial DNA base editing tools have established novel disease models and re

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Braga, Patrícia C., Marco G. Alves, Anabela S. Rodrigues, and Pedro F. Oliveira. "Mitochondrial Pathophysiology on Chronic Kidney Disease." International Journal of Molecular Sciences 23, no. 3 (2022): 1776. http://dx.doi.org/10.3390/ijms23031776.

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In healthy kidneys, interstitial fibroblasts are responsible for the maintenance of renal architecture. Progressive interstitial fibrosis is thought to be a common pathway for chronic kidney diseases (CKD). Diabetes is one of the boosters of CKD. There is no effective treatment to improve kidney function in CKD patients. The kidney is a highly demanding organ, rich in redox reactions occurring in mitochondria, making it particularly vulnerable to oxidative stress (OS). A dysregulation in OS leads to an impairment of the Electron transport chain (ETC). Gene deficiencies in the ETC are closely r

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Greener, Mark. "Is it time for mitochondria to take centre stage?" Prescriber 35, no. 3 (2024): 25–29. http://dx.doi.org/10.1002/psb.2128.

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Mitochondria have long been known as the ‘powerhouse of the cell’, but it has taken several decades for their essential role in human diseases and as a therapeutic target to be fully acknowledged. This article discusses the recent surge in interest in translating basic mitochondrial research into clinical therapies.

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Stamerra, Cosimo Andrea, Paolo Di Giosia, Paolo Giorgini, Claudio Ferri, Vasily N. Sukhorukov, and Amirhossein Sahebkar. "Mitochondrial Dysfunction and Cardiovascular Disease: Pathophysiology and Emerging Therapies." Oxidative Medicine and Cellular Longevity 2022 (August 2, 2022): 1–16. http://dx.doi.org/10.1155/2022/9530007.

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Mitochondria ensure the supply of cellular energy through the production of ATP via oxidative phosphorylation. The alteration of this process, called mitochondrial dysfunction, leads to a reduction in ATP and an increase in the production of reactive oxygen species (ROS). Mitochondrial dysfunction can be caused by mitochondrial/nuclear DNA mutations, or it can be secondary to pathological conditions such as cardiovascular disease, aging, and environmental stress. The use of therapies aimed at the prevention/correction of mitochondrial dysfunction, in the context of the specific treatment of ca

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Doblado, Laura, Claudia Lueck, Claudia Rey, et al. "Mitophagy in Human Diseases." International Journal of Molecular Sciences 22, no. 8 (2021): 3903. http://dx.doi.org/10.3390/ijms22083903.

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Mitophagy is a selective autophagic process, essential for cellular homeostasis, that eliminates dysfunctional mitochondria. Activated by inner membrane depolarization, it plays an important role during development and is fundamental in highly differentiated post-mitotic cells that are highly dependent on aerobic metabolism, such as neurons, muscle cells, and hepatocytes. Both defective and excessive mitophagy have been proposed to contribute to age-related neurodegenerative diseases, such as Parkinson’s and Alzheimer’s diseases, metabolic diseases, vascular complications of diabetes, myocardi

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Morciano, Giampaolo, Simone Patergnani, Massimo Bonora, et al. "Mitophagy in Cardiovascular Diseases." Journal of Clinical Medicine 9, no. 3 (2020): 892. http://dx.doi.org/10.3390/jcm9030892.

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Cardiovascular diseases are one of the leading causes of death. Increasing evidence has shown that pharmacological or genetic targeting of mitochondria can ameliorate each stage of these pathologies, which are strongly associated with mitochondrial dysfunction. Removal of inefficient and dysfunctional mitochondria through the process of mitophagy has been reported to be essential for meeting the energetic requirements and maintaining the biochemical homeostasis of cells. This process is useful for counteracting the negative phenotypic changes that occur during cardiovascular diseases, and unde

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Marra, Federica, Paola Lunetti, Rosita Curcio, et al. "An Overview of Mitochondrial Protein Defects in Neuromuscular Diseases." Biomolecules 11, no. 11 (2021): 1633. http://dx.doi.org/10.3390/biom11111633.

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Neuromuscular diseases (NMDs) are dysfunctions that involve skeletal muscle and cause incorrect communication between the nerves and muscles. The specific causes of NMDs are not well known, but most of them are caused by genetic mutations. NMDs are generally progressive and entail muscle weakness and fatigue. Muscular impairments can differ in onset, severity, prognosis, and phenotype. A multitude of possible injury sites can make diagnosis of NMDs difficult. Mitochondria are crucial for cellular homeostasis and are involved in various metabolic pathways; for this reason, their dysfunction can

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Matuz-Mares, Deyamira, Martin González-Andrade, Minerva Georgina Araiza-Villanueva, María Magdalena Vilchis-Landeros, and Héctor Vázquez-Meza. "Mitochondrial Calcium: Effects of Its Imbalance in Disease." Antioxidants 11, no. 5 (2022): 801. http://dx.doi.org/10.3390/antiox11050801.

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Calcium is used in many cellular processes and is maintained within the cell as free calcium at low concentrations (approximately 100 nM), compared with extracellular (millimolar) concentrations, to avoid adverse effects such as phosphate precipitation. For this reason, cells have adapted buffering strategies by compartmentalizing calcium into mitochondria and the endoplasmic reticulum (ER). In mitochondria, the calcium concentration is in the millimolar range, as it is in the ER. Mitochondria actively contribute to buffering cellular calcium, but if matrix calcium increases beyond physiologic

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