{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,17]],"date-time":"2026-09-17T16:36:15Z","timestamp":1789662975383,"version":"4.0.1"},"reference-count":99,"publisher":"MDPI AG","issue":"2","license":[{"start":{"date-parts":[[2021,2,20]],"date-time":"2021-02-20T00:00:00Z","timestamp":1613779200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100006769","name":"Russian Science Foundation","doi-asserted-by":"publisher","award":["20-15-00337"],"award-info":[{"award-number":["20-15-00337"]}],"id":[{"id":"10.13039\/501100006769","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Life"],"abstract":"<jats:p>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\u2014features 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.<\/jats:p>","DOI":"10.3390\/life11020165","type":"journal-article","created":{"date-parts":[[2021,2,21]],"date-time":"2021-02-21T22:04:15Z","timestamp":1613945055000},"page":"165","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":26,"title":["Disturbance of Mitochondrial Dynamics and Mitochondrial Therapies in Atherosclerosis"],"prefix":"10.3390","volume":"11","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-6649-7924","authenticated-orcid":false,"given":"Alexander M.","family":"Markin","sequence":"first","affiliation":[{"name":"Laboratory of Cellular and Molecular Pathology of Cardiovascular System, Institute of Human Morphology, 117418 Moscow, Russia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-2096-3237","authenticated-orcid":false,"given":"Viktoria A.","family":"Khotina","sequence":"additional","affiliation":[{"name":"Laboratory of Cellular and Molecular Pathology of Cardiovascular System, Institute of Human Morphology, 117418 Moscow, Russia"},{"name":"Laboratory of Angiopathology, Institute of General Pathology and Pathophysiology, 8, Baltiyskaya St., 125315 Moscow, Russia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1290-574X","authenticated-orcid":false,"given":"Xenia G.","family":"Zabudskaya","sequence":"additional","affiliation":[{"name":"FSBI National Medical Research Center of Oncology named after N.N. Blokhin of the Ministry of Health of Russia, 115478 Moscow, Russia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Anastasia I.","family":"Bogatyreva","sequence":"additional","affiliation":[{"name":"Laboratory of Cellular and Molecular Pathology of Cardiovascular System, Institute of Human Morphology, 117418 Moscow, Russia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9262-9233","authenticated-orcid":false,"given":"Antonina V.","family":"Starodubova","sequence":"additional","affiliation":[{"name":"Federal Research Centre for Nutrition, Biotechnology and Food Safety, Ustinsky Passage, 109240 Moscow, Russia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3159-2881","authenticated-orcid":false,"given":"Ekaterina","family":"Ivanova","sequence":"additional","affiliation":[{"name":"Department of Basic Research, Institute of Atherosclerosis Research, 121609 Moscow, Russia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nikita G.","family":"Nikiforov","sequence":"additional","affiliation":[{"name":"Laboratory of Cellular and Molecular Pathology of Cardiovascular System, Institute of Human Morphology, 117418 Moscow, Russia"},{"name":"National Medical Research Center of Cardiology, Institute of Experimental Cardiology, 117418 Moscow, Russia"},{"name":"Institute of Gene Biology, Centre of collective usage, 119344 Moscow, Russia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3318-4681","authenticated-orcid":false,"given":"Alexander N.","family":"Orekhov","sequence":"additional","affiliation":[{"name":"Laboratory of Cellular and Molecular Pathology of Cardiovascular System, Institute of Human Morphology, 117418 Moscow, Russia"},{"name":"Laboratory of Angiopathology, Institute of General Pathology and Pathophysiology, 8, Baltiyskaya St., 125315 Moscow, Russia"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2021,2,20]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"25","DOI":"10.2174\/1570161116666180206111725","article-title":"Screening for Rare Genetic Variants Associated with Atherosclerosis: Opportunity for Personalized Medicine","volume":"17","author":"Peterlin","year":"2019","journal-title":"Curr. Vasc. Pharmacol."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"391","DOI":"10.18097\/PBMC20166204391","article-title":"Atherogenic modification of low-density lipoproteins","volume":"62","author":"Sukhorukov","year":"2016","journal-title":"Biomed. Khim."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"1211","DOI":"10.1038\/nm1102-1211","article-title":"Atherogenesis in perspective: Hypercholesterolemia and inflammation as partners in crime","volume":"8","author":"Steinberg","year":"2002","journal-title":"Nat. Med."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"758","DOI":"10.1007\/s11538-014-0010-3","article-title":"A mathematical model of atherosclerosis with reverse cholesterol transport and associated risk factors","volume":"77","author":"Friedman","year":"2015","journal-title":"Bull. Math. Biol."},{"key":"ref_5","doi-asserted-by":"crossref","unstructured":"Formanowicz, D., Krawczyk, J.B., Perek, B., and Formanowicz, P. (2019). A Control-Theoretic Model of Atherosclerosis. Int. J. Mol. Sci., 20.","DOI":"10.3390\/ijms20030785"},{"key":"ref_6","first-page":"4614","article-title":"Modelling of atherosclerosis in genetically modified animals","volume":"11","author":"Mushenkova","year":"2019","journal-title":"Am. J. Transl. Res."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"16080","DOI":"10.1038\/nrdp.2016.80","article-title":"Mitochondrial diseases","volume":"2","author":"Gorman","year":"2016","journal-title":"Nat. Rev. Dis Primers"},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"235","DOI":"10.1042\/EBC20170097","article-title":"Clinical syndromes associated with mtDNA mutations: Where we stand after 30 years","volume":"62","author":"Carelli","year":"2018","journal-title":"Essays Biochem."},{"key":"ref_9","doi-asserted-by":"crossref","unstructured":"Poznyak, A.V., Ivanova, E.A., Sobenin, I.A., Yet, S.F., and Orekhov, A.N. (2020). The Role of Mitochondria in Cardiovascular Diseases. Biology, 9.","DOI":"10.3390\/biology9060137"},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"1410","DOI":"10.1038\/nm.2538","article-title":"Atherosclerosis: Current pathogenesis and therapeutic options","volume":"17","author":"Weber","year":"2011","journal-title":"Nat. Med."},{"key":"ref_11","doi-asserted-by":"crossref","unstructured":"Bajraktari, A., Byty\u00e7i, I., and Henein, M.Y. (2020). The Relationship between Coronary Artery Wall Shear Strain and Plaque Morphology: A Systematic Review and Meta-Analysis. Diagnostics, 10.","DOI":"10.3390\/diagnostics10020091"},{"key":"ref_12","doi-asserted-by":"crossref","unstructured":"Orekhov, A.N., Nikiforov, N.N., Ivanova, E.A., and Sobenin, I.A. (2020). Possible Role of Mitochondrial DNA Mutations in Chronification of Inflammation: Focus on Atherosclerosis. J. Clin. Med., 9.","DOI":"10.3390\/jcm9040978"},{"key":"ref_13","doi-asserted-by":"crossref","unstructured":"Orekhov, A.N., Poznyak, A.V., Sobenin, I.A., Nikifirov, N.N., and Ivanova, E.A. (2019). Mitochondrion as a selective target for treatment of atherosclerosis: Role of mitochondrial DNA mutations and defective mitophagy in the pathogenesis of atherosclerosis and chronic inflammation. Curr. Neuropharmacol.","DOI":"10.2174\/1570159X17666191118125018"},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"1642","DOI":"10.1038\/s41588-018-0264-z","article-title":"Mitochondrial genetic medicine","volume":"50","author":"Wallace","year":"2018","journal-title":"Nat. Genet."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"101","DOI":"10.1007\/s10863-016-9648-x","article-title":"Complex formation and turnover of mitochondrial transporters and ion channels","volume":"49","author":"McStay","year":"2017","journal-title":"J. Bioenerg. Biomembr."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"7023","DOI":"10.1113\/JP273839","article-title":"Oxidative phosphorylation: Regulation and role in cellular and tissue metabolism","volume":"595","author":"Wilson","year":"2017","journal-title":"J. Physiol."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"821","DOI":"10.1016\/j.tibs.2019.07.002","article-title":"Emerging Roles for the Mitochondrial ATP Synthase Supercomplexes","volume":"44","author":"Nesci","year":"2019","journal-title":"Trends Biochem. Sci."},{"key":"ref_18","doi-asserted-by":"crossref","unstructured":"Song, R., Hu, X., and Zhang, L. (2019). Mitochondrial MiRNA in Cardiovascular Function and Disease. Cells, 8.","DOI":"10.3390\/cells8121475"},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"473","DOI":"10.1038\/embor.2012.57","article-title":"The neglected genome","volume":"13","author":"Pesole","year":"2012","journal-title":"EMBO Rep."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"920","DOI":"10.2741\/4525","article-title":"Mitochondrial DNA repair and damage tolerance","volume":"22","author":"Stein","year":"2017","journal-title":"Front. Biosci."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"18054","DOI":"10.3390\/ijms160818054","article-title":"Genes and pathways involved in adult onset disorders featuring muscle mitochondrial DNA instability","volume":"16","author":"Ahmed","year":"2015","journal-title":"Int. J. Mol. Sci."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"292017","DOI":"10.1155\/2014\/292017","article-title":"Quantitative assessment of heteroplasmy of mitochondrial genome: Perspectives in diagnostics and methodological pitfalls","volume":"2014","author":"Sobenin","year":"2014","journal-title":"BioMed Res. Int."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"a021220","DOI":"10.1101\/cshperspect.a021220","article-title":"Mitochondrial DNA genetics and the heteroplasmy conundrum in evolution and disease","volume":"5","author":"Wallace","year":"2013","journal-title":"Cold Spring Harb. Perspect. Biol."},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"102","DOI":"10.2741\/e860","article-title":"The role of mitochondria in cardiovascular diseases related to atherosclerosis","volume":"12","author":"Glanz","year":"2020","journal-title":"Front. Biosci."},{"key":"ref_25","doi-asserted-by":"crossref","unstructured":"Li, H., Slone, J., Fei, L., and Huang, T. (2019). Mitochondrial DNA Variants and Common Diseases: A Mathematical Model for the Diversity of Age-Related mtDNA Mutations. Cells, 8.","DOI":"10.3390\/cells8060608"},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"605","DOI":"10.1093\/nar\/gkz1128","article-title":"mitoXplorer, a visual data mining platform to systematically analyze and visualize mitochondrial expression dynamics and mutations","volume":"48","author":"Yim","year":"2020","journal-title":"Nucleic Acids Res."},{"key":"ref_27","doi-asserted-by":"crossref","unstructured":"Moro, L. (2019). Mitochondrial Dysfunction in Aging and Cancer. J. Clin. Med., 8.","DOI":"10.3390\/jcm8111983"},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"2467","DOI":"10.1681\/ASN.2015060661","article-title":"Association between mitochondrial DNA copy number in peripheral blood and incident CKD in the atherosclerosis risk in communities study","volume":"27","author":"Tin","year":"2016","journal-title":"J. Am. Soc. Nephrol."},{"key":"ref_29","first-page":"135698","article-title":"Mitochondrial Dysfunction: A Basic Mechanism in Inflammation-Related Non-Communicable Diseases and Therapeutic Opportunities","volume":"2013","author":"Rull","year":"2013","journal-title":"Mediat. Inflamm."},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"713","DOI":"10.1161\/CIRCRESAHA.116.309326","article-title":"Roles of Vascular Oxidative Stress and Nitric Oxide in the Pathogenesis of Atherosclerosis","volume":"120","author":"Xia","year":"2017","journal-title":"Circ. Res."},{"key":"ref_31","doi-asserted-by":"crossref","first-page":"1245049","DOI":"10.1155\/2016\/1245049","article-title":"Role of ROS and RNS Sources in Physiological and Pathological Conditions","volume":"2016","author":"Reed","year":"2016","journal-title":"Oxidative Med. Cell Longev."},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"10492","DOI":"10.1038\/s41598-017-10673-8","article-title":"Mitochondrial permeability transition pore: Sensitivity to opening and mechanistic dependence on substrate availability","volume":"7","author":"Briston","year":"2017","journal-title":"Sci. Rep."},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"943","DOI":"10.1111\/acel.12650","article-title":"The path from mitochondrial ROS to aging runs through the mitochondrial permeability transition pore","volume":"16","author":"Rottenberg","year":"2017","journal-title":"Aging Cell"},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"486","DOI":"10.1038\/s41418-017-0012-4","article-title":"Molecular mechanisms of cell death: Recommendations of the Nomenclature Committee on Cell Death 2018","volume":"25","author":"Galluzzi","year":"2018","journal-title":"Cell Death Differ."},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"12","DOI":"10.3389\/fcvm.2020.00012","article-title":"Mitochondrial ROS Formation in the Pathogenesis of Diabetic Cardiomyopathy","volume":"7","author":"Kaludercic","year":"2020","journal-title":"Front. Cardiovasc. Med."},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"3258","DOI":"10.1093\/eurheartj\/ehu436","article-title":"Mitochondrial injury and dysfunction in hypertension-induced cardiac damage","volume":"35","author":"Eirin","year":"2014","journal-title":"Eur. Heart J."},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"513","DOI":"10.1016\/j.cmet.2017.11.002","article-title":"NAD(+) Intermediates: The Biology and Therapeutic Potential of NMN and NR","volume":"27","author":"Yoshino","year":"2018","journal-title":"Cell Metab."},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"140","DOI":"10.3988\/jcn.2014.10.2.140","article-title":"Antioxidant effects of statins in patients with atherosclerotic cerebrovascular disease","volume":"10","author":"Moon","year":"2014","journal-title":"J. Clin. Neurol."},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"329","DOI":"10.1038\/s41419-017-0027-2","article-title":"Mitochondria-associated membranes (MAMs) and inflammation","volume":"9","author":"Missiroli","year":"2018","journal-title":"Cell Death Dis."},{"key":"ref_40","doi-asserted-by":"crossref","first-page":"1402","DOI":"10.12688\/f1000research.18901.1","article-title":"Inflammation in atherosclerotic cardiovascular disease","volume":"8","author":"Shah","year":"2019","journal-title":"F1000Research"},{"key":"ref_41","doi-asserted-by":"crossref","first-page":"4706","DOI":"10.1093\/hmg\/ddt319","article-title":"The ALS disease-associated mutant TDP-43 impairs mitochondrial dynamics and function in motor neurons","volume":"22","author":"Wang","year":"2013","journal-title":"Hum. Mol. Genet."},{"key":"ref_42","doi-asserted-by":"crossref","first-page":"126","DOI":"10.1016\/j.bone.2017.10.010","article-title":"ALS-associated mutation SOD1G93A leads to abnormal mitochondrial dynamics in osteocytes","volume":"106","author":"Wang","year":"2018","journal-title":"Bone"},{"key":"ref_43","doi-asserted-by":"crossref","first-page":"970","DOI":"10.1002\/humu.23262","article-title":"Recessive mutations in MSTO1 cause mitochondrial dynamics impairment, leading to myopathy and ataxia","volume":"38","author":"Nasca","year":"2017","journal-title":"Hum. Mutat."},{"key":"ref_44","doi-asserted-by":"crossref","first-page":"2229","DOI":"10.1161\/ATVBAHA.117.310298","article-title":"Mitochondrial Respiration and Atherosclerosis: R-E-S-P-I-R-E. Find Out What it Means to M\u03d5 (and VSMC)","volume":"37","author":"Forrester","year":"2017","journal-title":"Arter. Thromb. Vasc. Biol."},{"key":"ref_45","doi-asserted-by":"crossref","unstructured":"Kondadi, A.K., Anand, R., and Reichert, A.S. (2019). Functional Interplay between Cristae Biogenesis, Mitochondrial Dynamics and Mitochondrial DNA Integrity. Int. J. Mol. Sci., 20.","DOI":"10.3390\/ijms20174311"},{"key":"ref_46","doi-asserted-by":"crossref","first-page":"587","DOI":"10.1007\/s10545-017-0027-5","article-title":"MtDNA-maintenance defects: Syndromes and genes","volume":"40","author":"Viscomi","year":"2017","journal-title":"J. Inherit. Metab. Dis."},{"key":"ref_47","doi-asserted-by":"crossref","first-page":"379","DOI":"10.1016\/j.ebiom.2019.06.018","article-title":"The R941L mutation in MYH14 disrupts mitochondrial fission and associates with peripheral neuropathy","volume":"45","author":"Almutawa","year":"2019","journal-title":"EBioMedicine"},{"key":"ref_48","doi-asserted-by":"crossref","first-page":"7687083","DOI":"10.1155\/2018\/7687083","article-title":"Defective Autophagy in Atherosclerosis: To Die or to Senesce?","volume":"2018","author":"Grootaert","year":"2018","journal-title":"Oxidative Med. Cell Longev."},{"key":"ref_49","doi-asserted-by":"crossref","first-page":"193","DOI":"10.2337\/db16-0915","article-title":"Metformin suppresses diabetes-accelerated atherosclerosis via the inhibition of Drp1-mediated mitochondrial fission","volume":"66","author":"Wang","year":"2017","journal-title":"Diabetes"},{"key":"ref_50","doi-asserted-by":"crossref","first-page":"481","DOI":"10.1002\/ddr.21521","article-title":"Ilexgenin A inhibits mitochondrial fiss, ion and promote Drp1 degradation by Nrf2-induced PSMB5 in endothelial cells","volume":"80","author":"Zhu","year":"2019","journal-title":"Drug Dev. Res."},{"key":"ref_51","doi-asserted-by":"crossref","first-page":"220","DOI":"10.1161\/CIRCRESAHA.116.310293","article-title":"Dynamin-Related Protein 1 Inhibition Attenuates Cardiovascular Calcification in the Presence of Oxidative Stress","volume":"121","author":"Rogers","year":"2017","journal-title":"Circ. Res."},{"key":"ref_52","doi-asserted-by":"crossref","first-page":"77","DOI":"10.1080\/21623945.2019.1574194","article-title":"Maintaining mitochondria in beige adipose tissue","volume":"8","author":"Lu","year":"2019","journal-title":"Adipocyte"},{"key":"ref_53","doi-asserted-by":"crossref","first-page":"159","DOI":"10.1016\/j.cmet.2018.05.003","article-title":"Cardiolipid synthesis in brown and beige fat mitochondria is essential for systemic energy homeostasis","volume":"28","author":"Sustarsic","year":"2018","journal-title":"Cell Metab."},{"key":"ref_54","doi-asserted-by":"crossref","first-page":"245","DOI":"10.1038\/s41467-017-02068-0","article-title":"TGR5 signalling promotes mitochondrial fission and beige remodelling of white adipose tissue","volume":"9","author":"Perino","year":"2018","journal-title":"Nat. Commun."},{"key":"ref_55","doi-asserted-by":"crossref","first-page":"52","DOI":"10.1038\/s41421-018-0041-2","article-title":"RPS3A positively regulates the mitochondrial function of human periaortic adipose tissue and is associated with coronary artery diseases","volume":"4","author":"Tang","year":"2018","journal-title":"Cell Discov."},{"key":"ref_56","doi-asserted-by":"crossref","unstructured":"Worthmann, A., Schlein, C., Berb\u00e9e, J.F.P., Rensen, P.C.N., Heeren, J., and Bartelt, A. (2019). Effects of Pharmacological Thermogenic Adipocyte Activation on Metabolism and Atherosclerotic Plaque Regression. Nutrients, 11.","DOI":"10.3390\/nu11020463"},{"key":"ref_57","doi-asserted-by":"crossref","unstructured":"Lizcano, F. (2019). The Beige Adipocyte as a Therapy for Metabolic Diseases. Int. J. Mol. Sci., 20.","DOI":"10.3390\/ijms20205058"},{"key":"ref_58","doi-asserted-by":"crossref","first-page":"548","DOI":"10.1016\/j.cell.2007.06.026","article-title":"Mitochondrial fusion protects against neurodegeneration in the cerebellum","volume":"130","author":"Chen","year":"2007","journal-title":"Cell"},{"key":"ref_59","doi-asserted-by":"crossref","unstructured":"Silva Ramos, E., Motori, E., Bruser, C., Kuhl, I., Yeroslaviz, A., Ruzzenente, B., Kauppila, J.H.K., Busch, J.D., Hultenby, K., and Habermann, B.H. (2019). Mitochondrial fusion is required for regulation of mitochondrial DNA replication. PLoS Genet., 15.","DOI":"10.1371\/journal.pgen.1008085"},{"key":"ref_60","doi-asserted-by":"crossref","first-page":"7743","DOI":"10.1074\/jbc.C200677200","article-title":"Loss of OPA1 perturbates the mitochondrial inner membrane structure and integrity, leading to cytochrome c release and apoptosis","volume":"278","author":"Olichon","year":"2003","journal-title":"J. Biol. Chem."},{"key":"ref_61","doi-asserted-by":"crossref","first-page":"e003012","DOI":"10.1161\/JAHA.112.003012","article-title":"OPA1 mutation and late-onset cardiomyopathy: Mitochondrial dysfunction and mtDNA instability","volume":"1","author":"Chen","year":"2012","journal-title":"J. Am. Heart Assoc."},{"key":"ref_62","doi-asserted-by":"crossref","first-page":"1374","DOI":"10.1016\/j.cmet.2017.04.021","article-title":"Age-Associated Loss of OPA1 in Muscle Impacts Muscle Mass, Metabolic Homeostasis, Systemic Inflammation, and Epithelial Senescence","volume":"25","author":"Tezze","year":"2017","journal-title":"Cell Metab."},{"key":"ref_63","doi-asserted-by":"crossref","first-page":"1013","DOI":"10.1007\/s00401-019-02059-z","article-title":"MSTO1 mutations cause mtDNA depletion, manifesting as muscular dystrophy with cerebellar involvement","volume":"138","author":"Donkervoort","year":"2019","journal-title":"Acta Neuropathol."},{"key":"ref_64","doi-asserted-by":"crossref","first-page":"11863","DOI":"10.1073\/pnas.1301951110","article-title":"Dynamics of nucleoid structure regulated by mitochondrial fission contributes to cristae reformation and release of cytochrome c","volume":"110","author":"Ishihara","year":"2013","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_65","doi-asserted-by":"crossref","first-page":"211","DOI":"10.1128\/MCB.01054-14","article-title":"Dynamics of mitochondrial DNA nucleoids regulated by mitochondrial fission is essential for maintenance of homogeneously active mitochondria during neonatal heart development","volume":"35","author":"Ishihara","year":"2015","journal-title":"Mol. Cell Biol."},{"key":"ref_66","doi-asserted-by":"crossref","first-page":"795","DOI":"10.1083\/jcb.201507035","article-title":"Titration of mitochondrial fusion rescues Mff-deficient cardiomyopathy","volume":"211","author":"Chen","year":"2015","journal-title":"J. Cell Biol."},{"key":"ref_67","doi-asserted-by":"crossref","first-page":"380","DOI":"10.1038\/cdd.2015.102","article-title":"Mic60\/Mitofilin determines MICOS assembly essential for mitochondrial dynamics and mtDNA nucleoid organization","volume":"23","author":"Li","year":"2016","journal-title":"Cell Death Differ."},{"key":"ref_68","first-page":"186","article-title":"Mitochondrial DNA depletion syndromes: Review and updates of genetic basis, manifestations, and therapeutic options","volume":"10","author":"Scaglia","year":"2013","journal-title":"Neurother J. Am. Soc. Exp. Neuro Ther."},{"key":"ref_69","doi-asserted-by":"crossref","first-page":"D789","DOI":"10.1093\/nar\/gku1205","article-title":"OMIM.org: Online Mendelian Inheritance in Man (OMIM\u00ae), an online catalog of human genes and genetic disorders","volume":"43","author":"Amberger","year":"2015","journal-title":"Nucleic Acids Res."},{"key":"ref_70","doi-asserted-by":"crossref","first-page":"933","DOI":"10.1111\/brv.12378","article-title":"Structure, function, and regulation of mitofusin-2 in health and disease","volume":"93","author":"Chandhok","year":"2018","journal-title":"Biol. Rev. Camb. Philos. Soc."},{"key":"ref_71","doi-asserted-by":"crossref","first-page":"e19264","DOI":"10.7554\/eLife.19264","article-title":"Mouse Tmem135 mutation reveals a mechanism involving mitochondrial dynamics that leads to age-dependent retinal pathologies","volume":"5","author":"Lee","year":"2016","journal-title":"eLife"},{"key":"ref_72","doi-asserted-by":"crossref","unstructured":"Serrat, R., L\u00f3pez-Dom\u00e9nech, G., Mirra, S., Quevedo, M., Garcia-Fern\u00e0ndez, J., Ulloa, F., Burgaya, F., and Soriano, E. (2013). The Non-Canonical Wnt\/PKC Pathway Regulates Mitochondrial Dynamics through Degradation of the Arm-Like Domain-Containing Protein Alex3. PLoS ONE, 8.","DOI":"10.1371\/journal.pone.0067773"},{"key":"ref_73","doi-asserted-by":"crossref","first-page":"1213","DOI":"10.1089\/ars.2018.7718","article-title":"Mutations in RHOT1 Disrupt Endoplasmic Reticulum-Mitochondria Contact Sites Interfering with Calcium Homeostasis and Mitochondrial Dynamics in Parkinson\u2019s Disease","volume":"31","author":"Grossmann","year":"2019","journal-title":"Antioxid. Redox Signal."},{"key":"ref_74","doi-asserted-by":"crossref","unstructured":"Hartman, J.H., Gonzalez-Hunt, C., Hall, S.M., Ryde, I.T., Caldwell, K.A., Caldwell, G.A., and Meyer, J.N. (2019). Genetic Defects in Mitochondrial Dynamics in Caenorhabditis elegans Impact Ultraviolet C Radiation- and 6-hydroxydopamine-Induced Neurodegeneration. Int. J. Mol. Sci., 20.","DOI":"10.3390\/ijms20133202"},{"key":"ref_75","doi-asserted-by":"crossref","first-page":"58","DOI":"10.1161\/CIRCRESAHA.117.311307","article-title":"Mitochondrial Reactive Oxygen Species in Lipotoxic Hearts Induce Post-Translational Modifications of AKAP121, DRP1, and OPA1 That Promote Mitochondrial Fission","volume":"122","author":"Tsushima","year":"2018","journal-title":"Circ. Res."},{"key":"ref_76","doi-asserted-by":"crossref","first-page":"6483572","DOI":"10.1155\/2017\/6483572","article-title":"PGC1\u03b1 Activators Mitigate Diabetic Tubulopathy by Improving Mitochondrial Dynamics and Quality Control","volume":"2017","author":"Lee","year":"2017","journal-title":"J. Diabetes Res."},{"key":"ref_77","doi-asserted-by":"crossref","first-page":"7169","DOI":"10.1038\/s41598-017-05168-5","article-title":"Loss of succinyl-CoA synthase ADP-forming \u03b2 subunit disrupts mtDNA stability and mitochondrial dynamics in neurons","volume":"7","author":"Zhao","year":"2017","journal-title":"Sci. Rep."},{"key":"ref_78","doi-asserted-by":"crossref","first-page":"694","DOI":"10.1172\/JCI120606","article-title":"Peroxisome-derived lipids regulate adipose thermogenesis by mediating cold-induced mitochondrial fission","volume":"129","author":"Park","year":"2019","journal-title":"J. Clin Investig."},{"key":"ref_79","doi-asserted-by":"crossref","first-page":"967","DOI":"10.15252\/emmm.201607058","article-title":"MSTO 1 is a cytoplasmic pro-mitochondrial fusion protein, whose mutation induces myopathy and ataxia in humans","volume":"9","author":"Gal","year":"2017","journal-title":"EMBO Mol. Med."},{"key":"ref_80","doi-asserted-by":"crossref","first-page":"4508762","DOI":"10.1155\/2019\/4508762","article-title":"Impaired Mitochondrial Fusion and Oxidative Phosphorylation Triggered by High Glucose Is Mediated by Tom22 in Endothelial Cells","volume":"2019","author":"Zeng","year":"2019","journal-title":"Oxidative Med. Cell Longev."},{"key":"ref_81","doi-asserted-by":"crossref","first-page":"236","DOI":"10.1016\/j.siny.2011.05.003","article-title":"Therapy for mitochondrial disorders: Little proof, high research activity, some promise","volume":"16","author":"Suomalainen","year":"2011","journal-title":"Semin. Fetal Neonatal Med."},{"key":"ref_82","doi-asserted-by":"crossref","first-page":"76","DOI":"10.1016\/j.nmd.2011.05.001","article-title":"176th ENMC International Workshop: Diagnosis and treatment of coenzyme Q (1) (0) deficiency","volume":"22","author":"Rahman","year":"2012","journal-title":"Neuromuscul Disord."},{"key":"ref_83","doi-asserted-by":"crossref","first-page":"2849","DOI":"10.1056\/NEJMc0800582","article-title":"Early coenzyme Q10 supplementation in primary coenzyme Q10 deficiency","volume":"358","author":"Montini","year":"2008","journal-title":"N. Engl. J. Med."},{"key":"ref_84","doi-asserted-by":"crossref","first-page":"456","DOI":"10.1067\/mpd.2001.117575","article-title":"Neonatal presentation of coenzyme Q10 deficiency","volume":"139","author":"Rahman","year":"2001","journal-title":"J. Pediatr."},{"key":"ref_85","doi-asserted-by":"crossref","first-page":"558","DOI":"10.1016\/j.ajhg.2009.03.018","article-title":"A nonsense mutation in COQ9 causes autosomal-recessive neonatal-onset primary coenzyme Q10 deficiency: A potentially treatable form of mitochondrial disease","volume":"84","author":"Duncan","year":"2009","journal-title":"Am. J. Hum. Genet."},{"key":"ref_86","doi-asserted-by":"crossref","first-page":"943","DOI":"10.1136\/jnnp.2008.158279","article-title":"Diagnosis and therapy in neuromuscular disorders: Diagnosis and new treatments in mitochondrial diseases","volume":"80","author":"Rahman","year":"2009","journal-title":"J. Neurol. Neurosurg. Psychiatry"},{"key":"ref_87","doi-asserted-by":"crossref","first-page":"10061","DOI":"10.1074\/jbc.M117.789800","article-title":"The mitochondrial outer membrane protein mitoNEET is a redox enzyme catalyzing electron transfer from FMNH2 to oxygen or ubiquinone","volume":"292","author":"Wang","year":"2017","journal-title":"J. Biol. Chem."},{"key":"ref_88","doi-asserted-by":"crossref","first-page":"465","DOI":"10.1038\/nrd4023","article-title":"Pharmacological approaches to restore mitochondrial function","volume":"12","author":"Andreux","year":"2013","journal-title":"Nat. Rev. Drug Discov."},{"key":"ref_89","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1101\/cshperspect.a015008","article-title":"Mitochondrial biogenesis through activation of nuclear signaling proteins","volume":"5","author":"Dominy","year":"2013","journal-title":"Cold Spring Harb. Perspect. Biol."},{"key":"ref_90","doi-asserted-by":"crossref","first-page":"22","DOI":"10.1038\/jcbfm.2012.158","article-title":"The dynamics of the mitochondrial organelle as a potential therapeutic target","volume":"33","author":"Stetler","year":"2013","journal-title":"J. Cereb. Blood Flow Metab."},{"key":"ref_91","doi-asserted-by":"crossref","first-page":"211","DOI":"10.1038\/79944","article-title":"OPA1, encoding a dynamin-related GTPase, is mutated in autosomal dominant optic atrophy linked to chromosome 3q28","volume":"26","author":"Alexander","year":"2000","journal-title":"Nat. Genet."},{"key":"ref_92","doi-asserted-by":"crossref","first-page":"449","DOI":"10.1038\/ng1341","article-title":"Mutations in the mitochondrial GTPase mitofusin 2 cause Charcot-Marie-Tooth neuropathy type 2A","volume":"36","author":"Zuchner","year":"2004","journal-title":"Nat. Genet."},{"key":"ref_93","doi-asserted-by":"crossref","first-page":"1736","DOI":"10.1056\/NEJMoa064436","article-title":"A lethal defect of mitochondrial and peroxisomal fission","volume":"356","author":"Waterham","year":"2007","journal-title":"N. Engl. J. Med."},{"key":"ref_94","doi-asserted-by":"crossref","first-page":"193","DOI":"10.1016\/j.devcel.2007.11.019","article-title":"Chemical inhibition of the mitochondrial division dynamin reveals its role in Bax\/Bak-dependent mitochondrial outer membrane permeabilization","volume":"14","author":"Chipuk","year":"2008","journal-title":"Dev. Cell."},{"key":"ref_95","doi-asserted-by":"crossref","first-page":"9302","DOI":"10.1002\/anie.201204589","article-title":"A small molecule promotes mitochondrial fusion in mammalian cells","volume":"51","author":"Wang","year":"2012","journal-title":"Angew. Chem. Int. Ed. Engl."},{"key":"ref_96","first-page":"789","article-title":"A novel Drp1 inhibitor diminishes aberrant mitochondrial fission and neurotoxicity","volume":"126","author":"Qi","year":"2013","journal-title":"J. Cell Sci."},{"key":"ref_97","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1186\/s13027-017-0145-6","article-title":"The efficacy of epigallocatechin-3-gallate (green tea) in the treatment of Alzheimer\u2019s disease: An overview of pre-clinical studies and translational perspectives in clinical practice","volume":"12","author":"Cascella","year":"2017","journal-title":"Infect. Agent Cancer"},{"key":"ref_98","doi-asserted-by":"crossref","first-page":"3489","DOI":"10.1111\/bph.14585","article-title":"Mitochondrial dysfunction in Alzheimer\u2019s disease: Role in pathogenesis and novel therapeutic opportunities","volume":"176","author":"Swerdlow","year":"2019","journal-title":"Br. J. Pharmacol."},{"key":"ref_99","doi-asserted-by":"crossref","first-page":"127","DOI":"10.1016\/j.ejogrb.2009.12.005","article-title":"Fetal programming of atherosclerosis: Possible role of the mitochondria","volume":"149","author":"Leduc","year":"2010","journal-title":"Eur. J. Obstet. Gynecol. Reprod. Biol."}],"container-title":["Life"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2075-1729\/11\/2\/165\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T05:27:15Z","timestamp":1760160435000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2075-1729\/11\/2\/165"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2021,2,20]]},"references-count":99,"journal-issue":{"issue":"2","published-online":{"date-parts":[[2021,2]]}},"alternative-id":["life11020165"],"URL":"https:\/\/doi.org\/10.3390\/life11020165","relation":{},"ISSN":["2075-1729"],"issn-type":[{"value":"2075-1729","type":"electronic"}],"subject":[],"published":{"date-parts":[[2021,2,20]]}}}