{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,29]],"date-time":"2026-08-29T00:35:33Z","timestamp":1787963733800,"version":"build-2784847793"},"reference-count":99,"publisher":"MDPI AG","issue":"21","license":[{"start":{"date-parts":[[2022,11,6]],"date-time":"2022-11-06T00:00:00Z","timestamp":1667692800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Start-up Grant for Stem Cell Regenerative Medicine","award":["5001-4001010"],"award-info":[{"award-number":["5001-4001010"]}]},{"name":"Start-up Grant for Stem Cell Regenerative Medicine","award":["JCYJ20210324114606019"],"award-info":[{"award-number":["JCYJ20210324114606019"]}]},{"name":"Shenzhen Science and Technology Program","award":["5001-4001010"],"award-info":[{"award-number":["5001-4001010"]}]},{"name":"Shenzhen Science and Technology Program","award":["JCYJ20210324114606019"],"award-info":[{"award-number":["JCYJ20210324114606019"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Cells"],"abstract":"<jats:p>Mitochondrial cardiomyopathy (MCM) is characterized by abnormal heart-muscle structure and function, caused by mutations in the nuclear genome or mitochondrial DNA. The heterogeneity of gene mutations and various clinical presentations in patients with cardiomyopathy make its diagnosis, molecular mechanism, and therapeutics great challenges. This review describes the molecular epidemiology of MCM and its clinical features, reviews the promising diagnostic tests applied for mitochondrial diseases and cardiomyopathies, and details the animal and cellular models used for modeling cardiomyopathy and to investigate disease pathogenesis in a controlled in vitro environment. It also discusses the emerging therapeutics tested in pre-clinical and clinical studies of cardiac regeneration.<\/jats:p>","DOI":"10.3390\/cells11213511","type":"journal-article","created":{"date-parts":[[2022,11,7]],"date-time":"2022-11-07T02:43:51Z","timestamp":1667789031000},"page":"3511","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":30,"title":["Mitochondrial Cardiomyopathy: Molecular Epidemiology, Diagnosis, Models, and Therapeutic Management"],"prefix":"10.3390","volume":"11","author":[{"given":"Jinjuan","family":"Yang","sequence":"first","affiliation":[{"name":"Cord Blood Bank Centre, Guangzhou Women and Children\u2019s Medical Centre, Guangzhou Medical University, Guangzhou 510180, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shaoxiang","family":"Chen","sequence":"additional","affiliation":[{"name":"Cord Blood Bank Centre, Guangzhou Women and Children\u2019s Medical Centre, Guangzhou Medical University, Guangzhou 510180, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5628-5226","authenticated-orcid":false,"given":"Fuyu","family":"Duan","sequence":"additional","affiliation":[{"name":"Cord Blood Bank Centre, Guangzhou Women and Children\u2019s Medical Centre, Guangzhou Medical University, Guangzhou 510180, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xiuxiu","family":"Wang","sequence":"additional","affiliation":[{"name":"Department of Laboratory Medicine, Pingyang People\u2019s Hospital Affiliated to Wenzhou Medical University, Wenzhou 325499, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xiaoxian","family":"Zhang","sequence":"additional","affiliation":[{"name":"Cord Blood Bank Centre, Guangzhou Women and Children\u2019s Medical Centre, Guangzhou Medical University, Guangzhou 510180, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0003-6632-1288","authenticated-orcid":false,"given":"Boonxuan","family":"Lian","sequence":"additional","affiliation":[{"name":"Adelaide Medical School, University of Adelaide, 30 Frome Rd., Adelaide, SA 5000, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Meng","family":"Kou","sequence":"additional","affiliation":[{"name":"Cord Blood Bank Centre, Guangzhou Women and Children\u2019s Medical Centre, Guangzhou Medical University, Guangzhou 510180, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zhixin","family":"Chiang","sequence":"additional","affiliation":[{"name":"Department of Allied Health Science Faculty of Science, Tunku Abdul Rahman University, Ipoh 31900, Malaysia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ziyue","family":"Li","sequence":"additional","affiliation":[{"name":"Cord Blood Bank Centre, Guangzhou Women and Children\u2019s Medical Centre, Guangzhou Medical University, Guangzhou 510180, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Qizhou","family":"Lian","sequence":"additional","affiliation":[{"name":"Cord Blood Bank Centre, Guangzhou Women and Children\u2019s Medical Centre, Guangzhou Medical University, Guangzhou 510180, China"},{"name":"Department of Surgery, Shenzhen Hong Kong University Hospital, Shenzhen 518053, China"},{"name":"State Key Laboratory of Pharmaceutical Biotechnology, University of Hong Kong, Hong Kong 999077, China"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2022,11,6]]},"reference":[{"key":"ref_1","first-page":"25","article-title":"Mitochondrial cardiomyopathies","volume":"3","author":"Scaglia","year":"2016","journal-title":"Front. Cardiovasc. Med."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"694","DOI":"10.1093\/cvr\/cvaa125","article-title":"Human-induced pluripotent stem cells for modelling metabolic perturbations and impaired bioenergetics underlying cardiomyopathies","volume":"117","author":"Ramachandra","year":"2021","journal-title":"Cardiovasc. Res."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"71","DOI":"10.2217\/fca.11.79","article-title":"Long-term cardiac prognosis and risk stratification in 260 adults presenting with mitochondrial diseases","volume":"8","author":"Limongelli","year":"2012","journal-title":"Future Cardiol."},{"key":"ref_4","first-page":"385","article-title":"Mitochondrial cardiomyopathy: Pathophysiology, diagnosis, and management","volume":"40","author":"Meyers","year":"2013","journal-title":"Texas Heart Inst. J."},{"key":"ref_5","first-page":"2886","article-title":"Long-term cardiac prognosis and risk stratification in 260 adults presenting with mitochondrial diseases","volume":"8","author":"Stojkovic","year":"2015","journal-title":"Future Cardiol."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"3023","DOI":"10.1093\/eurheartj\/ehs275","article-title":"Clinical update cardiac involvement in mitochondrial DNA disease: Clinical spectrum, diagnosis, and management","volume":"33","author":"Bates","year":"2012","journal-title":"Eur. Heart J."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"48","DOI":"10.1016\/j.ijcard.2021.06.042","article-title":"Long-term prognosis and genetic background of cardiomyopathy in 223 pediatric mitochondrial disease patients","volume":"341","author":"Matsunaga","year":"2021","journal-title":"Int. J. Cardiol."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"1360","DOI":"10.1016\/j.cjca.2015.08.017","article-title":"Mitochondrial diseases and cardiomyopathies","volume":"31","author":"Levtova","year":"2015","journal-title":"Can. J. Cardiol."},{"key":"ref_9","doi-asserted-by":"crossref","unstructured":"Kohda, M., Tokuzawa, Y., Kishita, Y., Nyuzuki, H., Moriyama, Y., Mizuno, Y., Hirata, T., Yatsuka, Y., Yamashita-Sugahara, Y., and Nakachi, Y. (2016). A comprehensive genomic analysis reveals the genetic landscape of mitochondrial respiratory chain complex deficiencies. PLoS Genet., 12.","DOI":"10.1371\/journal.pgen.1005679"},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"115","DOI":"10.1016\/j.ijcard.2019.01.017","article-title":"Cardiomyopathy in children with mitochondrial disease: Prognosis and genetic background","volume":"279","author":"Kishita","year":"2019","journal-title":"Int. J. Cardiol."},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"24","DOI":"10.1016\/j.ijcard.2009.03.057","article-title":"CMR gives clue to \u201cragged red fibers\u201d in the heart in a patient with mitochondrial myopathy","volume":"149","author":"Jose","year":"2011","journal-title":"Int. J. Cardiol."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"1832","DOI":"10.1161\/CIRCULATIONAHA.114.010779","article-title":"Extracellular volume imaging and quantitative T2 mapping for the diagnosis of mitochondrial cardiomyopathy","volume":"130","author":"Lee","year":"2014","journal-title":"Circulation"},{"key":"ref_13","doi-asserted-by":"crossref","unstructured":"Sharma, P., and Sampath, H. (2019). Mitochondrial DNA integrity: Role in health and disease. Cells, 8.","DOI":"10.3390\/cells8020100"},{"key":"ref_14","first-page":"227","article-title":"Images in cardiovascular medicine:cardiac magnetic resonance aids in the diagnosis of mitochondrial cardiomyopathy","volume":"123","author":"Cardiomyopathy","year":"2011","journal-title":"Circulation"},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"137","DOI":"10.1007\/s10741-016-9531-1","article-title":"Mitochondrial involvement in myocyte death and heart failure","volume":"21","author":"Goldenthal","year":"2016","journal-title":"Heart Fail. Rev."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"581","DOI":"10.3348\/kjr.2021.0815","article-title":"Differential diagnosis of thick myocardium according to histologic features revealed by multiparametric cardiac magnetic resonance imaging","volume":"23","author":"Cha","year":"2022","journal-title":"Korean J. Radiol."},{"key":"ref_17","first-page":"365","article-title":"Advanced pathological study for definite diagnosis of mitochondrial cardiomyopathy","volume":"17","author":"Takeda","year":"2020","journal-title":"J. Clin. pathol."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"754","DOI":"10.1016\/j.ijcard.2014.11.014","article-title":"Cardiac manifestations of primary mitochondrial disorders","volume":"177","author":"Finsterer","year":"2014","journal-title":"Int. J. Cardiol."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"221","DOI":"10.1016\/j.cjca.2018.11.018","article-title":"What the cardiologist should know about mitochondrial cardiomyopathy?","volume":"35","author":"Steinberg","year":"2019","journal-title":"Can. J. Cardiol."},{"key":"ref_20","first-page":"1380","article-title":"Patient care standards for primary mitochondrial disease: A consensus statement from the Mitochondrial Medicine Society","volume":"19","author":"Parikh","year":"2017","journal-title":"Genet. Med. Off. J. Am. Coll. Med. Genet."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"226","DOI":"10.1038\/ng0797-226","article-title":"A mouse model for mitochondrial myopathy and cardiomyopathy resulting from a deficiency in the heart\/muscle isoform of the adenine nucleotide translocator","volume":"16","author":"Graham","year":"1997","journal-title":"Nat. Genet."},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/j.jcmg.2010.06.018","article-title":"Adenine nucleotide translocase 1 deficiency results in dilated cardiomyopathy with defects in myocardial mechanics, histopathological alterations, and activation of apoptosis","volume":"4","author":"Narula","year":"2011","journal-title":"JACC. Cardiovasc. Imaging"},{"key":"ref_23","doi-asserted-by":"crossref","unstructured":"Richman, T.R., Ermer, J.A., Davies, S.M.K., Perks, K.L., Viola, H.M., Shearwood, A.-M.J., Hool, L.C., Rackham, O., and Filipovska, A. (2015). Mutation in MRPS34 compromises protein synthesis and causes mitochondrial dysfunction. PLoS Genet., 11.","DOI":"10.1371\/journal.pgen.1005089"},{"key":"ref_24","doi-asserted-by":"crossref","first-page":"19678","DOI":"10.1073\/pnas.1117835108","article-title":"Lethal mitochondrial cardiomyopathy in a hypomorphic Med30 mouse mutant is ameliorated by ketogenic diet","volume":"108","author":"Krebs","year":"2011","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_25","doi-asserted-by":"crossref","first-page":"6165","DOI":"10.1073\/pnas.1113987109","article-title":"Tissue-specific splicing of an Ndufs6 gene-trap insertion generates a mitochondrial complex I deficiency-specific cardiomyopathy","volume":"109","author":"Ke","year":"2012","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"74","DOI":"10.1161\/CIRCRESAHA.117.311349","article-title":"Mitochondrial cardiomyopathy caused by elevated reactive oxygen species and impaired cardiomyocyte proliferation","volume":"122","author":"Zhang","year":"2018","journal-title":"Circ. Res."},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"3136","DOI":"10.1073\/pnas.0308710100","article-title":"A switch in metabolism precedes increased mitochondrial biogenesis in respiratory chain-deficient mouse hearts","volume":"101","author":"Hansson","year":"2004","journal-title":"Proc. Natl. Acad. Sci. USA"},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"110475","DOI":"10.1016\/j.celrep.2022.110475","article-title":"Mutant CHCHD10 causes an extensive metabolic rewiring that precedes OXPHOS dysfunction in a murine model of mitochondrial cardiomyopathy","volume":"38","author":"Sayles","year":"2022","journal-title":"Cell Rep."},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"1547","DOI":"10.1093\/hmg\/ddaa077","article-title":"Loss of CHCHD2 and CHCHD10 activates OMA1 peptidase to disrupt mitochondrial cristae phenocopying patient mutations","volume":"29","author":"Liu","year":"2020","journal-title":"Hum. Mol. Genet."},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"381","DOI":"10.1002\/ame2.12193","article-title":"Novel rat model of multiple mitochondrial dysfunction syndromes (MMDS) complicated with cardiomyopathy","volume":"4","author":"Ling","year":"2021","journal-title":"Anim. Model. Exp. Med."},{"key":"ref_31","doi-asserted-by":"crossref","unstructured":"Meurs, K.M., Friedenberg, S.G., Olby, N.J., Condit, J., Weidman, J., Rosenthal, S., and Shelton, G.D. (2019). A QIL1 variant associated with ventricular arrhythmias and sudden cardiac death in the juvenile rhodesian ridgeback dog. Genes, 10.","DOI":"10.3390\/genes10020168"},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"13151","DOI":"10.1111\/jcmm.15921","article-title":"ndufa7 plays a critical role in cardiac hypertrophy","volume":"24","author":"Shi","year":"2020","journal-title":"J. Cell. Mol. Med."},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"263","DOI":"10.1067\/mlc.2002.127725","article-title":"Bimodal cardiac dysfunction in an animal model of iron overload","volume":"140","author":"Yang","year":"2002","journal-title":"J. Lab. Clin. Med."},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"542","DOI":"10.1038\/nm.3510","article-title":"Prevention and reversal of severe mitochondrial cardiomyopathy by gene therapy in a mouse model of Friedreich\u2019s ataxia","volume":"20","author":"Perdomini","year":"2014","journal-title":"Nat. Med."},{"key":"ref_35","doi-asserted-by":"crossref","first-page":"e9456","DOI":"10.15252\/emmm.201809456","article-title":"Alternative oxidase-mediated respiration prevents lethal mitochondrial cardiomyopathy","volume":"11","author":"Rajendran","year":"2019","journal-title":"EMBO Mol. Med."},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"e008289","DOI":"10.1161\/CIRCHEARTFAILURE.121.008289","article-title":"Cardiolipin remodeling defects impair mitochondrial architecture and function in a murine model of Barth syndrome cardiomyopathy","volume":"14","author":"Zhu","year":"2021","journal-title":"Circ. Heart Fail."},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"1173","DOI":"10.1093\/cvr\/cvx095","article-title":"Cardiomyocyte-specific loss of mitochondrial p32\/C1qbp causes cardiomyopathy and activates stress responses","volume":"113","author":"Saito","year":"2017","journal-title":"Cardiovasc. Res."},{"key":"ref_38","doi-asserted-by":"crossref","unstructured":"Shin, J., Lee, S.H., Kwon, M., Yang, D.K., Seo, H., Kim, J., Kim, Y., Im, S., Abel, E.D., and Kim, K. (2013). Cardiomyocyte specific deletion of Crif1 causes mitochondrial cardiomyopathy in mice. PLoS ONE, 8.","DOI":"10.1371\/journal.pone.0053577"},{"key":"ref_39","doi-asserted-by":"crossref","first-page":"475","DOI":"10.1016\/j.molmed.2011.05.001","article-title":"Pluripotent stem cell models of cardiac disease and their implication for drug discovery and development","volume":"17","author":"Davis","year":"2011","journal-title":"Trends Mol. Med."},{"key":"ref_40","doi-asserted-by":"crossref","first-page":"R115","DOI":"10.1093\/hmg\/ddw176","article-title":"Mouse models for mitochondrial diseases","volume":"25","author":"Ruzzenente","year":"2016","journal-title":"Hum. Mol. Genet."},{"key":"ref_41","doi-asserted-by":"crossref","first-page":"8173","DOI":"10.1093\/nar\/gkr546","article-title":"Correction of the consequences of mitochondrial 3243A>G mutation in the MT-TL1 gene causing the MELAS syndrome by tRNA import into mitochondria","volume":"39","author":"Karicheva","year":"2011","journal-title":"Nucleic Acids Res."},{"key":"ref_42","doi-asserted-by":"crossref","first-page":"5691215","DOI":"10.1155\/2017\/5691215","article-title":"Mitochondrial transfer from wharton\u2019s jelly mesenchymal stem cell to merrf cybrid reduces oxidative stress and improves mitochondrial bioenergetics","volume":"2017","author":"Chuang","year":"2017","journal-title":"Oxid. Med. Cell. Longev."},{"key":"ref_43","doi-asserted-by":"crossref","first-page":"857","DOI":"10.1016\/j.bbalip.2018.04.015","article-title":"Loss of tafazzin results in decreased myoblast differentiation in C2C12 cells: A myoblast model of Barth syndrome and cardiolipin deficiency","volume":"1863","author":"Lou","year":"2018","journal-title":"Biochim. Biophys. Acta Mol. Cell Biol. Lipid"},{"key":"ref_44","doi-asserted-by":"crossref","first-page":"401","DOI":"10.1042\/bj3180401","article-title":"Altered mitochondrial function in fibroblasts containing MELAS or MERRF mitochondrial DNA mutations","volume":"318","author":"James","year":"1996","journal-title":"Biochem. J."},{"key":"ref_45","doi-asserted-by":"crossref","first-page":"1994","DOI":"10.1016\/j.jacc.2003.06.015","article-title":"Phospholipid abnormalities in children with Barth syndrome","volume":"42","author":"Schlame","year":"2003","journal-title":"J. Am. Coll. Cardiol."},{"key":"ref_46","doi-asserted-by":"crossref","first-page":"1397","DOI":"10.1056\/NEJMoa0908679","article-title":"Patient-specific induced pluripotent stem-cell models for long-QT syndrome","volume":"363","author":"Moretti","year":"2010","journal-title":"N. Engl. J. Med."},{"key":"ref_47","doi-asserted-by":"crossref","first-page":"219","DOI":"10.1016\/j.fob.2015.03.008","article-title":"Impaired respiratory function in MELAS-induced pluripotent stem cells with high heteroplasmy levels","volume":"5","author":"Kodaira","year":"2015","journal-title":"FEBS Open Bio"},{"key":"ref_48","doi-asserted-by":"crossref","first-page":"e2551","DOI":"10.1038\/cddis.2016.484","article-title":"Mitochondrial respiratory dysfunction disturbs neuronal and cardiac lineage commitment of human iPSCs","volume":"8","author":"Yokota","year":"2017","journal-title":"Cell Death Dis."},{"key":"ref_49","doi-asserted-by":"crossref","first-page":"616","DOI":"10.1038\/nm.3545","article-title":"Modeling the mitochondrial cardiomyopathy of Barth syndrome with induced pluripotent stem cell and heart-on-chip technologies","volume":"20","author":"Wang","year":"2014","journal-title":"Nat. Med."},{"key":"ref_50","doi-asserted-by":"crossref","first-page":"3685","DOI":"10.1093\/eurheartj\/ehz326","article-title":"Modelling diastolic dysfunction in induced pluripotent stem cell-derived cardiomyocytes from hypertrophic cardiomyopathy patients","volume":"40","author":"Wu","year":"2019","journal-title":"Eur. Heart J."},{"key":"ref_51","doi-asserted-by":"crossref","first-page":"1831","DOI":"10.1007\/s00424-013-1414-x","article-title":"Modeling of friedreich ataxia-related iron overloading cardiomyopathy using patient-specific-induced pluripotent stem cells","volume":"466","author":"Lee","year":"2014","journal-title":"Pflugers Arch."},{"key":"ref_52","first-page":"608","article-title":"Neurons and cardiomyocytes derived from induced pluripotent stem cells as a model for mitochondrial defects in Friedreich\u2019s ataxia","volume":"6","author":"Hick","year":"2013","journal-title":"Dis. Model. Mech."},{"key":"ref_53","doi-asserted-by":"crossref","first-page":"808","DOI":"10.1016\/j.stemcr.2018.01.013","article-title":"Mitochondrial dysfunctions contribute to hypertrophic cardiomyopathy in patient iPSC-derived cardiomyocytes with MT-RNR2 mutation","volume":"10","author":"Li","year":"2018","journal-title":"Stem Cell Rep."},{"key":"ref_54","doi-asserted-by":"crossref","first-page":"101912","DOI":"10.1016\/j.scr.2020.101912","article-title":"Generation of two induced pluripotent stem cell lines (XACHi0010-A, XACHi0011-A)from a Chinese family with combined oxidative phosphorylation deficiency carrying homozygous and heterozygous C1QBP-L275F mutation","volume":"47","author":"Zhang","year":"2020","journal-title":"Stem Cell Res."},{"key":"ref_55","doi-asserted-by":"crossref","first-page":"554","DOI":"10.1016\/j.cjca.2019.09.021","article-title":"Reversible mitochondrial fragmentation in ipsc-derived cardiomyocytes from children with DCMA, a mitochondrial cardiomyopathy","volume":"36","author":"Rohani","year":"2019","journal-title":"Can. J. Cardiol."},{"key":"ref_56","doi-asserted-by":"crossref","first-page":"240","DOI":"10.1038\/mt.2012.207","article-title":"Induced pluripotent mesenchymal stromal cell clones retain donor-derived differences in DNA methylation profiles","volume":"21","author":"Shao","year":"2013","journal-title":"Mol. Ther. J. Am. Soc. Gene Ther."},{"key":"ref_57","doi-asserted-by":"crossref","first-page":"221","DOI":"10.4330\/wjc.v11.i10.221","article-title":"Cellular models for human cardiomyopathy: What is the best option?","volume":"11","author":"Greenway","year":"2019","journal-title":"World J. Cardiol."},{"key":"ref_58","doi-asserted-by":"crossref","first-page":"53","DOI":"10.1055\/s-0037-1608761","article-title":"Regenerative medicine\/cardiac cell therapy: Pluripotent stem cells","volume":"66","author":"Duran","year":"2018","journal-title":"Thorac. Cardiovasc. Surg."},{"key":"ref_59","first-page":"800529","article-title":"Recent Advances in Modeling Mitochondrial Cardiomyopathy Using Human Induced Pluripotent Stem Cells","volume":"9","author":"Cyganek","year":"2021","journal-title":"Front. Cell. Dev. Biol."},{"key":"ref_60","doi-asserted-by":"crossref","first-page":"1509","DOI":"10.1111\/jcmm.12356","article-title":"Serum supplemented culture medium masks hypertrophic phenotypes in human pluripotent stem cell derived cardiomyocytes","volume":"18","author":"Dambrot","year":"2014","journal-title":"J. Cell. Mol. Med."},{"key":"ref_61","doi-asserted-by":"crossref","first-page":"595","DOI":"10.1016\/j.stem.2012.02.014","article-title":"Erosion of dosage compensation impacts human iPSC disease modeling","volume":"10","author":"Mekhoubad","year":"2012","journal-title":"Cell Stem Cell"},{"key":"ref_62","doi-asserted-by":"crossref","first-page":"4302","DOI":"10.1111\/bph.14431","article-title":"Mitochondrial function in the heart: The insight into mechanisms and therapeutic potentials","volume":"176","author":"Bui","year":"2019","journal-title":"Br. J. Pharmacol."},{"key":"ref_63","doi-asserted-by":"crossref","first-page":"238","DOI":"10.1038\/nrcardio.2016.203","article-title":"Expert consensus document: Mitochondrial function as a therapeutic target in heart failure","volume":"14","author":"Brown","year":"2017","journal-title":"Nat. Rev. Cardiol."},{"key":"ref_64","doi-asserted-by":"crossref","first-page":"2256","DOI":"10.1161\/CIRCULATIONAHA.116.026099","article-title":"Nicotinamide riboside preserves cardiac function in a mouse model of dilated cardiomyopathy","volume":"137","author":"Diguet","year":"2018","journal-title":"Circulation"},{"key":"ref_65","doi-asserted-by":"crossref","first-page":"1449","DOI":"10.1016\/j.hlc.2021.05.074","article-title":"Stomatin-like protein-2: A potential target to treat mitochondrial cardiomyopathy","volume":"30","author":"Hu","year":"2021","journal-title":"Heart Lung Circ."},{"key":"ref_66","doi-asserted-by":"crossref","first-page":"18","DOI":"10.1016\/j.freeradbiomed.2018.01.012","article-title":"MitoQ improves mitochondrial dysfunction in heart failure induced by pressure overload","volume":"117","author":"Dabkowski","year":"2018","journal-title":"Free Radic. Biol. Med."},{"key":"ref_67","doi-asserted-by":"crossref","first-page":"1056","DOI":"10.1161\/HYPERTENSIONAHA.117.10787","article-title":"Chronic supplementation with a mitochondrial antioxidant (MitoQ) improves vascular function in healthy older adults","volume":"71","author":"Rossman","year":"2018","journal-title":"Hypertension"},{"key":"ref_68","doi-asserted-by":"crossref","unstructured":"Himori, K., Abe, M., Tatebayashi, D., Lee, J., Westerblad, H., Lanner, J.T., and Yamada, T. (2017). Superoxide dismutase\/catalase mimetic EUK-134 prevents diaphragm muscle weakness in monocrotalin-induced pulmonary hypertension. PLoS ONE, 12.","DOI":"10.1371\/journal.pone.0169146"},{"key":"ref_69","doi-asserted-by":"crossref","first-page":"39469","DOI":"10.18632\/oncotarget.5783","article-title":"Mitochondria-targeted antioxidant preserves contractile properties and mitochondrial function of skeletal muscle in aged rats","volume":"6","author":"Javadov","year":"2015","journal-title":"Oncotarget"},{"key":"ref_70","doi-asserted-by":"crossref","first-page":"167","DOI":"10.3389\/fcvm.2019.00167","article-title":"SS-31 peptide reverses the mitochondrial fragmentation present in fibroblasts from patients with DCMA, a mitochondrial cardiomyopathy","volume":"6","author":"Machiraju","year":"2019","journal-title":"Front. Cardiovasc. Med."},{"key":"ref_71","doi-asserted-by":"crossref","first-page":"106","DOI":"10.1161\/CIRCRESAHA.109.214601","article-title":"Therapeutic targeting of mitochondrial superoxide in hypertension","volume":"107","author":"Dikalova","year":"2010","journal-title":"Circ. Res."},{"key":"ref_72","doi-asserted-by":"crossref","first-page":"372","DOI":"10.1016\/S0008-6363(03)00533-9","article-title":"Mitochondrial permeability transition pore opening during myocardial reperfusion-a target for cardioprotection","volume":"61","author":"Halestrap","year":"2004","journal-title":"Cardiovasc. Res."},{"key":"ref_73","doi-asserted-by":"crossref","first-page":"150","DOI":"10.1016\/j.yjmcc.2006.09.013","article-title":"Cyclosporine A attenuates mitochondrial permeability transition and improves mitochondrial respiratory function in cardiomyocytes isolated from dogs with heart failure","volume":"42","author":"Sharov","year":"2007","journal-title":"J. Mol. Cell. Cardiol."},{"key":"ref_74","doi-asserted-by":"crossref","first-page":"494","DOI":"10.1038\/s41467-017-00664-8","article-title":"Metabolic stress-induced cardiomyopathy is caused by mitochondrial dysfunction due to attenuated Erk5 signaling","volume":"8","author":"Liu","year":"2017","journal-title":"Nat. Commun."},{"key":"ref_75","doi-asserted-by":"crossref","first-page":"455","DOI":"10.1042\/EBC20170113","article-title":"Advances in methods for reducing mitochondrial DNA disease by replacing or manipulating the mitochondrial genome","volume":"62","author":"Rai","year":"2018","journal-title":"Essays Biochem."},{"key":"ref_76","doi-asserted-by":"crossref","first-page":"383","DOI":"10.1038\/nature18303","article-title":"Towards clinical application of pronuclear transfer to prevent mitochondrial DNA disease","volume":"534","author":"Hyslop","year":"2016","journal-title":"Nature"},{"key":"ref_77","doi-asserted-by":"crossref","first-page":"627","DOI":"10.1038\/nature11647","article-title":"Towards germline gene therapy of inherited mitochondrial diseases","volume":"493","author":"Tachibana","year":"2013","journal-title":"Nature"},{"key":"ref_78","doi-asserted-by":"crossref","first-page":"1120","DOI":"10.1016\/j.stemcr.2018.09.012","article-title":"Connexin 43-mediated mitochondrial transfer of iPSC-MSCs alleviates asthma inflammation","volume":"11","author":"Yao","year":"2018","journal-title":"Stem Cell Rep."},{"key":"ref_79","doi-asserted-by":"crossref","first-page":"BSR20182417","DOI":"10.1042\/BSR20182417","article-title":"Mesenchymal stem cells and their mitochondrial transfer: A double-edged sword","volume":"39","author":"Li","year":"2019","journal-title":"Biosci. Rep."},{"key":"ref_80","doi-asserted-by":"crossref","first-page":"66","DOI":"10.1186\/s13578-022-00805-7","article-title":"Mitochondrial transfer\/transplantation: An emerging therapeutic approach for multiple diseases","volume":"12","author":"Liu","year":"2022","journal-title":"Cell Biosci."},{"key":"ref_81","doi-asserted-by":"crossref","first-page":"711","DOI":"10.1016\/j.athoracsur.2019.06.075","article-title":"Delayed transplantation of autologous mitochondria for cardioprotection in a porcine model","volume":"109","author":"Blitzer","year":"2020","journal-title":"Ann. Thorac. Surg."},{"key":"ref_82","doi-asserted-by":"crossref","first-page":"836","DOI":"10.1093\/ejcts\/ezz326","article-title":"Mitochondrial transplantation for myocardial protection in diabetic hearts","volume":"57","author":"Doulamis","year":"2020","journal-title":"Eur. J. Cardio-Thorac. Surg."},{"key":"ref_83","doi-asserted-by":"crossref","first-page":"241","DOI":"10.3389\/fphar.2017.00241","article-title":"Mitotherapy for fatty liver by intravenous administration of exogenous mitochondria in male mice","volume":"8","author":"Fu","year":"2017","journal-title":"Front. Pharmacol."},{"key":"ref_84","doi-asserted-by":"crossref","first-page":"913","DOI":"10.3727\/096368915X689785","article-title":"Transferring xenogenic mitochondria provides neural protection against ischemic stress in ischemic rat brains","volume":"25","author":"Huang","year":"2016","journal-title":"Cell Transpl."},{"key":"ref_85","doi-asserted-by":"crossref","first-page":"2395","DOI":"10.7150\/thno.29422","article-title":"Donation of mitochondria by iPSC-derived mesenchymal stem cells protects retinal ganglion cells against mitochondrial complex I defect-induced degeneration","volume":"9","author":"Jiang","year":"2019","journal-title":"Theranostics"},{"key":"ref_86","doi-asserted-by":"crossref","first-page":"7260","DOI":"10.7150\/thno.46332","article-title":"Theranostics bioenergetic crosstalk between mesenchymal stem cells and various ocular cells through the intercellular trafficking of mitochondria","volume":"10","author":"Jiang","year":"2020","journal-title":"Theranostics"},{"key":"ref_87","doi-asserted-by":"crossref","first-page":"921","DOI":"10.1016\/j.cmet.2016.04.007","article-title":"Mitochondrial transfer by photothermal nanoblade restores metabolite profile in mammalian cells","volume":"23","author":"Wu","year":"2016","journal-title":"Cell Metab."},{"key":"ref_88","doi-asserted-by":"crossref","first-page":"455","DOI":"10.1165\/rcmb.2013-0529OC","article-title":"Mitochondrial transfer of induced pluripotent stem cell-derived mesenchymal stem cells to airway epithelial cells attenuates cigarette smoke-induced damage","volume":"51","author":"Li","year":"2014","journal-title":"Am. J. Respir. Cell Mol. Biol."},{"key":"ref_89","doi-asserted-by":"crossref","first-page":"31284","DOI":"10.18632\/oncotarget.8893","article-title":"Mitochondrial transplantation attenuates hypoxic pulmonary vasoconstriction","volume":"7","author":"Zhou","year":"2016","journal-title":"Oncotarget"},{"key":"ref_90","doi-asserted-by":"crossref","first-page":"113114","DOI":"10.1016\/j.expneurol.2019.113114","article-title":"Therapeutic use of extracellular mitochondria in CNS injury and disease","volume":"324","author":"Nakamura","year":"2020","journal-title":"Exp. Neurol."},{"key":"ref_91","doi-asserted-by":"crossref","first-page":"40","DOI":"10.1016\/j.trsl.2015.12.003","article-title":"Allogeneic\/xenogeneic transplantation of peptide-labeled mitochondria in Parkinson\u2019s disease: Restoration of mitochondria functions and attenuation of 6-hydroxydopamine-induced neurotoxicity","volume":"170","author":"Chang","year":"2016","journal-title":"Transl. Res."},{"key":"ref_92","doi-asserted-by":"crossref","first-page":"1014","DOI":"10.1016\/j.jvs.2019.03.079","article-title":"Mitochondrial transplantation ameliorates acute limb ischemia","volume":"71","author":"Orfany","year":"2020","journal-title":"J. Vasc. Surg."},{"key":"ref_93","doi-asserted-by":"crossref","first-page":"e014501","DOI":"10.1161\/JAHA.119.014501","article-title":"Bioenergetics consequences of mitochondrial transplantation in cardiomyocytes","volume":"9","author":"Kenney","year":"2020","journal-title":"J. Am. Heart Assoc."},{"key":"ref_94","doi-asserted-by":"crossref","first-page":"C489","DOI":"10.1152\/ajpcell.00152.2021","article-title":"Mitochondrial transplantation in cardiomyocytes: Foundation, methods, and outcomes","volume":"321","author":"Hosseinian","year":"2021","journal-title":"Am. J. Physiol. Cell Physiol."},{"key":"ref_95","doi-asserted-by":"crossref","unstructured":"Louwagie, E.J., Larsen, T.D., Wachal, A.L., Gandy, T.C.T., and Baack, M.L. (2021). Mitochondrial transfer improves cardiomyocyte bioenergetics and viability in male rats exposed to pregestational diabetes. Int. J. Mol. Sci., 22.","DOI":"10.3390\/ijms22052382"},{"key":"ref_96","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1155\/2019\/7092151","article-title":"Vascular inflammation and oxidative stress: Major triggers for cardiovascular disease","volume":"2019","author":"Steven","year":"2019","journal-title":"Oxid. Med. Cell. Longev."},{"key":"ref_97","doi-asserted-by":"crossref","first-page":"749","DOI":"10.1016\/j.stemcr.2016.08.009","article-title":"iPSC-MSCs with high intrinsic Miro1 and sensitivity to TNF-\u03b1 yield efficacious mitochondrial transfer to rescue anthracycline-induced cardiomyopathy","volume":"7","author":"Zhang","year":"2016","journal-title":"Stem Cell Rep."},{"key":"ref_98","doi-asserted-by":"crossref","first-page":"286","DOI":"10.1016\/j.jtcvs.2017.02.018","article-title":"Autologous mitochondrial transplantation for dysfunction after ischemia-reperfusion injury","volume":"154","author":"Emani","year":"2017","journal-title":"J. Thorac. Cardiovasc. Surg."},{"key":"ref_99","first-page":"1630","article-title":"The functions, methods, and mobility of mitochondrial transfer between cells","volume":"11","author":"Qin","year":"2021","journal-title":"Front. Oncol."}],"container-title":["Cells"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2073-4409\/11\/21\/3511\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T01:11:24Z","timestamp":1760145084000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2073-4409\/11\/21\/3511"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,11,6]]},"references-count":99,"journal-issue":{"issue":"21","published-online":{"date-parts":[[2022,11]]}},"alternative-id":["cells11213511"],"URL":"https:\/\/doi.org\/10.3390\/cells11213511","relation":{},"ISSN":["2073-4409"],"issn-type":[{"value":"2073-4409","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,11,6]]}}}