{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,11,6]],"date-time":"2026-11-06T02:40:19Z","timestamp":1793932819972,"version":"4.8.0"},"reference-count":137,"publisher":"Elsevier BV","issue":"12","license":[{"start":{"date-parts":[[2022,12,1]],"date-time":"2022-12-01T00:00:00Z","timestamp":1669852800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2022,12,1]],"date-time":"2022-12-01T00:00:00Z","timestamp":1669852800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"},{"start":{"date-parts":[[2022,4,20]],"date-time":"2022-04-20T00:00:00Z","timestamp":1650412800000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["clinicalkey.com","clinicalkey.com.au","clinicalkey.es","clinicalkey.fr","clinicalkey.jp","jacc.org","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["JACC: Basic to Translational Science"],"published-print":{"date-parts":[[2022,12]]},"DOI":"10.1016\/j.jacbts.2022.04.005","type":"journal-article","created":{"date-parts":[[2022,7,13]],"date-time":"2022-07-13T17:05:29Z","timestamp":1657731929000},"page":"1267-1283","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":31,"title":["Cardiovascular Research in Friedreich\u00a0Ataxia"],"prefix":"10.1016","volume":"7","author":[{"given":"R. Mark","family":"Payne","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.jacbts.2022.04.005_bib1","doi-asserted-by":"crossref","first-page":"66","DOI":"10.3389\/fphar.2013.00066","article-title":"Burden of Friedreich's ataxia to the patients and healthcare systems in the United States and Canada","volume":"4","author":"Polek","year":"2013","journal-title":"Front Pharmacol"},{"issue":"5254","key":"10.1016\/j.jacbts.2022.04.005_bib2","doi-asserted-by":"crossref","first-page":"1423","DOI":"10.1126\/science.271.5254.1423","article-title":"Friedreich's ataxia: autosomal recessive disease caused by an intronic GAA triplet repeat expansion","volume":"271","author":"Campuzano","year":"1996","journal-title":"Science"},{"issue":"5","key":"10.1016\/j.jacbts.2022.04.005_bib3","doi-asserted-by":"crossref","first-page":"743","DOI":"10.1001\/archneur.59.5.743","article-title":"Friedreich ataxia: effects of genetic understanding on clinical evaluation and therapy","volume":"59","author":"Lynch","year":"2002","journal-title":"Arch Neurol"},{"issue":"22","key":"10.1016\/j.jacbts.2022.04.005_bib4","doi-asserted-by":"crossref","first-page":"3521","DOI":"10.1093\/hmg\/ddn244","article-title":"The in\u00a0vivo mitochondrial two-step maturation of human frataxin","volume":"17","author":"Schmucker","year":"2008","journal-title":"Hum Mol Genet"},{"key":"10.1016\/j.jacbts.2022.04.005_bib5","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/bs.ampbs.2020.01.001","article-title":"Making iron-sulfur cluster: structure, regulation and evolution of the bacterial ISC system","volume":"76","author":"Baussier","year":"2020","journal-title":"Adv Microb Physiol"},{"issue":"18","key":"10.1016\/j.jacbts.2022.04.005_bib6","doi-asserted-by":"crossref","first-page":"6821","DOI":"10.3390\/ijms21186821","article-title":"A\u00a0highly conserved iron-sulfur cluster assembly machinery between humans and amoeba Dictyostelium discoideum: the characterization of frataxin","volume":"21","author":"Olmos","year":"2020","journal-title":"Int J Mol Sci"},{"issue":"5","key":"10.1016\/j.jacbts.2022.04.005_bib7","doi-asserted-by":"crossref","first-page":"269","DOI":"10.1080\/10409230600846058","article-title":"The structure and function of frataxin","volume":"41","author":"Bencze","year":"2006","journal-title":"Crit Rev Biochem Mol Biol"},{"issue":"4","key":"10.1016\/j.jacbts.2022.04.005_bib8","doi-asserted-by":"crossref","first-page":"352","DOI":"10.1038\/ng0897-352","article-title":"Respiratory deficiency due to loss of mitochondrial DNA in yeast lacking the frataxin homologue","volume":"16","author":"Wilson","year":"1997","journal-title":"Nat Genet"},{"issue":"6","key":"10.1016\/j.jacbts.2022.04.005_bib9","doi-asserted-by":"crossref","first-page":"1493","DOI":"10.1016\/j.bbamcr.2014.09.009","article-title":"Iron-sulfur cluster biogenesis in mammalian cells: new insights into the molecular mechanisms of cluster delivery","volume":"1853","author":"Maio","year":"2015","journal-title":"Biochim Biophys Acta"},{"issue":"22","key":"10.1016\/j.jacbts.2022.04.005_bib10","doi-asserted-by":"crossref","first-page":"2651","DOI":"10.1093\/hmg\/ddm163","article-title":"Frataxin is essential for extramitochondrial Fe-S cluster proteins in mammalian tissues","volume":"16","author":"Martelli","year":"2007","journal-title":"Hum Mol Genet"},{"issue":"3","key":"10.1016\/j.jacbts.2022.04.005_bib11","doi-asserted-by":"crossref","first-page":"133","DOI":"10.1016\/j.tibs.2005.01.006","article-title":"Iron-sulfur-protein biogenesis in eukaryotes","volume":"30","author":"Lill","year":"2005","journal-title":"Trends Biochem Sci"},{"issue":"12","key":"10.1016\/j.jacbts.2022.04.005_bib12","doi-asserted-by":"crossref","first-page":"2688","DOI":"10.1093\/hmg\/dds095","article-title":"Friedreich's ataxia reveals a mechanism for coordinate regulation of oxidative metabolism via feedback inhibition of the SIRT3 deacetylase","volume":"21","author":"Wagner","year":"2012","journal-title":"Hum Mol Genet"},{"issue":"14","key":"10.1016\/j.jacbts.2022.04.005_bib13","doi-asserted-by":"crossref","DOI":"10.1172\/jci.insight.93885","article-title":"Nicotinamide mononucleotide requires SIRT3 to improve cardiac function and bioenergetics in a Friedreich's ataxia cardiomyopathy model","volume":"2","author":"Martin","year":"2017","journal-title":"JCI Insight"},{"issue":"1","key":"10.1016\/j.jacbts.2022.04.005_bib14","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pgen.1000812","article-title":"Altered gene expression and DNA damage in peripheral blood cells from Friedreich's ataxia patients: cellular model of pathology","volume":"6","author":"Haugen","year":"2010","journal-title":"PLoS Genet"},{"issue":"5","key":"10.1016\/j.jacbts.2022.04.005_bib15","doi-asserted-by":"crossref","first-page":"101123","DOI":"10.1016\/j.isci.2020.101123","article-title":"Rare genetic diseases: nature's experiments on human development","volume":"23","author":"Lee","year":"2020","journal-title":"iScience"},{"issue":"13","key":"10.1016\/j.jacbts.2022.04.005_bib16","doi-asserted-by":"crossref","first-page":"1626","DOI":"10.1161\/CIRCULATIONAHA.111.059477","article-title":"The heart in Friedreich ataxia: definition of cardiomyopathy, disease severity, and correlation with neurological symptoms","volume":"125","author":"Weidemann","year":"2012","journal-title":"Circulation"},{"issue":"4","key":"10.1016\/j.jacbts.2022.04.005_bib17","doi-asserted-by":"crossref","first-page":"257","DOI":"10.1080\/14734220600913246","article-title":"Iron and iron-responsive proteins in the cardiomyopathy of Friedreich's ataxia","volume":"5","author":"Michael","year":"2006","journal-title":"Cerebellum"},{"issue":"1-2","key":"10.1016\/j.jacbts.2022.04.005_bib18","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/j.jns.2011.01.010","article-title":"Friedreich's ataxia: pathology, pathogenesis, and molecular genetics","volume":"303","author":"Koeppen","year":"2011","journal-title":"J\u00a0Neurol Sci"},{"issue":"7","key":"10.1016\/j.jacbts.2022.04.005_bib19","doi-asserted-by":"crossref","first-page":"749","DOI":"10.1161\/CIRCRESAHA.117.311059","article-title":"Hypertrophic cardiomyopathy: genetics, pathogenesis, clinical manifestations, diagnosis, and therapy","volume":"121","author":"Marian","year":"2017","journal-title":"Circ Res"},{"issue":"6","key":"10.1016\/j.jacbts.2022.04.005_bib20","doi-asserted-by":"crossref","first-page":"1230","DOI":"10.1093\/hmg\/ddr554","article-title":"A\u00a0TAT-frataxin fusion protein increases lifespan and cardiac function in a conditional Friedreich's ataxia mouse model","volume":"21","author":"Vyas","year":"2012","journal-title":"Hum Mol Genet"},{"issue":"3","key":"10.1016\/j.jacbts.2022.04.005_bib21","doi-asserted-by":"crossref","first-page":"366","DOI":"10.1007\/s00246-011-9943-6","article-title":"Cardiomyopathy of Friedreich's ataxia: use of mouse models to understand human disease and guide therapeutic development","volume":"32","author":"Payne","year":"2011","journal-title":"Pediatr Cardiol"},{"issue":"4","key":"10.1016\/j.jacbts.2022.04.005_bib22","doi-asserted-by":"crossref","first-page":"1622","DOI":"10.1016\/j.ijcard.2012.04.159","article-title":"Elevation of serum cardiac troponin I in a cross-sectional cohort of asymptomatic subjects with Friedreich ataxia","volume":"167","author":"Friedman","year":"2013","journal-title":"Int J Cardiol"},{"issue":"8","key":"10.1016\/j.jacbts.2022.04.005_bib23","doi-asserted-by":"crossref","first-page":"1834","DOI":"10.1007\/s00246-021-02675-1","article-title":"Myocardial perfusion reserve in children with Friedreich ataxia","volume":"42","author":"Hutchens","year":"2021","journal-title":"Pediatr Cardiol"},{"key":"10.1016\/j.jacbts.2022.04.005_bib24","doi-asserted-by":"crossref","first-page":"50","DOI":"10.1016\/j.ijcard.2015.05.074","article-title":"The cardiomyopathy in Friedreich's ataxia\u2014new biomarker for staging cardiac involvement","volume":"194","author":"Weidemann","year":"2015","journal-title":"Int J Cardiol"},{"issue":"9","key":"10.1016\/j.jacbts.2022.04.005_bib25","doi-asserted-by":"crossref","first-page":"1179","DOI":"10.1177\/0883073812448535","article-title":"Cardiomyopathy in Friedreich ataxia: clinical findings and research","volume":"27","author":"Payne","year":"2012","journal-title":"J\u00a0Child Neurol"},{"issue":"5","key":"10.1016\/j.jacbts.2022.04.005_bib26","doi-asserted-by":"crossref","first-page":"561","DOI":"10.1093\/eurheartj\/ehq443","article-title":"Impaired myocardial perfusion reserve and fibrosis in Friedreich ataxia: a mitochondrial cardiomyopathy with metabolic syndrome","volume":"32","author":"Raman","year":"2011","journal-title":"Eur Heart J"},{"issue":"1","key":"10.1016\/j.jacbts.2022.04.005_bib27","doi-asserted-by":"crossref","first-page":"1","DOI":"10.4330\/wjc.v11.i1.1","article-title":"Heart disease in Friedreich's ataxia","volume":"11","author":"Hanson","year":"2019","journal-title":"World J Cardiol"},{"issue":"1-2","key":"10.1016\/j.jacbts.2022.04.005_bib28","doi-asserted-by":"crossref","first-page":"29","DOI":"10.1016\/j.jns.2014.04.015","article-title":"Cross-sectional analysis of glucose metabolism in Friedreich ataxia","volume":"342","author":"Greeley","year":"2014","journal-title":"J\u00a0Neurol Sci"},{"issue":"suppl 1","key":"10.1016\/j.jacbts.2022.04.005_bib29","doi-asserted-by":"crossref","first-page":"94","DOI":"10.1111\/jnc.12216","article-title":"Diabetes in Friedreich ataxia","volume":"126","author":"Cnop","year":"2013","journal-title":"J\u00a0Neurochem"},{"issue":"1-2","key":"10.1016\/j.jacbts.2022.04.005_bib30","doi-asserted-by":"crossref","first-page":"46","DOI":"10.1016\/j.jns.2011.05.023","article-title":"Mortality in Friedreich ataxia","volume":"307","author":"Tsou","year":"2011","journal-title":"J\u00a0Neurol Sci"},{"key":"10.1016\/j.jacbts.2022.04.005_bib31","doi-asserted-by":"crossref","first-page":"29","DOI":"10.1016\/j.pediatrneurol.2021.01.004","article-title":"Ectopic burden via Holter monitors in Friedreich ataxia","volume":"117","author":"Mejia","year":"2021","journal-title":"Pediatr Neurol"},{"issue":"suppl 1","key":"10.1016\/j.jacbts.2022.04.005_bib32","doi-asserted-by":"crossref","first-page":"88","DOI":"10.1111\/jnc.12217","article-title":"Cardiomyopathy of Friedreich ataxia","volume":"126","author":"Weidemann","year":"2013","journal-title":"J\u00a0Neurochem"},{"issue":"2","key":"10.1016\/j.jacbts.2022.04.005_bib33","doi-asserted-by":"crossref","first-page":"181","DOI":"10.1038\/84818","article-title":"Mouse models for Friedreich ataxia exhibit cardiomyopathy, sensory nerve defect and Fe-S enzyme deficiency followed by intramitochondrial iron deposits","volume":"27","author":"Puccio","year":"2001","journal-title":"Nat Genet"},{"issue":"5","key":"10.1016\/j.jacbts.2022.04.005_bib34","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0178354","article-title":"Progressive mitochondrial protein lysine acetylation and heart failure in a model of Friedreich's ataxia cardiomyopathy","volume":"12","author":"Stram","year":"2017","journal-title":"PLoS One"},{"issue":"5","key":"10.1016\/j.jacbts.2022.04.005_bib35","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's ataxia","volume":"20","author":"Perdomini","year":"2014","journal-title":"Nat Med"},{"issue":"15-16","key":"10.1016\/j.jacbts.2022.04.005_bib36","doi-asserted-by":"crossref","first-page":"819","DOI":"10.1089\/hum.2019.363","article-title":"Stress-Induced Mouse Model of the Cardiac Manifestations of Friedreich's Ataxia Corrected by AAV-mediated Gene Therapy","volume":"31","author":"Salami","year":"2020","journal-title":"Hum Gene Ther"},{"issue":"1","key":"10.1016\/j.jacbts.2022.04.005_bib37","doi-asserted-by":"crossref","first-page":"49","DOI":"10.2217\/nmt.15.73","article-title":"Emerging therapies in Friedreich's ataxia","volume":"6","author":"Aranca","year":"2016","journal-title":"Neurodegener Dis Manag"},{"issue":"4","key":"10.1016\/j.jacbts.2022.04.005_bib38","doi-asserted-by":"crossref","first-page":"1032","DOI":"10.1007\/s13311-019-00764-x","article-title":"Molecular mechanisms and therapeutics for the GAA\u00b7TTC expansion disease Friedreich ataxia","volume":"16","author":"Gottesfeld","year":"2019","journal-title":"Neurotherapeutics"},{"issue":"4","key":"10.1016\/j.jacbts.2022.04.005_bib39","doi-asserted-by":"crossref","first-page":"558","DOI":"10.1001\/archneur.64.4.558","article-title":"Neurological, cardiological, and oculomotor progression in 104 patients with Friedreich ataxia during long-term follow-up","volume":"64","author":"Ribai","year":"2007","journal-title":"Arch Neurol"},{"issue":"1","key":"10.1016\/j.jacbts.2022.04.005_bib40","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1007\/s12311-010-0212-7","article-title":"Combined therapy with idebenone and deferiprone in patients with Friedreich's ataxia","volume":"10","author":"Velasco-Sanchez","year":"2011","journal-title":"Cerebellum"},{"issue":"4","key":"10.1016\/j.jacbts.2022.04.005_bib41","doi-asserted-by":"crossref","first-page":"463","DOI":"10.1093\/hmg\/ddi042","article-title":"Friedreich ataxia: the oxidative stress paradox","volume":"14","author":"Seznec","year":"2005","journal-title":"Hum Mol Genet"},{"key":"10.1016\/j.jacbts.2022.04.005_bib42","first-page":"CD007791","article-title":"Pharmacological treatments for Friedreich ataxia","volume":"8","author":"Kearney","year":"2016","journal-title":"Cochrane Database Syst Rev"},{"issue":"21","key":"10.1016\/j.jacbts.2022.04.005_bib43","doi-asserted-by":"crossref","first-page":"2512","DOI":"10.1161\/01.CIR.0000017264.66561.3D","article-title":"Losartan-dependent regression of myocardial fibrosis is associated with reduction of left ventricular chamber stiffness in hypertensive patients","volume":"105","author":"Diez","year":"2002","journal-title":"Circulation"},{"issue":"9","key":"10.1016\/j.jacbts.2022.04.005_bib44","doi-asserted-by":"crossref","first-page":"684","DOI":"10.1002\/acn3.332","article-title":"Progression of Friedreich ataxia: quantitative characterization over 5 years","volume":"3","author":"Patel","year":"2016","journal-title":"Ann Clin Transl Neurol"},{"issue":"11","key":"10.1016\/j.jacbts.2022.04.005_bib45","doi-asserted-by":"crossref","first-page":"1334","DOI":"10.1001\/jamaneurol.2015.1855","article-title":"A\u00a022-year follow-up study of long-term cardiac outcome and predictors of survival in Friedreich ataxia","volume":"72","author":"Pousset","year":"2015","journal-title":"JAMA Neurol"},{"issue":"3","key":"10.1016\/j.jacbts.2022.04.005_bib46","doi-asserted-by":"crossref","first-page":"483","DOI":"10.1001\/archinte.1986.00360150081010","article-title":"Five-year follow-up of Friedreich's ataxia cardiomyopathy","volume":"146","author":"Hawley","year":"1986","journal-title":"Arch Intern Med"},{"issue":"10","key":"10.1016\/j.jacbts.2022.04.005_bib47","doi-asserted-by":"crossref","first-page":"895","DOI":"10.1016\/S0002-9149(97)89194-1","article-title":"The varying evolution of Friedreich's ataxia cardiomyopathy","volume":"77","author":"Casazza","year":"1996","journal-title":"Am J Cardiol"},{"issue":"208","key":"10.1016\/j.jacbts.2022.04.005_bib48","first-page":"489","article-title":"The heart disease of Friedreich's ataxia: a clinical and electrocardiographic study of 115 patients, with an analysis of serial electrocardiographic changes in 30 cases","volume":"52","author":"Harding","year":"1983","journal-title":"Q\u00a0J Med"},{"key":"10.1016\/j.jacbts.2022.04.005_bib49","doi-asserted-by":"crossref","first-page":"259","DOI":"10.1016\/j.ijcard.2021.02.032","article-title":"Prognostic value of longitudinal strain and ejection fraction in Friedreich ataxia","volume":"330","author":"Legrand","year":"2021","journal-title":"Int J Cardiol"},{"issue":"5619","key":"10.1016\/j.jacbts.2022.04.005_bib50","doi-asserted-by":"crossref","first-page":"649","DOI":"10.1136\/bmj.3.5619.649","article-title":"Study of fatal cases of Friedreich's ataxia","volume":"3","author":"Hewer","year":"1968","journal-title":"Br Med J"},{"issue":"6","key":"10.1016\/j.jacbts.2022.04.005_bib51","doi-asserted-by":"crossref","first-page":"1370","DOI":"10.1016\/S0735-1097(86)80159-0","article-title":"Cardiac involvement in Friedreich's ataxia: a clinical study of 75 patients","volume":"7","author":"Child","year":"1986","journal-title":"J\u00a0Am Coll Cardiol"},{"issue":"6","key":"10.1016\/j.jacbts.2022.04.005_bib52","doi-asserted-by":"crossref","first-page":"776","DOI":"10.1016\/j.jacc.2017.04.025","volume":"70","author":"Yancy","year":"2017","journal-title":"J\u00a0Am Coll Cardiol"},{"issue":"3","key":"10.1016\/j.jacbts.2022.04.005_bib53","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0246633","article-title":"Pre-clinical left ventricular myocardial remodeling in patients with Friedreich's ataxia: a cardiac MRI study","volume":"16","author":"Takazaki","year":"2021","journal-title":"PLoS One"},{"issue":"11","key":"10.1016\/j.jacbts.2022.04.005_bib54","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0225147","article-title":"Left ventricular structural and functional changes in Friedreich ataxia\u2014relationship with body size, sex, age and genetic severity","volume":"14","author":"Peverill","year":"2019","journal-title":"PLoS One"},{"issue":"12","key":"10.1016\/j.jacbts.2022.04.005_bib55","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0209410","article-title":"Differences in the determinants of right ventricular and regional left ventricular long-axis dysfunction in Friedreich ataxia","volume":"13","author":"Peverill","year":"2018","journal-title":"PLoS One"},{"issue":"12","key":"10.1016\/j.jacbts.2022.04.005_bib56","doi-asserted-by":"crossref","first-page":"986","DOI":"10.1093\/eurheartj\/ehy798","article-title":"Clinical presentation and survival of childhood hypertrophic cardiomyopathy: a retrospective study in United Kingdom","volume":"40","author":"Norrish","year":"2019","journal-title":"Eur Heart J"},{"issue":"3","key":"10.1016\/j.jacbts.2022.04.005_bib57","doi-asserted-by":"crossref","first-page":"401","DOI":"10.1016\/j.amjcard.2011.09.025","article-title":"Analysis of echocardiograms in a large heterogeneous cohort of patients with Friedreich ataxia","volume":"109","author":"Regner","year":"2012","journal-title":"Am J Cardiol"},{"issue":"9","key":"10.1016\/j.jacbts.2022.04.005_bib58","doi-asserted-by":"crossref","first-page":"1187","DOI":"10.1177\/0883073812448461","article-title":"Cross-sectional analysis of electrocardiograms in a large heterogeneous cohort of Friedreich ataxia subjects","volume":"27","author":"Schadt","year":"2012","journal-title":"J\u00a0Child Neurol"},{"issue":"7","key":"10.1016\/j.jacbts.2022.04.005_bib59","doi-asserted-by":"crossref","first-page":"782","DOI":"10.1016\/j.echo.2011.04.004","article-title":"Early changes in left ventricular long-axis function in Friedreich ataxia: relation with the FXN gene mutation and cardiac structural change","volume":"24","author":"Mottram","year":"2011","journal-title":"J\u00a0Am Soc Echocardiogr"},{"issue":"7","key":"10.1016\/j.jacbts.2022.04.005_bib60","doi-asserted-by":"crossref","first-page":"846","DOI":"10.1002\/mds.22864","article-title":"Analysis of the factors influencing the cardiac phenotype in Friedreich's ataxia","volume":"25","author":"Rajagopalan","year":"2010","journal-title":"Mov Disord"},{"issue":"11","key":"10.1016\/j.jacbts.2022.04.005_bib61","doi-asserted-by":"crossref","first-page":"1615","DOI":"10.1002\/mds.21590","article-title":"Cardiomyopathy in Friedreich's ataxia-assessment by cardiac MRI","volume":"22","author":"Meyer","year":"2007","journal-title":"Mov Disord"},{"issue":"4","key":"10.1016\/j.jacbts.2022.04.005_bib62","doi-asserted-by":"crossref","first-page":"621","DOI":"10.1001\/archneur.62.4.621","article-title":"Antioxidant treatment of patients with Friedreich ataxia: four-year follow-up","volume":"62","author":"Hart","year":"2005","journal-title":"Arch Neurol"},{"issue":"1","key":"10.1016\/j.jacbts.2022.04.005_bib63","doi-asserted-by":"crossref","first-page":"111","DOI":"10.1016\/S0008-6363(01)00357-1","article-title":"Cardiac energetics are abnormal in Friedreich ataxia patients in the absence of cardiac dysfunction and hypertrophy: an in\u00a0vivo 31P magnetic resonance spectroscopy study","volume":"52","author":"Lodi","year":"2001","journal-title":"Cardiovasc Res"},{"issue":"5","key":"10.1016\/j.jacbts.2022.04.005_bib64","doi-asserted-by":"crossref","first-page":"590","DOI":"10.1002\/ana.1001","article-title":"Antioxidant treatment improves in\u00a0vivo cardiac and skeletal muscle bioenergetics in patients with Friedreich's ataxia","volume":"49","author":"Lodi","year":"2001","journal-title":"Ann Neurol"},{"issue":"11","key":"10.1016\/j.jacbts.2022.04.005_bib65","doi-asserted-by":"crossref","first-page":"1276","DOI":"10.1161\/01.CIR.102.11.1276","article-title":"Echocardiographic characterization of cardiomyopathy in Friedreich's ataxia with tissue Doppler echocardiographically derived myocardial velocity gradients","volume":"102","author":"Dutka","year":"2000","journal-title":"Circulation"},{"issue":"2","key":"10.1016\/j.jacbts.2022.04.005_bib66","doi-asserted-by":"crossref","first-page":"141","DOI":"10.1136\/hrt.81.2.141","article-title":"Marked variation in the cardiomyopathy associated with Friedreich's ataxia","volume":"81","author":"Dutka","year":"1999","journal-title":"Heart"},{"issue":"5","key":"10.1016\/j.jacbts.2022.04.005_bib67","doi-asserted-by":"crossref","first-page":"340","DOI":"10.1016\/j.nmd.2010.02.019","article-title":"Friedreich ataxia presenting as sudden cardiac death in childhood: clinical, genetic and pathological correlation, with implications for genetic testing and counselling","volume":"20","author":"Quercia","year":"2010","journal-title":"Neuromuscul Disord"},{"issue":"20","key":"10.1016\/j.jacbts.2022.04.005_bib68","doi-asserted-by":"crossref","first-page":"11492","DOI":"10.1073\/pnas.96.20.11492","article-title":"Deficit of in\u00a0vivo mitochondrial ATP production in patients with Friedreich ataxia","volume":"96","author":"Lodi","year":"1999","journal-title":"Proc Natl Acad Sci U S A"},{"issue":"9386","key":"10.1016\/j.jacbts.2022.04.005_bib69","doi-asserted-by":"crossref","first-page":"767","DOI":"10.1016\/S0140-6736(03)14283-3","article-title":"CHARM Investigators and Committees. Effects of candesartan in patients with chronic heart failure and reduced left-ventricular systolic function taking angiotensin-converting-enzyme inhibitors: the CHARM-Added trial","volume":"362","author":"McMurray","year":"2003","journal-title":"Lancet"},{"issue":"24","key":"10.1016\/j.jacbts.2022.04.005_bib70","doi-asserted-by":"crossref","first-page":"3738","DOI":"10.1161\/CIRCULATIONAHA.105.561423","article-title":"CHARM Investigators. Influence of ejection fraction on cardiovascular outcomes in a broad spectrum of heart failure patients","volume":"112","author":"Solomon","year":"2005","journal-title":"Circulation"},{"issue":"3","key":"10.1016\/j.jacbts.2022.04.005_bib71","doi-asserted-by":"crossref","first-page":"296","DOI":"10.1016\/j.jcmg.2016.12.003","article-title":"Use of imaging endpoints in clinical trials","volume":"10","author":"Gillam","year":"2017","journal-title":"J\u00a0Am Coll Cardiol Img"},{"issue":"11","key":"10.1016\/j.jacbts.2022.04.005_bib72","doi-asserted-by":"crossref","first-page":"728","DOI":"10.1038\/jhh.2009.16","article-title":"Indexation of left ventricular mass to body surface area and height to allometric power of 2.7: is the difference limited to obese hypertensives?","volume":"23","author":"Cuspidi","year":"2009","journal-title":"J\u00a0Hum Hypertens"},{"issue":"8","key":"10.1016\/j.jacbts.2022.04.005_bib73","doi-asserted-by":"crossref","first-page":"1079","DOI":"10.1093\/ajh\/hpu019","article-title":"Differential value of left ventricular mass index and wall thickness in predicting cardiovascular prognosis: data from the PAMELA population","volume":"27","author":"Cuspidi","year":"2014","journal-title":"Am J Hypertens"},{"issue":"5","key":"10.1016\/j.jacbts.2022.04.005_bib74","doi-asserted-by":"crossref","first-page":"1251","DOI":"10.1016\/0735-1097(92)90385-Z","article-title":"Left ventricular mass and body size in normotensive children and adults: assessment of allometric relations and impact of overweight","volume":"20","author":"de Simone","year":"1992","journal-title":"J\u00a0Am Coll Cardiol"},{"issue":"1","key":"10.1016\/j.jacbts.2022.04.005_bib75","doi-asserted-by":"crossref","first-page":"71","DOI":"10.1111\/echo.12613","article-title":"Speckle strain echocardiography predicts outcome in patients with heart failure with both depressed and preserved left ventricular ejection fraction","volume":"32","author":"Stampehl","year":"2015","journal-title":"Echocardiography"},{"issue":"12","key":"10.1016\/j.jacbts.2022.04.005_bib76","article-title":"Phenotyping heart failure according to the longitudinal ejection fraction change: myocardial strain, predictors, and outcomes","volume":"9","author":"Park","year":"2020","journal-title":"J\u00a0Am Heart Assoc"},{"issue":"1","key":"10.1016\/j.jacbts.2022.04.005_bib77","doi-asserted-by":"crossref","first-page":"50","DOI":"10.1111\/echo.12287","article-title":"Longitudinal strain in Friedreich Ataxia: a potential marker for early left ventricular dysfunction","volume":"31","author":"St John Sutton","year":"2014","journal-title":"Echocardiography"},{"issue":"23","key":"10.1016\/j.jacbts.2022.04.005_bib78","doi-asserted-by":"crossref","first-page":"2614","DOI":"10.1016\/j.jacc.2009.11.011","article-title":"ACCF\/ACR\/AHA\/NASCI\/SCMR 2010 expert consensus document on cardiovascular magnetic resonance: a report of the American College of Cardiology Foundation Task Force on Expert Consensus Documents","volume":"55","author":"Hundley","year":"2010","journal-title":"J\u00a0Am Coll Cardiol"},{"issue":"6","key":"10.1016\/j.jacbts.2022.04.005_bib79","doi-asserted-by":"crossref","first-page":"692","DOI":"10.1136\/hrt.59.6.692","article-title":"Biventricular function in Friedreich's ataxia: a radionuclide angiographic study","volume":"59","author":"Palagi","year":"1988","journal-title":"Br Heart J"},{"issue":"5","key":"10.1016\/j.jacbts.2022.04.005_bib80","doi-asserted-by":"crossref","first-page":"622","DOI":"10.1016\/S0002-9149(02)03325-8","article-title":"Quantification of regional right and left ventricular function by ultrasonic strain rate and strain indexes in Friedreich's ataxia","volume":"91","author":"Weidemann","year":"2003","journal-title":"Am J Cardiol"},{"issue":"6","key":"10.1016\/j.jacbts.2022.04.005_bib81","doi-asserted-by":"crossref","first-page":"FSO398","DOI":"10.2144\/fsoa-2019-0026","article-title":"The current state of biomarker research for Friedreich's ataxia: a report from the 2018 FARA biomarker meeting","volume":"5","author":"Blair","year":"2019","journal-title":"Future Sci OA"},{"issue":"4","key":"10.1016\/j.jacbts.2022.04.005_bib82","doi-asserted-by":"crossref","first-page":"S172","DOI":"10.1016\/j.healun.2016.01.479","article-title":"Cardiac serum biomarkers in Friedreich ataxia may reflect fibrosis, myocyte injury, and degree of hypertrophy","volume":"35","author":"Bui","year":"2016","journal-title":"J\u00a0Heart Lung Transplant"},{"issue":"4","key":"10.1016\/j.jacbts.2022.04.005_bib83","doi-asserted-by":"crossref","first-page":"305","DOI":"10.14503\/THIJ-14-4198","article-title":"Serum versus imaging biomarkers in Friedreich ataxia to indicate left ventricular remodeling and outcomes","volume":"43","author":"Mehta","year":"2016","journal-title":"Tex Heart Inst J"},{"issue":"6","key":"10.1016\/j.jacbts.2022.04.005_bib84","first-page":"1630","article-title":"Significance of NT-proBNP and high-sensitivity troponin in Friedreich ataxia","volume":"9","author":"Legrand","year":"2020","journal-title":"J\u00a0Clin Med"},{"issue":"7","key":"10.1016\/j.jacbts.2022.04.005_bib85","doi-asserted-by":"crossref","first-page":"604","DOI":"10.1111\/j.1365-2044.2011.06763.x","article-title":"Outcomes in vascular surgical patients with isolated postoperative troponin leak: a meta-analysis","volume":"66","author":"Redfern","year":"2011","journal-title":"Anaesthesia"},{"issue":"4","key":"10.1016\/j.jacbts.2022.04.005_bib86","doi-asserted-by":"crossref","first-page":"372","DOI":"10.1055\/s-2005-916151","article-title":"Pulmonary arterial hypertension: evaluation of the newly diagnosed patient","volume":"26","author":"Torbicki","year":"2005","journal-title":"Semin Respir Crit Care Med"},{"issue":"3","key":"10.1016\/j.jacbts.2022.04.005_bib87","doi-asserted-by":"crossref","first-page":"634","DOI":"10.1161\/ATVBAHA.109.201210","article-title":"Subclinical myocardial necrosis and cardiovascular risk in stable patients undergoing elective cardiac evaluation","volume":"30","author":"Tang","year":"2010","journal-title":"Arterioscler Thromb Vasc Biol"},{"issue":"5","key":"10.1016\/j.jacbts.2022.04.005_bib88","doi-asserted-by":"crossref","first-page":"1877","DOI":"10.1378\/chest.125.5.1877","article-title":"Common causes of troponin elevations in the absence of acute myocardial infarction: incidence and clinical significance","volume":"125","author":"Roongsritong","year":"2004","journal-title":"Chest"},{"issue":"1","key":"10.1016\/j.jacbts.2022.04.005_bib89","doi-asserted-by":"crossref","first-page":"15","DOI":"10.1186\/1532-429X-10-15","article-title":"Myocardial ischemia in the absence of epicardial coronary artery disease in Friedreich's ataxia","volume":"10","author":"Raman","year":"2008","journal-title":"J\u00a0Cardiovasc Magn Reson"},{"key":"10.1016\/j.jacbts.2022.04.005_bib90","doi-asserted-by":"crossref","first-page":"100094","DOI":"10.1016\/j.mcpro.2021.100094","article-title":"Reverse phase protein array reveals correlation of retinoic acid metabolism with cardiomyopathy in Friedreich's ataxia","volume":"20","author":"Napierala","year":"2021","journal-title":"Mol Cell Proteomics"},{"issue":"2","key":"10.1016\/j.jacbts.2022.04.005_bib91","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0192779","article-title":"Low apolipoprotein A-I levels in Friedreich's ataxia and in frataxin-deficient cells: Implications for therapy","volume":"13","author":"Wang","year":"2018","journal-title":"PLoS One"},{"issue":"11","key":"10.1016\/j.jacbts.2022.04.005_bib92","doi-asserted-by":"crossref","first-page":"1719","DOI":"10.1212\/WNL.55.11.1719","article-title":"Oxidative stress in patients with Friedreich ataxia","volume":"55","author":"Schulz","year":"2000","journal-title":"Neurology"},{"issue":"9","key":"10.1016\/j.jacbts.2022.04.005_bib93","doi-asserted-by":"crossref","first-page":"1238","DOI":"10.1161\/CIRCRESAHA.117.311002","article-title":"Cardiovascular metabolomics","volume":"122","author":"McGarrah","year":"2018","journal-title":"Circ Res"},{"issue":"6","key":"10.1016\/j.jacbts.2022.04.005_bib94","doi-asserted-by":"crossref","first-page":"1239","DOI":"10.1002\/acn3.51352","article-title":"Scoliosis in Friedreich's ataxia: longitudinal characterization in a large heterogeneous cohort","volume":"8","author":"Rummey","year":"2021","journal-title":"Ann Clin Transl Neurol"},{"issue":"2","key":"10.1016\/j.jacbts.2022.04.005_bib95","doi-asserted-by":"crossref","first-page":"234","DOI":"10.1097\/BPO.0b013e318164fa79","article-title":"Friedreich's ataxia and scoliosis: the experience at two institutions","volume":"28","author":"Milbrandt","year":"2008","journal-title":"J\u00a0Pediatr Orthop"},{"issue":"5","key":"10.1016\/j.jacbts.2022.04.005_bib96","doi-asserted-by":"crossref","first-page":"684","DOI":"10.1302\/0301-620X.94B5.28391","article-title":"Scoliosis in patients with Friedreich's ataxia","volume":"94","author":"Tsirikos","year":"2012","journal-title":"J\u00a0Bone Joint Surg Br"},{"issue":"5","key":"10.1016\/j.jacbts.2022.04.005_bib97","doi-asserted-by":"crossref","first-page":"455","DOI":"10.1097\/HCO.0000000000000556","article-title":"An introduction to left ventricular strain","volume":"33","author":"Narang","year":"2018","journal-title":"Curr Opin Cardiol"},{"issue":"8","key":"10.1016\/j.jacbts.2022.04.005_bib98","doi-asserted-by":"crossref","first-page":"1219","DOI":"10.1093\/hmg\/9.8.1219","article-title":"Inactivation of the Friedreich ataxia mouse gene leads to early embryonic lethality without iron accumulation","volume":"9","author":"Cossee","year":"2000","journal-title":"Hum Mol Genet"},{"issue":"2","key":"10.1016\/j.jacbts.2022.04.005_bib99","doi-asserted-by":"crossref","first-page":"167","DOI":"10.1136\/adc.84.2.167","article-title":"Friedreich's ataxia presenting after cardiac transplantation","volume":"84","author":"Leonard","year":"2001","journal-title":"Arch Dis Child"},{"issue":"10","key":"10.1016\/j.jacbts.2022.04.005_bib100","doi-asserted-by":"crossref","first-page":"672","DOI":"10.1016\/j.cardfail.2017.09.012","article-title":"The subclinical cardiomyopathy of Friedreich's ataxia in a pediatric population","volume":"24","author":"Plehn","year":"2018","journal-title":"J\u00a0Card Fail"},{"issue":"7","key":"10.1016\/j.jacbts.2022.04.005_bib101","doi-asserted-by":"crossref","first-page":"1044","DOI":"10.1016\/j.apmr.2010.04.007","article-title":"Exercise capacity and idebenone intervention in children and adolescents with Friedreich ataxia","volume":"91","author":"Drinkard","year":"2010","journal-title":"Arch Phys Med Rehabil"},{"issue":"3","key":"10.1016\/j.jacbts.2022.04.005_bib102","doi-asserted-by":"crossref","first-page":"306","DOI":"10.1007\/s00246-008-9305-1","article-title":"The longitudinal course of cardiomyopathy in Friedreich's ataxia during childhood","volume":"30","author":"Kipps","year":"2009","journal-title":"Pediatr Cardiol"},{"issue":"4","key":"10.1016\/j.jacbts.2022.04.005_bib103","doi-asserted-by":"crossref","first-page":"467","DOI":"10.1080\/10715760290021333","article-title":"Heart hypertrophy and function are improved by idebenone in Friedreich's ataxia","volume":"36","author":"Rustin","year":"2002","journal-title":"Free Radic Res"},{"issue":"4","key":"10.1016\/j.jacbts.2022.04.005_bib104","doi-asserted-by":"crossref","first-page":"346","DOI":"10.1136\/heart.87.4.346","article-title":"Idebenone and reduced cardiac hypertrophy in Friedreich's ataxia","volume":"87","author":"Hausse","year":"2002","journal-title":"Heart"},{"issue":"9177","key":"10.1016\/j.jacbts.2022.04.005_bib105","doi-asserted-by":"crossref","first-page":"477","DOI":"10.1016\/S0140-6736(99)01341-0","article-title":"Effect of idebenone on cardiomyopathy in Friedreich's ataxia: a preliminary study","volume":"354","author":"Rustin","year":"1999","journal-title":"Lancet"},{"issue":"2","key":"10.1016\/j.jacbts.2022.04.005_bib106","doi-asserted-by":"crossref","first-page":"72","DOI":"10.1055\/s-2007-973463","article-title":"Clinical and genetic correlate in childhood onset Friedreich ataxia","volume":"30","author":"Alikasifoglu","year":"1999","journal-title":"Neuropediatrics"},{"issue":"3","key":"10.1016\/j.jacbts.2022.04.005_bib107","doi-asserted-by":"crossref","first-page":"458","DOI":"10.1002\/ajmg.1320340327","article-title":"Prenatal diagnosis of Friedreich ataxia","volume":"34","author":"Wallis","year":"1989","journal-title":"Am J Med Genet"},{"issue":"1","key":"10.1016\/j.jacbts.2022.04.005_bib108","doi-asserted-by":"crossref","first-page":"15788","DOI":"10.1038\/s41598-020-72884-w","article-title":"Extra-mitochondrial mouse frataxin and its implications for mouse models of Friedreich's ataxia","volume":"10","author":"Weng","year":"2020","journal-title":"Sci Rep"},{"issue":"4","key":"10.1016\/j.jacbts.2022.04.005_bib109","doi-asserted-by":"crossref","first-page":"739","DOI":"10.1002\/path.1700580414","article-title":"Myocarditis in Friedreich's ataxia","volume":"58","author":"Russell","year":"1946","journal-title":"J\u00a0Pathol Bacteriol"},{"issue":"1","key":"10.1016\/j.jacbts.2022.04.005_bib110","doi-asserted-by":"crossref","first-page":"5","DOI":"10.1136\/hrt.31.1.5","article-title":"The heart in Friedreich's ataxia","volume":"31","author":"Hewer","year":"1969","journal-title":"Br Heart J"},{"issue":"1","key":"10.1016\/j.jacbts.2022.04.005_bib111","first-page":"39","article-title":"Microvascular pathology in Friedreich cardiomyopathy","volume":"35","author":"Koeppen","year":"2020","journal-title":"Histol Histopathol"},{"issue":"3","key":"10.1016\/j.jacbts.2022.04.005_bib112","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0116396","article-title":"The pathogenesis of cardiomyopathy in Friedreich ataxia","volume":"10","author":"Koeppen","year":"2015","journal-title":"PLoS One"},{"key":"10.1016\/j.jacbts.2022.04.005_bib113","doi-asserted-by":"crossref","first-page":"446","DOI":"10.1136\/hrt.57.5.446","article-title":"Coronary disease, cardioneuropathy, and conduction system abnormalities in the cardiomyopathy of Friedreich's ataxia","volume":"57","author":"James","year":"1987","journal-title":"Br Heart J"},{"issue":"4","key":"10.1016\/j.jacbts.2022.04.005_bib114","doi-asserted-by":"crossref","first-page":"323","DOI":"10.1007\/s00109-009-0565-x","article-title":"The ins and outs of mitochondrial iron-loading: the metabolic defect in Friedreich's ataxia","volume":"88","author":"Richardson","year":"2010","journal-title":"J\u00a0Mol Med (Berl)"},{"issue":"12","key":"10.1016\/j.jacbts.2022.04.005_bib115","doi-asserted-by":"crossref","DOI":"10.1371\/journal.pone.0189990","article-title":"Somatic instability of the expanded GAA repeats in Friedreich's ataxia","volume":"12","author":"Long","year":"2017","journal-title":"PLoS One"},{"issue":"1","key":"10.1016\/j.jacbts.2022.04.005_bib116","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/j.ygeno.2007.04.001","article-title":"Somatic instability of the expanded GAA triplet-repeat sequence in Friedreich ataxia progresses throughout life","volume":"90","author":"De Biase","year":"2007","journal-title":"Genomics"},{"issue":"5","key":"10.1016\/j.jacbts.2022.04.005_bib117","doi-asserted-by":"crossref","DOI":"10.1073\/pnas.2013080118","article-title":"Replication-independent instability of Friedreich's ataxia GAA repeats during chronological aging","volume":"118","author":"Neil","year":"2021","journal-title":"Proc Natl Acad Sci U S A"},{"issue":"5","key":"10.1016\/j.jacbts.2022.04.005_bib118","doi-asserted-by":"crossref","first-page":"848","DOI":"10.1016\/j.yjmcc.2011.07.001","article-title":"A\u00a0polymorphic miR-155 binding site in AGTR1 is associated with cardiac hypertrophy in Friedreich ataxia","volume":"51","author":"Kelly","year":"2011","journal-title":"J\u00a0Mol Cell Cardiol"},{"key":"10.1016\/j.jacbts.2022.04.005_bib119","doi-asserted-by":"crossref","first-page":"100266","DOI":"10.1016\/j.imu.2019.100266","article-title":"Analysis of Friedreich's ataxia patient clinical data reveals importance of accurate GAA repeat determination in disease prognosis and gender differences in cardiac measures","volume":"17","author":"Ghorbani","year":"2019","journal-title":"Inform Med Unlocked"},{"key":"10.1016\/j.jacbts.2022.04.005_bib120","first-page":"689798","article-title":"Comparative (computational) analysis of the DNA methylation status of trinucleotide repeat expansion diseases","volume":"2013","author":"Ghorbani","year":"2013","journal-title":"J\u00a0Nucleic Acids"},{"issue":"23","key":"10.1016\/j.jacbts.2022.04.005_bib121","doi-asserted-by":"crossref","first-page":"3818","DOI":"10.1093\/hmg\/ddaa267","article-title":"Methylated and unmethylated epialleles support variegated epigenetic silencing in Friedreich ataxia","volume":"29","author":"Rodden","year":"2021","journal-title":"Hum Mol Genet"},{"issue":"11","key":"10.1016\/j.jacbts.2022.04.005_bib122","doi-asserted-by":"crossref","first-page":"1140","DOI":"10.1056\/NEJMra063052","article-title":"The failing heart\u2014an engine out of fuel","volume":"356","author":"Neubauer","year":"2007","journal-title":"N\u00a0Engl J Med"},{"issue":"suppl 2","key":"10.1016\/j.jacbts.2022.04.005_bib123","doi-asserted-by":"crossref","first-page":"S145","DOI":"10.2337\/dc11-s209","article-title":"Heart in diabetes: a microvascular disease","volume":"34","author":"Laakso","year":"2011","journal-title":"Diabetes Care"},{"issue":"10","key":"10.1016\/j.jacbts.2022.04.005_bib124","doi-asserted-by":"crossref","first-page":"2054","DOI":"10.2337\/dbi20-0024","article-title":"Metabolic flexibility of mitochondria plays a key role in balancing glucose and fatty acid metabolism in the diabetic heart","volume":"69","author":"Gambardella","year":"2020","journal-title":"Diabetes"},{"key":"10.1016\/j.jacbts.2022.04.005_bib125","doi-asserted-by":"crossref","first-page":"68","DOI":"10.3389\/fcvm.2018.00068","article-title":"Loss of metabolic flexibility in the failing heart","volume":"5","author":"Karwi","year":"2018","journal-title":"Front Cardiovasc Med"},{"issue":"6","key":"10.1016\/j.jacbts.2022.04.005_bib126","doi-asserted-by":"crossref","first-page":"E452","DOI":"10.1152\/ajpendo.00437.2015","article-title":"Assessment of myocardial metabolic flexibility and work efficiency in human type 2 diabetes using 16-[18F]fluoro-4-thiapalmitate, a novel PET fatty acid tracer","volume":"310","author":"Mather","year":"2016","journal-title":"Am J Physiol Endocrinol Metab"},{"key":"10.1016\/j.jacbts.2022.04.005_bib127","doi-asserted-by":"crossref","first-page":"7","DOI":"10.1016\/j.ebiom.2017.03.020","article-title":"Mitochondria in cell senescence: is mitophagy the weakest link?","volume":"21","author":"Korolchuk","year":"2017","journal-title":"EBioMedicine"},{"issue":"1","key":"10.1016\/j.jacbts.2022.04.005_bib128","doi-asserted-by":"crossref","first-page":"6095","DOI":"10.1038\/s41598-020-62952-6","article-title":"Long-term voluntary running prevents the onset of symptomatic Friedreich's ataxia in mice","volume":"10","author":"Zhao","year":"2020","journal-title":"Sci Rep"},{"issue":"14","key":"10.1016\/j.jacbts.2022.04.005_bib129","doi-asserted-by":"crossref","first-page":"1810","DOI":"10.1016\/j.cub.2015.05.059","article-title":"Iron-starvation-induced mitophagy mediates lifespan extension upon mitochondrial stress in C.\u00a0elegans","volume":"25","author":"Schiavi","year":"2015","journal-title":"Curr Biol"},{"key":"10.1016\/j.jacbts.2022.04.005_bib130","doi-asserted-by":"crossref","first-page":"35","DOI":"10.1016\/j.neuint.2017.08.002","article-title":"Mitochondrial dysfunction in the neuro-degenerative and cardio-degenerative disease, Friedreich's ataxia","volume":"117","author":"Chiang","year":"2018","journal-title":"Neurochem Int"},{"issue":"16","key":"10.1016\/j.jacbts.2022.04.005_bib131","doi-asserted-by":"crossref","first-page":"16106","DOI":"10.1074\/jbc.M414285200","article-title":"pH-dependent substrate preference of pig heart lipoamide dehydrogenase varies with oligomeric state: response to mitochondrial matrix acidification","volume":"280","author":"Klyachko","year":"2005","journal-title":"J\u00a0Biol Chem"},{"issue":"15","key":"10.1016\/j.jacbts.2022.04.005_bib132","doi-asserted-by":"crossref","first-page":"6158","DOI":"10.1073\/pnas.0610618104","article-title":"Cryptic proteolytic activity of dihydrolipoamide dehydrogenase","volume":"104","author":"Babady","year":"2007","journal-title":"Proc Natl Acad Sci U S A"},{"issue":"6","key":"10.1016\/j.jacbts.2022.04.005_bib133","doi-asserted-by":"crossref","first-page":"1284","DOI":"10.1016\/j.bbamcr.2014.11.018","article-title":"Fe-S proteins that regulate gene expression","volume":"1853","author":"Mettert","year":"2015","journal-title":"Biochim Biophys Acta"},{"issue":"1","key":"10.1016\/j.jacbts.2022.04.005_bib134","doi-asserted-by":"crossref","first-page":"9","DOI":"10.1039\/C7MT00180K","article-title":"Iron-sulfur cluster biosynthesis and trafficking\u2014impact on human disease conditions","volume":"10","author":"Wachnowsky","year":"2018","journal-title":"Metallomics"},{"key":"10.1016\/j.jacbts.2022.04.005_bib135","doi-asserted-by":"crossref","first-page":"101529","DOI":"10.1016\/j.scr.2019.101529","article-title":"Excision of the expanded GAA repeats corrects cardiomyopathy phenotypes of iPSC-derived Friedreich's ataxia cardiomyocytes","volume":"40","author":"Li","year":"2019","journal-title":"Stem Cell Res"},{"key":"10.1016\/j.jacbts.2022.04.005_bib136","doi-asserted-by":"crossref","DOI":"10.7554\/eLife.30054","article-title":"Inducible and reversible phenotypes in a novel mouse model\u00a0of Friedreich's Ataxia","volume":"6","author":"Chandran","year":"2017","journal-title":"eLife"},{"issue":"17","key":"10.1016\/j.jacbts.2022.04.005_bib137","doi-asserted-by":"crossref","first-page":"e272","DOI":"10.1161\/CIRCULATIONAHA.112.115287","article-title":"Letter by Peverill regarding article, \"The heart in Friedreich ataxia: definition of cardiomyopathy, disease severity, and correlation with neurological symptoms","volume":"126","author":"Peverill","year":"2012","journal-title":"Circulation"}],"container-title":["JACC: Basic to Translational Science"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S2452302X22001425?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S2452302X22001425?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2025,11,11]],"date-time":"2025-11-11T00:26:11Z","timestamp":1762820771000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S2452302X22001425"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,12]]},"references-count":137,"journal-issue":{"issue":"12","published-print":{"date-parts":[[2022,12]]}},"alternative-id":["S2452302X22001425"],"URL":"https:\/\/doi.org\/10.1016\/j.jacbts.2022.04.005","relation":{},"ISSN":["2452-302X"],"issn-type":[{"value":"2452-302X","type":"print"}],"subject":[],"published":{"date-parts":[[2022,12]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Cardiovascular Research in Friedreich\u00a0Ataxia","name":"articletitle","label":"Article Title"},{"value":"JACC: Basic to Translational Science","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.jacbts.2022.04.005","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2022 The Author. Published by Elsevier on behalf of the American College of Cardiology Foundation.","name":"copyright","label":"Copyright"}]}}