{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,16]],"date-time":"2026-09-16T12:10:28Z","timestamp":1789560628722,"version":"build-2803163510"},"reference-count":71,"publisher":"Elsevier BV","issue":"2","license":[{"start":{"date-parts":[[2011,5,1]],"date-time":"2011-05-01T00:00:00Z","timestamp":1304208000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2011,5,1]],"date-time":"2011-05-01T00:00:00Z","timestamp":1304208000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"}],"funder":[{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["1P01HL 085098"],"award-info":[{"award-number":["1P01HL 085098"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000002","name":"National Institutes of Health","doi-asserted-by":"publisher","award":["R21 NS052198"],"award-info":[{"award-number":["R21 NS052198"]}],"id":[{"id":"10.13039\/100000002","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100002108","name":"Friedreich's Ataxia Research Alliance","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100002108","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["clinicalkey.com","clinicalkey.com.au","clinicalkey.es","clinicalkey.fr","clinicalkey.jp","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Progress in Pediatric Cardiology"],"published-print":{"date-parts":[[2011,5]]},"DOI":"10.1016\/j.ppedcard.2011.02.007","type":"journal-article","created":{"date-parts":[[2011,4,15]],"date-time":"2011-04-15T13:57:35Z","timestamp":1302875855000},"page":"103-109","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":34,"title":["The heart in Friedreich's Ataxia: Basic findings and clinical implications"],"prefix":"10.1016","volume":"31","author":[{"given":"R. Mark","family":"Payne","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"78","reference":[{"key":"10.1016\/j.ppedcard.2011.02.007_bb0005","doi-asserted-by":"crossref","first-page":"589","DOI":"10.1093\/brain\/104.3.589","article-title":"Friedreich's ataxia: a clinical and genetic study of 90 families with an analysis of early diagnostic criteria and intrafamilial clustering of clinical features","volume":"104","author":"Harding","year":"1981","journal-title":"Brain"},{"key":"10.1016\/j.ppedcard.2011.02.007_bb0010","doi-asserted-by":"crossref","first-page":"200","DOI":"10.1016\/j.clineuro.2006.09.003","article-title":"Friedreich's ataxia: a clinical and genetic analysis","volume":"109","author":"Reddy","year":"2007","journal-title":"Clin Neurol Neurosurg"},{"key":"10.1016\/j.ppedcard.2011.02.007_bb0015","doi-asserted-by":"crossref","first-page":"55","DOI":"10.1002\/ana.21052","article-title":"Progressive GAA expansions in dorsal root ganglia of Friedreich's ataxia patients","volume":"61","author":"De Biase","year":"2007","journal-title":"Ann Neurol"},{"key":"10.1016\/j.ppedcard.2011.02.007_bb0020","doi-asserted-by":"crossref","first-page":"15","DOI":"10.1086\/321283","article-title":"Friedreich ataxia: from GAA triplet-repeat expansion to frataxin deficiency","volume":"69","author":"Patel","year":"2001","journal-title":"Am J Hum Genet"},{"key":"10.1016\/j.ppedcard.2011.02.007_bb0025","doi-asserted-by":"crossref","first-page":"391","DOI":"10.1007\/BF01930976","article-title":"Ueber degenerative Atrophie der spinalen Hinterstr\u00e4nge","volume":"26","author":"Friedreich","year":"1863","journal-title":"Virchows Arch"},{"key":"10.1016\/j.ppedcard.2011.02.007_bb0030","doi-asserted-by":"crossref","first-page":"493","DOI":"10.1161\/01.CIR.25.3.493","article-title":"Cardiac aspects of Friedreich's ataxia","volume":"25","author":"Boyer","year":"1962","journal-title":"Circulation"},{"key":"10.1016\/j.ppedcard.2011.02.007_bb0035","doi-asserted-by":"crossref","first-page":"2131","DOI":"10.1093\/brain\/120.12.2131","article-title":"Friedreich's ataxia: revision of the phenotype according to molecular genetics","volume":"120","author":"Schols","year":"1997","journal-title":"Brain"},{"key":"10.1016\/j.ppedcard.2011.02.007_bb0040","doi-asserted-by":"crossref","first-page":"351","DOI":"10.1007\/BF00867594","article-title":"Prevalence of hereditary ataxias and spastic paraplegias in Molise, a region of Italy","volume":"239","author":"Filla","year":"1992","journal-title":"J Neurol"},{"key":"10.1016\/j.ppedcard.2011.02.007_bb0045","first-page":"523","article-title":"Incidence of Friedreich ataxia in Italy estimated from consanguineous marriages","volume":"35","author":"Romeo","year":"1983","journal-title":"Am J Hum Genet"},{"key":"10.1016\/j.ppedcard.2011.02.007_bb0050","doi-asserted-by":"crossref","first-page":"287","DOI":"10.1111\/j.1399-0004.1975.tb00331.x","article-title":"Friedreich's ataxia in Western Norway","volume":"7","author":"Skre","year":"1975","journal-title":"Clin Genet"},{"key":"10.1016\/j.ppedcard.2011.02.007_bb0055","doi-asserted-by":"crossref","first-page":"834","DOI":"10.1007\/s004390050458","article-title":"Differential stability of the (GAA)n tract in the Friedreich ataxia (STM7) gene","volume":"99","author":"Epplen","year":"1997","journal-title":"Hum Genet"},{"key":"10.1016\/j.ppedcard.2011.02.007_bb0060","doi-asserted-by":"crossref","first-page":"1296","DOI":"10.1001\/archneur.65.10.1296","article-title":"Friedreich ataxia","volume":"65","author":"Pandolfo","year":"2008","journal-title":"Arch Neurol"},{"key":"10.1016\/j.ppedcard.2011.02.007_bb0065","doi-asserted-by":"crossref","first-page":"583","DOI":"10.1016\/S1097-2765(00)80058-1","article-title":"GAA instability in Friedreich's Ataxia shares a common, DNA-directed and intraallelic mechanism with other trinucleotide diseases","volume":"1","author":"Gacy","year":"1998","journal-title":"Mol Cell"},{"key":"10.1016\/j.ppedcard.2011.02.007_bb0070","doi-asserted-by":"crossref","first-page":"27171","DOI":"10.1074\/jbc.M101879200","article-title":"Sticky DNA, a self-associated complex formed at long GAA*TTC repeats in intron 1 of the frataxin gene, inhibits transcription","volume":"276","author":"Sakamoto","year":"2001","journal-title":"J Biol Chem"},{"key":"10.1016\/j.ppedcard.2011.02.007_bb0075","doi-asserted-by":"crossref","first-page":"551","DOI":"10.1038\/nchembio815","article-title":"Histone deacetylase inhibitors reverse gene silencing in Friedreich's ataxia","volume":"2","author":"Herman","year":"2006","journal-title":"Nat Chem Biol"},{"key":"10.1016\/j.ppedcard.2011.02.007_bb0080","doi-asserted-by":"crossref","first-page":"887","DOI":"10.1093\/hmg\/9.6.887","article-title":"Recent advances in the molecular pathogenesis of Friedreich ataxia","volume":"9","author":"Puccio","year":"2000","journal-title":"Hum Mol Genet"},{"key":"10.1016\/j.ppedcard.2011.02.007_bb0085","doi-asserted-by":"crossref","first-page":"16381","DOI":"10.1073\/pnas.0906784106","article-title":"Elucidation of the mechanism of mitochondrial iron loading in Friedreich's ataxia by analysis of a mouse mutant","volume":"106","author":"Huang","year":"2009","journal-title":"Proc Natl Acad Sci USA"},{"key":"10.1016\/j.ppedcard.2011.02.007_bb0090","doi-asserted-by":"crossref","first-page":"41469","DOI":"10.1074\/jbc.M006539200","article-title":"Two-step processing of human frataxin by mitochondrial processing peptidase. Precursor and intermediate forms are cleaved at different rates","volume":"275","author":"Cavadini","year":"2000","journal-title":"J Biol Chem"},{"key":"10.1016\/j.ppedcard.2011.02.007_bb0095","doi-asserted-by":"crossref","first-page":"1534","DOI":"10.1093\/hmg\/ddm102","article-title":"In vivo maturation of human frataxin","volume":"16","author":"Condo","year":"2007","journal-title":"Hum Mol Genet"},{"key":"10.1016\/j.ppedcard.2011.02.007_bb0100","doi-asserted-by":"crossref","first-page":"3521","DOI":"10.1093\/hmg\/ddn244","article-title":"The in vivo mitochondrial two-step maturation of human frataxin","volume":"17","author":"Schmucker","year":"2008","journal-title":"Hum Mol Genet"},{"key":"10.1016\/j.ppedcard.2011.02.007_bb0105","doi-asserted-by":"crossref","first-page":"433","DOI":"10.1016\/j.jmb.2004.10.074","article-title":"Supramolecular assemblies of human frataxin are formed via subunit\u2013subunit interactions mediated by a non-conserved amino-terminal region","volume":"345","author":"O'Neill","year":"2005","journal-title":"J Mol Biol"},{"key":"10.1016\/j.ppedcard.2011.02.007_bb0110","doi-asserted-by":"crossref","first-page":"537","DOI":"10.1021\/bi048459j","article-title":"Assembly of human frataxin is a mechanism for detoxifying redox-active iron","volume":"44","author":"O'Neill","year":"2005","journal-title":"Biochemistry"},{"key":"10.1016\/j.ppedcard.2011.02.007_bb0115","doi-asserted-by":"crossref","first-page":"4948","DOI":"10.1021\/bi800052m","article-title":"Structural basis of the iron storage function of frataxin from single-particle reconstruction of the iron-loaded oligomer","volume":"47","author":"Schagerlof","year":"2008","journal-title":"Biochemistry"},{"key":"10.1016\/j.ppedcard.2011.02.007_bb0120","doi-asserted-by":"crossref","first-page":"5971","DOI":"10.1021\/bi027021l","article-title":"Structure of frataxin iron cores: an X-ray absorption spectroscopic study","volume":"42","author":"Nichol","year":"2003","journal-title":"Biochemistry"},{"key":"10.1016\/j.ppedcard.2011.02.007_bb0125","doi-asserted-by":"crossref","first-page":"22493","DOI":"10.1074\/jbc.M604246200","article-title":"Mrs3p, Mrs4p, and frataxin provide iron for Fe\u2013S cluster synthesis in mitochondria","volume":"281","author":"Zhang","year":"2006","journal-title":"J Biol Chem"},{"key":"10.1016\/j.ppedcard.2011.02.007_bb0130","doi-asserted-by":"crossref","first-page":"929","DOI":"10.1093\/hmg\/ddm038","article-title":"Mitochondrial frataxin interacts with ISD11 of the NFS1\/ISCU complex and multiple mitochondrial chaperones","volume":"16","author":"Shan","year":"2007","journal-title":"Hum Mol Genet"},{"key":"10.1016\/j.ppedcard.2011.02.007_bb0135","doi-asserted-by":"crossref","first-page":"25943","DOI":"10.1074\/jbc.C400107200","article-title":"Frataxin-mediated iron delivery to ferrochelatase in the final step of heme biosynthesis","volume":"279","author":"Yoon","year":"2004","journal-title":"J Biol Chem"},{"key":"10.1016\/j.ppedcard.2011.02.007_bb0140","doi-asserted-by":"crossref","first-page":"2651","DOI":"10.1093\/hmg\/ddm163","article-title":"Frataxin is essential for extramitochondrial Fe\u2013S cluster proteins in mammalian tissues","volume":"16","author":"Martelli","year":"2007","journal-title":"Hum Mol Genet"},{"key":"10.1016\/j.ppedcard.2011.02.007_bb0145","doi-asserted-by":"crossref","first-page":"215","DOI":"10.1038\/ng1097-215","article-title":"Aconitase and mitochondrial iron\u2013sulphur protein deficiency in Friedreich ataxia","volume":"17","author":"Rotig","year":"1997","journal-title":"Nat Genet"},{"key":"10.1016\/j.ppedcard.2011.02.007_bb0150","doi-asserted-by":"crossref","first-page":"11492","DOI":"10.1073\/pnas.96.20.11492","article-title":"Deficit of in vivo mitochondrial ATP production in patients with Friedreich ataxia","volume":"96","author":"Lodi","year":"1999","journal-title":"Proc Natl Acad Sci USA"},{"key":"10.1016\/j.ppedcard.2011.02.007_bb0155","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 vivo 31P magnetic resonance spectroscopy study","volume":"52","author":"Lodi","year":"2001","journal-title":"Cardiovasc Res"},{"key":"10.1016\/j.ppedcard.2011.02.007_bb0160","first-page":"143","article-title":"Iron and Friedreich ataxia","author":"Pandolfo","year":"2006","journal-title":"J Neural Transm"},{"key":"10.1016\/j.ppedcard.2011.02.007_bb0165","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"},{"key":"10.1016\/j.ppedcard.2011.02.007_bb0170","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\u2013S enzyme deficiency followed by intramitochondrial iron deposits","volume":"27","author":"Puccio","year":"2001","journal-title":"Nat Genet"},{"key":"10.1016\/j.ppedcard.2011.02.007_bb0175","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"},{"key":"10.1016\/j.ppedcard.2011.02.007_bb0180","doi-asserted-by":"crossref","first-page":"9757","DOI":"10.1073\/pnas.0804261105","article-title":"The MCK mouse heart model of Friedreich's ataxia: alterations in iron-regulated proteins and cardiac hypertrophy are limited by iron chelation","volume":"105","author":"Whitnall","year":"2008","journal-title":"Proc Natl Acad Sci USA"},{"key":"10.1016\/j.ppedcard.2011.02.007_bb0185","doi-asserted-by":"crossref","first-page":"9470","DOI":"10.1021\/bi101057v","article-title":"TAT fusion protein transduction into isolated mitochondria is accelerated by sodium channel inhibitors","volume":"49","author":"Rayapureddi","year":"2010","journal-title":"Biochemistry"},{"key":"10.1016\/j.ppedcard.2011.02.007_bb0190","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 (London, England)"},{"key":"10.1016\/j.ppedcard.2011.02.007_bb0195","doi-asserted-by":"crossref","first-page":"735","DOI":"10.1093\/hmg\/ddm346","article-title":"The Friedreich ataxia GAA repeat expansion mutation induces comparable epigenetic changes in human and transgenic mouse brain and heart tissues","volume":"17","author":"Al-Mahdawi","year":"2008","journal-title":"Hum Mol Genet"},{"key":"10.1016\/j.ppedcard.2011.02.007_bb0200","doi-asserted-by":"crossref","first-page":"291","DOI":"10.1016\/S0014-5793(02)02251-2","article-title":"Frataxin knockin mouse","volume":"512","author":"Miranda","year":"2002","journal-title":"FEBS Lett"},{"key":"10.1016\/j.ppedcard.2011.02.007_bb0205","doi-asserted-by":"crossref","first-page":"e1958","DOI":"10.1371\/journal.pone.0001958","article-title":"HDAC inhibitors correct frataxin deficiency in a Friedreich ataxia mouse model","volume":"3","author":"Rai","year":"2008","journal-title":"PLoS ONE"},{"key":"10.1016\/j.ppedcard.2011.02.007_bb0210","doi-asserted-by":"crossref","first-page":"675","DOI":"10.1002\/ana.410410518","article-title":"Phenotypic variability in Friedreich ataxia: role of the associated GAA triplet repeat expansion","volume":"41","author":"Montermini","year":"1997","journal-title":"Ann Neurol"},{"key":"10.1016\/j.ppedcard.2011.02.007_bb0215","first-page":"554","article-title":"The relationship between trinucleotide (GAA) repeat length and clinical features in Friedreich ataxia","volume":"59","author":"Filla","year":"1996","journal-title":"Am J Hum Genet"},{"key":"10.1016\/j.ppedcard.2011.02.007_bb0220","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"},{"key":"10.1016\/j.ppedcard.2011.02.007_bb0225","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"},{"key":"10.1016\/j.ppedcard.2011.02.007_bb0230","first-page":"117","article-title":"Friedreich's ataxia cardiomyopathy: case based discussion and management issues","volume":"103","author":"Hanley","year":"2010","journal-title":"Ir Med J"},{"key":"10.1016\/j.ppedcard.2011.02.007_bb0235","doi-asserted-by":"crossref","first-page":"121","DOI":"10.1002\/ana.10419","article-title":"Cardiac energetics correlates to myocardial hypertrophy in Friedreich's ataxia","volume":"53","author":"Bunse","year":"2003","journal-title":"Ann Neurol"},{"key":"10.1016\/j.ppedcard.2011.02.007_bb0240","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"},{"key":"10.1016\/j.ppedcard.2011.02.007_bb0245","doi-asserted-by":"crossref","first-page":"1676","DOI":"10.1212\/01.WNL.0000055872.50364.FC","article-title":"Idebenone treatment in Friedreich patients: one-year-long randomized placebo-controlled trial","volume":"60","author":"Mariotti","year":"2003","journal-title":"Neurology"},{"key":"10.1016\/j.ppedcard.2011.02.007_bb0250","doi-asserted-by":"crossref","first-page":"470","DOI":"10.1016\/j.ejpn.2007.11.006","article-title":"Idebenone treatment in paediatric and adult patients with Friedreich ataxia: long-term follow-up","volume":"12","author":"Pineda","year":"2008","journal-title":"Eur J Paediatr Neurol"},{"key":"10.1016\/j.ppedcard.2011.02.007_bb0255","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 (London, England)"},{"key":"10.1016\/j.ppedcard.2011.02.007_bb0260","doi-asserted-by":"crossref","DOI":"10.1002\/14651858.CD007791.pub2","article-title":"Antioxidants and other pharmacological treatments for Friedreich ataxia","author":"Kearney","year":"2009","journal-title":"Cochrane Database Syst Rev"},{"key":"10.1016\/j.ppedcard.2011.02.007_bb0265","doi-asserted-by":"crossref","first-page":"941","DOI":"10.1001\/archneurol.2010.168","article-title":"A phase 3, double-blind, placebo-controlled trial of idebenone in friedreich ataxia","volume":"67","author":"Lynch","year":"2010","journal-title":"Arch Neurol"},{"key":"10.1016\/j.ppedcard.2011.02.007_bb0270","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"},{"key":"10.1016\/j.ppedcard.2011.02.007_bb0275","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"},{"key":"10.1016\/j.ppedcard.2011.02.007_bb0280","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"},{"key":"10.1016\/j.ppedcard.2011.02.007_bb0285","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"},{"key":"10.1016\/j.ppedcard.2011.02.007_bb0290","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"},{"key":"10.1016\/j.ppedcard.2011.02.007_bb0295","doi-asserted-by":"crossref","first-page":"1171","DOI":"10.1001\/jama.298.10.1171","article-title":"Carvedilol for children and adolescents with heart failure: a randomized controlled trial","volume":"298","author":"Shaddy","year":"2007","journal-title":"JAMA"},{"key":"10.1016\/j.ppedcard.2011.02.007_bb0300","doi-asserted-by":"crossref","first-page":"235","DOI":"10.1517\/13543784.12.2.235","article-title":"Friedreich's ataxia: iron chelators that target the mitochondrion as a therapeutic strategy?","volume":"12","author":"Richardson","year":"2003","journal-title":"Expert Opin Investig Drugs"},{"key":"10.1016\/j.ppedcard.2011.02.007_bb0305","doi-asserted-by":"crossref","first-page":"1972","DOI":"10.1096\/fj.03-0240fje","article-title":"Mitochondria-targeted antioxidants protect Friedreich Ataxia fibroblasts from endogenous oxidative stress more effectively than untargeted antioxidants","volume":"17","author":"Jauslin","year":"2003","journal-title":"FASEB J"},{"key":"10.1016\/j.ppedcard.2011.02.007_bb0310","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"},{"key":"10.1016\/j.ppedcard.2011.02.007_bb0315","doi-asserted-by":"crossref","first-page":"401","DOI":"10.1182\/blood-2006-12-065433","article-title":"Selective iron chelation in Friedreich ataxia: biologic and clinical implications","volume":"110","author":"Boddaert","year":"2007","journal-title":"Blood"},{"key":"10.1016\/j.ppedcard.2011.02.007_bb0320","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1182\/blood-2007-04-084509","article-title":"Ironing out a therapy for Friedreich ataxia","volume":"110","author":"Isaya","year":"2007","journal-title":"Blood"},{"key":"10.1016\/j.ppedcard.2011.02.007_bb0325","doi-asserted-by":"crossref","first-page":"1S","DOI":"10.1097\/01.gim.0000223891.82390.ad","article-title":"Newborn screening: toward a uniform screening panel and system","volume":"8","author":"Watson","year":"2006","journal-title":"Genet Med"},{"key":"10.1016\/j.ppedcard.2011.02.007_bb0330","doi-asserted-by":"crossref","first-page":"32541","DOI":"10.1074\/jbc.M405930200","article-title":"Transactivator of transcription fusion protein transduction causes membrane inversion","volume":"279","author":"Del Gaizo Moore","year":"2004","journal-title":"J Biol Chem"},{"key":"10.1016\/j.ppedcard.2011.02.007_bb0335","doi-asserted-by":"crossref","first-page":"170","DOI":"10.1016\/j.ymgme.2003.08.017","article-title":"Targeting proteins to mitochondria using TAT","volume":"80","author":"Del Gaizo-Moore","year":"2003","journal-title":"Mol Genet Metab"},{"key":"10.1016\/j.ppedcard.2011.02.007_bb0340","doi-asserted-by":"crossref","first-page":"720","DOI":"10.1016\/S1525-0016(03)00130-8","article-title":"A novel TAT-Mitochondrial signal sequence fusion protein is processed, stays in mitochondria, and crosses the placenta","volume":"7","author":"Del Gaizo","year":"2003","journal-title":"Mol Ther"},{"key":"10.1016\/j.ppedcard.2011.02.007_bb0345","doi-asserted-by":"crossref","first-page":"691","DOI":"10.1038\/mt.2008.4","article-title":"TAT-mediated delivery of LAD restores pyruvate dehydrogenase complex activity in the mitochondria of patients with LAD deficiency","volume":"16","author":"Rapoport","year":"2008","journal-title":"Mol Ther"},{"key":"10.1016\/j.ppedcard.2011.02.007_bb0350","doi-asserted-by":"crossref","first-page":"735","DOI":"10.1161\/01.CIR.0000023943.50821.F7","article-title":"TAT protein transduction into isolated perfused hearts: TAT-apoptosis repressor with caspase recruitment domain is cardioprotective","volume":"106","author":"Gustafsson","year":"2002","journal-title":"Circulation"},{"key":"10.1016\/j.ppedcard.2011.02.007_bb0355","doi-asserted-by":"crossref","first-page":"2717","DOI":"10.1172\/JCI25052","article-title":"TAT-mediated intracellular delivery of purine nucleoside phosphorylase corrects its deficiency in mice","volume":"116","author":"Toro","year":"2006","journal-title":"J Clin Invest"}],"container-title":["Progress in Pediatric Cardiology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S1058981311000117?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S1058981311000117?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2025,10,21]],"date-time":"2025-10-21T01:01:16Z","timestamp":1761008476000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S1058981311000117"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2011,5]]},"references-count":71,"journal-issue":{"issue":"2","published-print":{"date-parts":[[2011,5]]}},"alternative-id":["S1058981311000117"],"URL":"https:\/\/doi.org\/10.1016\/j.ppedcard.2011.02.007","relation":{},"ISSN":["1058-9813"],"issn-type":[{"value":"1058-9813","type":"print"}],"subject":[],"published":{"date-parts":[[2011,5]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"The heart in Friedreich's Ataxia: Basic findings and clinical implications","name":"articletitle","label":"Article Title"},{"value":"Progress in Pediatric Cardiology","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.ppedcard.2011.02.007","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"Copyright \u00a9 2011 Elsevier Ireland Ltd. All rights reserved.","name":"copyright","label":"Copyright"}]}}