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However, FA hearts maintain adequate function until advanced disease stages, suggesting initial adaptation to the loss of frataxin (FXN). Conditional cardiac knockout mouse models of FXN show transcriptional and metabolic profiles of the mitochondrial integrated stress response (ISRmt), which could play an adaptive role. However, the ISRmt has not been investigated in models with disease-relevant, partial decrease in FXN. We characterized the heart transcriptomes and metabolomes of three mouse models with varying degrees of FXN depletion: YG8-800, KIKO-700 and FXNG127V. Few metabolites were changed in YG8-800 mice, which did not provide a signature of cardiomyopathy or ISRmt; several metabolites were altered in FXNG127V and KIKO-700 hearts. Transcriptional changes were found in all models, but differentially expressed genes consistent with cardiomyopathy and ISRmt were only identified in FXNG127V hearts. However, these changes were surprisingly mild even at advanced age (18\u2005months), despite a severe decrease in FXN levels to 1% of those of wild type. These findings indicate that the mouse heart has low reliance on FXN, highlighting the difficulty in modeling genetically relevant FA cardiomyopathy.<\/jats:p>","DOI":"10.1242\/dmm.050114","type":"journal-article","created":{"date-parts":[[2023,9,11]],"date-time":"2023-09-11T04:14:31Z","timestamp":1694405671000},"source":"Crossref","is-referenced-by-count":12,"title":["Comparative multi-omic analyses of cardiac mitochondrial stress in three mouse models of frataxin deficiency"],"prefix":"10.1242","volume":"16","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-7653-4282","authenticated-orcid":false,"given":"Nicole M.","family":"Sayles","sequence":"first","affiliation":[{"name":"Feil Family Brain and Mind Research Institute, Weill Cornell Medicine 1 , 407 East 61st Street, New York, NY 10065 , USA"},{"name":"Will Cornell Graduate School of Medical Sciences 2 Neuroscience Graduate Program , , 1300 York Ave, New York, NY 10065 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5141-0822","authenticated-orcid":false,"given":"Jill S.","family":"Napierala","sequence":"additional","affiliation":[{"name":"University of Alabama at Birmingham 3 Department of Biochemistry and Molecular Genetics , , Birmingham, AL 35294 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-0468-9162","authenticated-orcid":false,"given":"Josef","family":"Anrather","sequence":"additional","affiliation":[{"name":"Feil Family Brain and Mind Research Institute, Weill Cornell Medicine 1 , 407 East 61st Street, New York, NY 10065 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nad\u00e8ge","family":"Diedhiou","sequence":"additional","affiliation":[{"name":"Institut de Ge\u0301ne\u0301tique et de Biologie Mole\u0301culaire et Cellulaire (IGBMC) 4 , CNRS\/Universite\u0301 de Strasbourg UMR7104, Inserm U1258, B. P. 163, 67404 Illkirch , France"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jixue","family":"Li","sequence":"additional","affiliation":[{"name":"Department of Neurology, Peter O'Donnell Jr. Brain Institute, University of Texas Southwestern Medical Center 5 , Dallas, TX 75390 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9987-0641","authenticated-orcid":false,"given":"Marek","family":"Napierala","sequence":"additional","affiliation":[{"name":"University of Alabama at Birmingham 3 Department of Biochemistry and Molecular Genetics , , Birmingham, AL 35294 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4711-2248","authenticated-orcid":false,"given":"H\u00e9l\u00e8ne","family":"Puccio","sequence":"additional","affiliation":[{"name":"Institut de Ge\u0301ne\u0301tique et de Biologie Mole\u0301culaire et Cellulaire (IGBMC) 4 , CNRS\/Universite\u0301 de Strasbourg UMR7104, Inserm U1258, B. 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