{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,2,11]],"date-time":"2026-02-11T21:06:20Z","timestamp":1770843980607,"version":"3.50.1"},"reference-count":39,"publisher":"Elsevier BV","license":[{"start":{"date-parts":[[2020,4,1]],"date-time":"2020-04-01T00:00:00Z","timestamp":1585699200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/tdm\/userlicense\/1.0\/"},{"start":{"date-parts":[[2020,4,1]],"date-time":"2020-04-01T00:00:00Z","timestamp":1585699200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/www.elsevier.com\/legal\/tdmrep-license"}],"funder":[{"DOI":"10.13039\/501100008530","name":"European Regional Development Fund","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100008530","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100010434","name":"\u201cla Caixa\u201d Foundation","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100010434","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100003329","name":"Ministerio de Econom\u00eda y Competitividad","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100003329","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["clinicalkey.com","clinicalkey.com.au","clinicalkey.es","clinicalkey.fr","clinicalkey.jp","translationalres.com","elsevier.com","sciencedirect.com"],"crossmark-restriction":true},"short-container-title":["Translational Research"],"published-print":{"date-parts":[[2020,4]]},"DOI":"10.1016\/j.trsl.2019.12.004","type":"journal-article","created":{"date-parts":[[2019,12,26]],"date-time":"2019-12-26T20:04:17Z","timestamp":1577390657000},"page":"43-56","update-policy":"https:\/\/doi.org\/10.1016\/elsevier_cm_policy","source":"Crossref","is-referenced-by-count":18,"special_numbering":"C","title":["Pathogenic implications of dysregulated miRNAs in propionic acidemia related cardiomyopathy"],"prefix":"10.1016","volume":"218","author":[{"given":"Alejandro","family":"Fulgencio-Covi\u00e1n","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Esmeralda","family":"Alonso-Barroso","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Adam J.","family":"Guenzel","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ana","family":"Rivera-Barahona","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Magdalena","family":"Ugarte","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Bel\u00e9n","family":"P\u00e9rez","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Michael A.","family":"Barry","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Celia","family":"P\u00e9rez-Cerd\u00e1","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Eva","family":"Richard","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Lourdes R.","family":"Desviat","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"78","reference":[{"key":"10.1016\/j.trsl.2019.12.004_bib0001","doi-asserted-by":"crossref","first-page":"310","DOI":"10.1016\/j.celrep.2015.12.031","article-title":"Elucidating microRNA regulatory networks using transcriptional, post-transcriptional, and histone modification measurements","volume":"14","author":"Gosline","year":"2016","journal-title":"Cell Rep"},{"key":"10.1016\/j.trsl.2019.12.004_bib0002","doi-asserted-by":"crossref","first-page":"471","DOI":"10.1007\/s10545-017-0018-6","article-title":"Role of miRNAs in human disease and inborn errors of metabolism","volume":"40","author":"Rivera-Barahona","year":"2017","journal-title":"J Inherit Metab Dis"},{"key":"10.1016\/j.trsl.2019.12.004_bib0003","doi-asserted-by":"crossref","first-page":"846","DOI":"10.1016\/j.clinbiochem.2013.03.015","article-title":"Circulating miRNAs as sensitive and specific biomarkers for the diagnosis and monitoring of human diseases: promises and challenges","volume":"46","author":"De Guire","year":"2013","journal-title":"Clin Biochem"},{"key":"10.1016\/j.trsl.2019.12.004_bib0004","doi-asserted-by":"crossref","first-page":"19","DOI":"10.1016\/j.ijdevneu.2014.04.005","article-title":"MicroRNA-9 regulates neural apoptosis in methylmalonic acidemia via targeting BCL2L11","volume":"36","author":"Li","year":"2014","journal-title":"Int J Dev Neurosci"},{"key":"10.1016\/j.trsl.2019.12.004_bib0005","doi-asserted-by":"crossref","first-page":"5840","DOI":"10.1038\/ncomms6840","article-title":"A non-conserved miRNA regulates lysosomal function and impacts on a human lysosomal storage disorder","volume":"5","author":"Frankel","year":"2014","journal-title":"Nat Commun"},{"key":"10.1016\/j.trsl.2019.12.004_bib0006","doi-asserted-by":"crossref","first-page":"5727","DOI":"10.1038\/s41598-017-06420-8","article-title":"Dysregulated miRNAs and their pathogenic implications for the neurometabolic disease propionic acidemia","volume":"7","author":"Rivera-Barahona","year":"2017","journal-title":"Sci Rep"},{"key":"10.1016\/j.trsl.2019.12.004_bib0007","doi-asserted-by":"crossref","first-page":"1427","DOI":"10.1517\/21678707.2015.1092380","article-title":"Understanding molecular mechanisms in propionic acidemia and investigated therapeutic strategies","volume":"3","author":"Richard","year":"2015","journal-title":"Expert Opin Orphan Drugs"},{"key":"10.1016\/j.trsl.2019.12.004_bib0008","doi-asserted-by":"crossref","first-page":"1641","DOI":"10.1002\/ajmg.a.35387","article-title":"Survey of health status and complications among propionic acidemia patients","volume":"158A","author":"Pena","year":"2012","journal-title":"Am J Med Genet A"},{"key":"10.1016\/j.trsl.2019.12.004_bib0009","doi-asserted-by":"crossref","first-page":"730","DOI":"10.1002\/jimd.12129","article-title":"Pathophysiology of propionic and methylmalonic acidemias. Part 1: Complications","volume":"42","author":"Haijes","year":"2019","journal-title":"J Inherit Metab Dis"},{"key":"10.1016\/j.trsl.2019.12.004_bib0010","doi-asserted-by":"crossref","first-page":"1316","DOI":"10.1038\/mt.2013.68","article-title":"Generation of a hypomorphic model of propionic acidemia amenable to gene therapy testing","volume":"21","author":"Guenzel","year":"2013","journal-title":"Mol Ther"},{"key":"10.1016\/j.trsl.2019.12.004_bib0011","doi-asserted-by":"crossref","DOI":"10.1016\/j.bbadis.2019.165586","article-title":"Intracellular calcium mishandling leads to cardiac dysfunction and ventricular arrhythmias in a mouse model of propionic acidemia","volume":"1866","author":"Tamayo","year":"2020","journal-title":"Biochim Biophys Acta Mol Basis Dis."},{"key":"10.1016\/j.trsl.2019.12.004_bib0012","doi-asserted-by":"crossref","first-page":"629","DOI":"10.1113\/expphysiol.2012.068577","article-title":"High-resolution echocardiography in the assessment of cardiac physiology and disease in preclinical models","volume":"98","author":"Moran","year":"2013","journal-title":"Exp Physiol"},{"key":"10.1016\/j.trsl.2019.12.004_bib0013","doi-asserted-by":"crossref","first-page":"20","DOI":"10.1016\/j.yjmcc.2017.10.006","article-title":"Echocardiographic evaluation of diastolic function in mouse models of heart disease","volume":"114","author":"Schnelle","year":"2018","journal-title":"J Mol Cell Cardiol"},{"key":"10.1016\/j.trsl.2019.12.004_bib0014","doi-asserted-by":"crossref","first-page":"107","DOI":"10.1042\/BJ20060221","article-title":"Secondary mitochondrial dysfunction in propionic aciduria: a pathogenic role for endogenous mitochondrial toxins","volume":"398","author":"Schwab","year":"2006","journal-title":"Biochem J"},{"key":"10.1016\/j.trsl.2019.12.004_bib0015","doi-asserted-by":"crossref","first-page":"749","DOI":"10.3390\/ijms17050749","article-title":"Overview of MicroRNAs in cardiac hypertrophy, fibrosis, and apoptosis","volume":"17","author":"Wang","year":"2016","journal-title":"Int J Mol Sci"},{"key":"10.1016\/j.trsl.2019.12.004_bib0016","doi-asserted-by":"crossref","first-page":"1391","DOI":"10.1002\/jcp.22852","article-title":"Attenuation of microRNA-22 derepressed PTEN to effectively protect rat cardiomyocytes from hypertrophy","volume":"227","author":"Xu","year":"2012","journal-title":"J Cell Physiol"},{"key":"10.1016\/j.trsl.2019.12.004_bib0017","doi-asserted-by":"crossref","first-page":"1205","DOI":"10.1038\/cdd.2015.95","article-title":"miR-199a impairs autophagy and induces cardiac hypertrophy through mTOR activation","volume":"24","author":"Li","year":"2017","journal-title":"Cell Death Differ"},{"key":"10.1016\/j.trsl.2019.12.004_bib0018","doi-asserted-by":"crossref","first-page":"341","DOI":"10.1016\/j.cmet.2013.08.009","article-title":"The hypoxia-inducible microRNA cluster miR-199a-214 targets myocardial PPARdelta and impairs mitochondrial fatty acid oxidation","volume":"18","author":"el Azzouzi","year":"2013","journal-title":"Cell Metab"},{"key":"10.1016\/j.trsl.2019.12.004_bib0019","doi-asserted-by":"crossref","first-page":"2772","DOI":"10.1172\/JCI36154","article-title":"MicroRNA-208a is a regulator of cardiac hypertrophy and conduction in mice","volume":"119","author":"Callis","year":"2009","journal-title":"J Clin Invest"},{"key":"10.1016\/j.trsl.2019.12.004_bib0020","doi-asserted-by":"crossref","first-page":"e325","DOI":"10.5858\/2003-127-e325-PAANCR","article-title":"Propionic acidemia: a neuropathology case report and review of prior cases","volume":"127","author":"Feliz","year":"2003","journal-title":"Arch Pathol Lab Med"},{"key":"10.1016\/j.trsl.2019.12.004_bib0021","doi-asserted-by":"crossref","first-page":"201","DOI":"10.1016\/j.ijcard.2016.11.279","article-title":"Relations between circulating microRNAs (miR-21, miR-26, miR-29, miR-30 and miR-133a), extracellular matrix fibrosis and serum markers of fibrosis in dilated cardiomyopathy","volume":"231","author":"Rubis","year":"2017","journal-title":"Int J Cardiol"},{"key":"10.1016\/j.trsl.2019.12.004_bib0022","doi-asserted-by":"crossref","first-page":"634","DOI":"10.1016\/j.acvd.2015.07.003","article-title":"Circulating microribonucleic acids miR-1, miR-21 and miR-208a in patients with symptomatic heart failure: Preliminary results","volume":"108","author":"Sygitowicz","year":"2015","journal-title":"Arch Cardiovasc Dis"},{"key":"10.1016\/j.trsl.2019.12.004_bib0023","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1016\/j.freeradbiomed.2016.04.007","article-title":"In vivo evidence of mitochondrial dysfunction and altered redox homeostasis in a genetic mouse model of propionic acidemia: Implications for the pathophysiology of this disorder","volume":"96","author":"Gallego-Villar","year":"2016","journal-title":"Free Radic Biol Med"},{"key":"10.1016\/j.trsl.2019.12.004_bib0024","doi-asserted-by":"crossref","first-page":"143","DOI":"10.1186\/s12967-016-0893-x","article-title":"Regulatory mechanisms of microRNA expression","volume":"14","author":"Gulyaeva","year":"2016","journal-title":"J Transl Med"},{"key":"10.1016\/j.trsl.2019.12.004_bib0025","first-page":"5244","article-title":"Sodium propionate and sodium butyrate effects on histone deacetylase (HDAC) activity, histone acetylation, and inflammatory gene expression in bovine mammary epithelial cells","volume":"96","author":"Silva","year":"2018","journal-title":"J Anim Sci"},{"key":"10.1016\/j.trsl.2019.12.004_bib0026","doi-asserted-by":"crossref","first-page":"151","DOI":"10.1016\/j.yjmcc.2019.04.003","article-title":"Histone deacetylases in cardiovascular and metabolic diseases","volume":"130","author":"Bagchi","year":"2019","journal-title":"J Mol Cell Cardiol"},{"key":"10.1016\/j.trsl.2019.12.004_bib0027","doi-asserted-by":"crossref","first-page":"1048","DOI":"10.1038\/nsmb.3490","article-title":"Histone propionylation is a mark of active chromatin","volume":"24","author":"Kebede","year":"2017","journal-title":"Nat Struct Mol Biol"},{"key":"10.1016\/j.trsl.2019.12.004_bib0028","doi-asserted-by":"crossref","DOI":"10.1155\/2018\/1246069","article-title":"Altered redox homeostasis in branched-chain amino acid disorders, organic acidurias, and homocystinuria","volume":"2018","author":"Richard","year":"2018","journal-title":"Oxid Med Cell Longev"},{"key":"10.1016\/j.trsl.2019.12.004_bib0029","doi-asserted-by":"crossref","first-page":"H2181","DOI":"10.1152\/ajpheart.00554.2011","article-title":"Oxidative stress and heart failure","volume":"301","author":"Tsutsui","year":"2011","journal-title":"Am J Physiol Heart Circ Physiol"},{"key":"10.1016\/j.trsl.2019.12.004_bib0030","doi-asserted-by":"crossref","first-page":"404","DOI":"10.1016\/j.cardfail.2010.01.002","article-title":"Expression of microRNA-208 is associated with adverse clinical outcomes in human dilated cardiomyopathy","volume":"16","author":"Satoh","year":"2010","journal-title":"J Card Fail"},{"key":"10.1016\/j.trsl.2019.12.004_bib0031","doi-asserted-by":"crossref","first-page":"829","DOI":"10.1536\/ihj.17-276","article-title":"MicroRNA-208a Correlates Apoptosis and Oxidative Stress Induced by H2O2 through Protein Tyrosine Kinase\/Phosphatase Balance in Cardiomyocytes","volume":"59","author":"Liu","year":"2018","journal-title":"Int Heart J"},{"key":"10.1016\/j.trsl.2019.12.004_bib0032","doi-asserted-by":"crossref","first-page":"E622","DOI":"10.1152\/ajpendo.00081.2018","article-title":"Propionate-induced changes in cardiac metabolism, notably CoA trapping, are not altered by l-carnitine","volume":"315","author":"Wang","year":"2018","journal-title":"Am J Physiol Endocrinol Metab"},{"key":"10.1016\/j.trsl.2019.12.004_bib0033","doi-asserted-by":"crossref","first-page":"19","DOI":"10.1016\/j.phrs.2019.03.006","article-title":"Properties of FDA-approved small molecule protein kinase inhibitors","volume":"144","author":"Roskoski","year":"2019","journal-title":"Pharmacol Res"},{"key":"10.1016\/j.trsl.2019.12.004_bib0034","doi-asserted-by":"crossref","first-page":"68","DOI":"10.4103\/2229-3485.179431","article-title":"MicroRNA therapeutics: Discovering novel targets and developing specific therapy","volume":"7","author":"Christopher","year":"2016","journal-title":"Perspect Clin Res"},{"key":"10.1016\/j.trsl.2019.12.004_bib0035","doi-asserted-by":"crossref","DOI":"10.1161\/JAHA.113.000284","article-title":"Long-term miR-669a therapy alleviates chronic dilated cardiomyopathy in dystrophic mice","volume":"2","author":"Quattrocelli","year":"2013","journal-title":"J Am Heart Assoc"},{"key":"10.1016\/j.trsl.2019.12.004_bib0036","doi-asserted-by":"crossref","first-page":"e39","DOI":"10.1038\/mtna.2012.26","article-title":"Expression analysis in multiple muscle groups and serum reveals complexity in the microRNA transcriptome of the mdx mouse with implications for therapy","volume":"1","author":"Roberts","year":"2012","journal-title":"Mol Ther Nucleic Acids"},{"key":"10.1016\/j.trsl.2019.12.004_bib0037","doi-asserted-by":"crossref","first-page":"809","DOI":"10.1038\/nmeth.3014","article-title":"Evaluation of quantitative miRNA expression platforms in the microRNA quality control (miRQC) study","volume":"11","author":"Mestdagh","year":"2014","journal-title":"Nat Methods"},{"key":"10.1016\/j.trsl.2019.12.004_bib0038","doi-asserted-by":"crossref","first-page":"119","DOI":"10.1016\/j.yjmcc.2007.05.015","article-title":"Metabolic and signaling alterations in dystrophin-deficient hearts precede overt cardiomyopathy","volume":"43","author":"Khairallah","year":"2007","journal-title":"J Mol Cell Cardiol"},{"key":"10.1016\/j.trsl.2019.12.004_bib0039","doi-asserted-by":"crossref","first-page":"128","DOI":"10.1016\/j.jpeds.2009.07.002","article-title":"Cardiomyopathies in propionic aciduria are reversible after liver transplantation","volume":"156","author":"Romano","year":"2010","journal-title":"J Pediatr"}],"container-title":["Translational Research"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S1931524419302580?httpAccept=text\/xml","content-type":"text\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.elsevier.com\/content\/article\/PII:S1931524419302580?httpAccept=text\/plain","content-type":"text\/plain","content-version":"vor","intended-application":"text-mining"}],"deposited":{"date-parts":[[2025,10,30]],"date-time":"2025-10-30T12:39:41Z","timestamp":1761827981000},"score":1,"resource":{"primary":{"URL":"https:\/\/linkinghub.elsevier.com\/retrieve\/pii\/S1931524419302580"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,4]]},"references-count":39,"alternative-id":["S1931524419302580"],"URL":"https:\/\/doi.org\/10.1016\/j.trsl.2019.12.004","relation":{},"ISSN":["1931-5244"],"issn-type":[{"value":"1931-5244","type":"print"}],"subject":[],"published":{"date-parts":[[2020,4]]},"assertion":[{"value":"Elsevier","name":"publisher","label":"This article is maintained by"},{"value":"Pathogenic implications of dysregulated miRNAs in propionic acidemia related cardiomyopathy","name":"articletitle","label":"Article Title"},{"value":"Translational Research","name":"journaltitle","label":"Journal Title"},{"value":"https:\/\/doi.org\/10.1016\/j.trsl.2019.12.004","name":"articlelink","label":"CrossRef DOI link to publisher maintained version"},{"value":"article","name":"content_type","label":"Content Type"},{"value":"\u00a9 2019 Elsevier Inc. All rights reserved.","name":"copyright","label":"Copyright"}]}}