{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,25]],"date-time":"2026-09-25T06:00:24Z","timestamp":1790316024859,"version":"4.1.0"},"reference-count":33,"publisher":"The Company of Biologists","issue":"9","license":[{"start":{"date-parts":[[2026,9,25]],"date-time":"2026-09-25T00:00:00Z","timestamp":1790294400000},"content-version":"vor","delay-in-days":24,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"name":"Children's Hospital of Pittsburgh","id":[{"id":"https:\/\/ror.org\/03763ep67","id-type":"ROR","asserted-by":"crossref"}]},{"name":"University of Pittsburgh","id":[{"id":"https:\/\/ror.org\/01an3r305","id-type":"ROR","asserted-by":"crossref"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"published-print":{"date-parts":[[2026,9,1]]},"abstract":"<jats:title>ABSTRACT<\/jats:title>\n                  <jats:p>Cardiomyopathy is an important manifestation in patients with fatty acid oxidation disorders and represents a major cause of morbidity and early mortality in mitochondrial trifunctional protein (TFP) deficiency. Although a mouse model carrying the TFP \u03b2-subunit p.Met404Lys mutation (\u03b2TFP-deficient mice) has been described, cardiac involvement in this model has not been systematically characterized. Here, we combined cardiac histology and multiparametric cardiac magnetic resonance imaging (CMR) to define myocardial structure, function and tissue characteristics in this mouse model. Histological analysis with automated whole-slide collagen quantification revealed myocardial fibrosis with collagen deposition in mutant hearts, and CMR demonstrated increased myocardial extracellular volume in both male and female homozygous mutants. Homozygous males showed reduced ejection fraction, impaired systolic strain and increased left ventricular end-systolic volume, indicating systolic dysfunction. Male mice were more severely affected than females and exhibited reduced survival. Together, these findings demonstrate that \u03b2TFP-deficient mice develop fibrotic cardiomyopathy with systolic dysfunction, reproducing important cardiac features observed in human TFP deficiency. This work establishes the model as a relevant platform for investigating disease mechanisms and therapeutic strategies for cardiomyopathy in TFP deficiency.<\/jats:p>","DOI":"10.1242\/dmm.052921","type":"journal-article","created":{"date-parts":[[2026,8,3]],"date-time":"2026-08-03T06:41:25Z","timestamp":1785739285000},"source":"Crossref","is-referenced-by-count":0,"title":["Cardiac magnetic resonance imaging reveals myocardial fibrosis and systolic dysfunction in mitochondrial trifunctional protein-deficient mice"],"prefix":"10.1242","volume":"19","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-0247-837X","authenticated-orcid":false,"given":"Eduardo","family":"Vieira Neto","sequence":"first","affiliation":[{"name":"UPMC Children's Hospital of Pittsburgh, University of Pittsburgh 1 Department of Pediatrics, Genetic and Genomic Medicine Division , , Pittsburgh, PA 15224 ,","place":["USA"]},{"name":"Children's Neuroscience Institute 2 , Department of Pediatrics , , Pittsburgh, PA 15224 ,","place":["USA"]},{"name":"School of Medicine, University of Pittsburgh 2 , Department of Pediatrics , , Pittsburgh, PA 15224 ,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shakuntala","family":"Basu","sequence":"additional","affiliation":[{"name":"UPMC Children's Hospital of Pittsburgh, University of Pittsburgh 1 Department of Pediatrics, Genetic and Genomic Medicine Division , , Pittsburgh, PA 15224 ,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0008-0540-4289","authenticated-orcid":false,"given":"Thomas J.","family":"Becker-Szurszewski","sequence":"additional","affiliation":[{"name":"School of Medicine, University of Pittsburgh 3 Division of Neurology and Child Development , , Pittsburgh, PA 15224 ,","place":["USA"]},{"name":"Rangos Research Center Animal Imaging Core, School of Medicine, University of Pittsburgh 4 , Pittsburgh, PA 15224 ,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0006-2474-5623","authenticated-orcid":false,"given":"Sean","family":"Hartwick","sequence":"additional","affiliation":[{"name":"School of Medicine, University of Pittsburgh 3 Division of Neurology and Child Development , , Pittsburgh, PA 15224 ,","place":["USA"]},{"name":"Rangos Research Center Animal Imaging Core, School of Medicine, University of Pittsburgh 4 , Pittsburgh, PA 15224 ,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kristina E.","family":"Schwab","sequence":"additional","affiliation":[{"name":"School of Medicine, University of Pittsburgh 3 Division of Neurology and Child Development , , Pittsburgh, PA 15224 ,","place":["USA"]},{"name":"Rangos Research Center Animal Imaging Core, School of Medicine, University of Pittsburgh 4 , Pittsburgh, PA 15224 ,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-9509-5417","authenticated-orcid":false,"given":"Yijen","family":"Wu","sequence":"additional","affiliation":[{"name":"School of Medicine, University of Pittsburgh 3 Division of Neurology and Child Development , , Pittsburgh, PA 15224 ,","place":["USA"]},{"name":"Rangos Research Center Animal Imaging Core, School of Medicine, University of Pittsburgh 4 , Pittsburgh, PA 15224 ,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8180-6457","authenticated-orcid":false,"given":"Jerry","family":"Vockley","sequence":"additional","affiliation":[{"name":"UPMC Children's Hospital of Pittsburgh, University of Pittsburgh 1 Department of Pediatrics, Genetic and Genomic Medicine Division , , Pittsburgh, PA 15224 ,","place":["USA"]},{"name":"School of Public Health, Center for Rare Disease Therapy, UPMC Children's Hospital of Pittsburgh, University of Pittsburgh 5 Department of Human Genetics , , Pittsburgh, PA 15261 ,","place":["USA"]}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"237","published-online":{"date-parts":[[2026,9,25]]},"reference":[{"key":"2026092501450846900_DMM052921C1","doi-asserted-by":"publisher","first-page":"73","DOI":"10.1016\/j.mito.2018.02.009","article-title":"Mitochondrial \u03b2-oxidation of saturated fatty acids in humans","volume":"46","author":"Adeva-Andany","year":"2019","journal-title":"Mitochondrion"},{"key":"2026092501450846900_DMM052921C2","doi-asserted-by":"publisher","first-page":"108351","DOI":"10.1016\/j.ymgme.2024.108351","article-title":"A review of fatty acid oxidation disorder mouse models","volume":"142","author":"Babcock","year":"2024","journal-title":"Mol. 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