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The Journal of Biological Chemistry|October 7, 2004
Leaky beta-oxidation of a trans-fatty acid: incomplete beta-oxidation of elaidic acid is due to the accumulation of 5-trans-tetradecenoyl-CoA and its hydrolysis and conversion to 5-trans-tetradecenoylcarnitine in the matrix of rat mitochondriaWenfeng Yu, Xiquan Liang, Regina E Ensenauer, et al.
The Journal of Biological Chemistry|July 29, 2010
Evidence for physical association of mitochondrial fatty acid oxidation and oxidative phosphorylation complexesYudong Wang, Al-Walid Mohsen, Stephanie J Mihalik, et al.
Current Protocols|October 28, 2025
Preparation of Vitrified Mammalian Cells for In Situ Cryo-Electron TomographyEva Martin-Solana, Sonia Frendi, Jiying Ning, et al.
Molecular Genetics and Metabolism|May 28, 2023
Challenges and strategies for clinical trials in propionic and methylmalonic acidemiasJerry Vockley, Barbara Burton, Agnieszka Jurecka, et al.
Molecular Neurobiology|September 9, 2024
Disruption of Mitochondrial Quality Control in Inherited Metabolic DisordersManuela Bianchin Marcuzzo, Josyane de Andrade Silveira, Emílio L Streck, et al.
Frontiers in Genetics|February 15, 2021
Physiological Perspectives on the Use of Triheptanoin as Anaplerotic Therapy for Long Chain Fatty Acid Oxidation DisordersEvgenia Sklirou, Ahmad N Alodaib, Steven F Dobrowolski, et al.
Neurology|March 4, 2018
Randomized dose-escalation trial of elamipretide in adults with primary mitochondrial myopathyAmel Karaa, Richard Haas, Amy Goldstein, et al.
Molecular Genetics and Metabolism|June 28, 2015
Long-term major clinical outcomes in patients with long chain fatty acid oxidation disorders before and after transition to triheptanoin treatment--A retrospective chart reviewJerry Vockley, Deborah Marsden, Elizabeth McCracken, et al.
Journal of Inherited Metabolic Disease|January 25, 2022
Medium branched chain fatty acids improve the profile of tricarboxylic acid cycle intermediates in mitochondrial fatty acid β-oxidation deficient cells: A comparative studyAnuradha Karunanidhi, Clinton Van't Land, Dhivyaa Rajasundaram, et al.
Molecular Genetics and Metabolism|May 3, 2005
Synergistic heterozygosity in mice with inherited enzyme deficiencies of mitochondrial fatty acid beta-oxidationA Michele Schuler, Barbara A Gower, Dietrich Matern, et al.
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