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An excess of VLCFAs disrupts essential homeostatic functions crucial for axonal maintenance, including redox metabolism, glycolysis and mitochondrial respiration. As mitochondrial function and morphology are intertwined, we set out to investigate the role of mitochondrial dynamics in X-ALD models.<\/jats:p>\n                  <jats:p>Using quantitative 3D transmission electron microscopy, we revealed mitochondrial fragmentation in corticospinal axons in Abcd1\u2212 mice. In patient fibroblasts, an excess of VLCFAs triggers mitochondrial fragmentation through the redox-dependent phosphorylation of DRP1 (DRP1S616). The blockade of DRP1-driven fission by the peptide P110 effectively preserved mitochondrial morphology. Furthermore, mRNA inhibition of DRP1 not only prevented mitochondrial fragmentation but also protected axonal health in a Caenorhabditis elegans model of X-ALD, underscoring DRP1 as a potential therapeutic target.<\/jats:p>\n                  <jats:p>Elevated levels of circulating cell-free mtDNA in patients\u2019 CSF align this leukodystrophy with primary mitochondrial disorders. Our findings underscore the intricate interplay between peroxisomal dysfunction, mitochondrial dynamics and axonal integrity in X-ALD, shedding light on potential avenues for therapeutic intervention.<\/jats:p>","DOI":"10.1093\/brain\/awae038","type":"journal-article","created":{"date-parts":[[2024,2,13]],"date-time":"2024-02-13T00:31:33Z","timestamp":1707784293000},"page":"2069-2084","source":"Crossref","is-referenced-by-count":14,"title":["Imbalanced mitochondrial dynamics contributes to the pathogenesis of X-linked adrenoleukodystrophy"],"prefix":"10.1093","volume":"147","author":[{"given":"Nathalie","family":"Launay","sequence":"first","affiliation":[{"name":"Neurometabolic Diseases Laboratory, Institute of Neuropathology, IDIBELL , 08908 L\u2019Hospitalet de Llobregat, Barcelona , Spain"},{"name":"CIBERER, Centro de Investigaci\u00f3n Biom\u00e9dica en Red de Enfermedades Raras, ISCIII , 28029 Madrid , Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jone","family":"Lopez-Erauskin","sequence":"additional","affiliation":[{"name":"Neurometabolic Diseases Laboratory, Institute of Neuropathology, IDIBELL , 08908 L\u2019Hospitalet de Llobregat, Barcelona , Spain"},{"name":"Department of Cellular and Molecular Medicine, Ludwig Institute for Cancer Research, University of California at San Diego , La Jolla, CA 92093 , USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Patrizia","family":"Bianchi","sequence":"additional","affiliation":[{"name":"Neurometabolic Diseases Laboratory, Institute of Neuropathology, IDIBELL , 08908 L\u2019Hospitalet de Llobregat, Barcelona , Spain"},{"name":"Physiology and Immunology, Facultat de Medicina, Institut de Neuroci\u00e8ncies and Department of Cell Biology, Universitat Aut\u00f2noma de Barcelona , 08193 Bellaterra , 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08908 L\u2019Hospitalet de Llobregat, Barcelona , Spain"},{"name":"CIBERER, Centro de Investigaci\u00f3n Biom\u00e9dica en Red de Enfermedades Raras, ISCIII , 28029 Madrid , Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Abraham J","family":"Paredes-Fuentes","sequence":"additional","affiliation":[{"name":"Division of Inborn Errors of Metabolism-IBC, Biochemistry and Molecular Genetics Department, Hospital Cl\u00ednic de Barcelona , 08028 Barcelona , Spain"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-3422-9685","authenticated-orcid":false,"given":"Rafael","family":"Artuch","sequence":"additional","affiliation":[{"name":"CIBERER, Centro de Investigaci\u00f3n Biom\u00e9dica en Red de Enfermedades Raras, ISCIII , 28029 Madrid , Spain"},{"name":"Clinical Biochemistry Department, Institut de Recerca Sant Joan de D\u00e9u, Hospital Sant Joan de D\u00e9u , 08950 Barcelona , 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