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Propionic acid (PPA) is widely used to study mitochondrial dysfunction in preclinical models of ASD. However, the molecular mechanisms that drive PPA-induced neurotoxicity are unresolved. Here, we examined mitochondrial remodeling under PPA-induced stress in neuroblastoma SH-SY5Y cells. PPA systemically altered the transcriptional regulation of mitochondrial dynamics and disrupted canonical proteins involved in mitochondrial fusion (L-OPA1, MFN2), fission (DRP1) and quality control (LC3-II). Confocal microscopy revealed an upregulation of both fission and fusion events and impairments to mitochondrial integrity, connectivity and turnover. Live-cell respirometry demonstrated consequent deficits in both oxidative and glycolytic energy production, while respiratory chain electron flow assays illustrated a shift in TCA cycle flux driven by a remodeling of mitochondrial substrate utilization. This work describes a molecular signature of metabolic stress in the SH-SY5Y system, providing novel insights into the mechanisms and manifestations of PPA-induced neurotoxicity.<\/jats:p>","DOI":"10.3390\/biology15161424","type":"journal-article","created":{"date-parts":[[2026,8,18]],"date-time":"2026-08-18T13:11:57Z","timestamp":1787058717000},"page":"1424","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Propionic Acid Remodels Mitochondrial Metabolism in SH-SY5Y Cells"],"prefix":"10.3390","volume":"15","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9050-2415","authenticated-orcid":false,"given":"Caitlyn","family":"Mahony","sequence":"first","affiliation":[{"name":"Department of Molecular and Cell Biology, University of Cape Town, Cape Town 7700, South Africa"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6046-6203","authenticated-orcid":false,"given":"Erin","family":"Buchanan","sequence":"additional","affiliation":[{"name":"Department of Molecular and Cell Biology, University of Cape Town, Cape Town 7700, South Africa"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3719-4657","authenticated-orcid":false,"given":"Colleen","family":"O\u2019Ryan","sequence":"additional","affiliation":[{"name":"Department of Molecular and Cell Biology, University of Cape Town, Cape Town 7700, South Africa"},{"name":"Neuroscience Institute, University of Cape Town, Cape Town 7700, South Africa"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2026,8,18]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"238","DOI":"10.1186\/s12967-024-05041-w","article-title":"Mitochondrial Metabolism in Neural Stem Cells and Implications for Neurodevelopmental and Neurodegenerative Diseases","volume":"22","author":"Garone","year":"2024","journal-title":"J. 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