Dysregulated mitochondrial fission and neurodegeneration proteomic signature in ACSF3-deficient cells
•ACSF3 catalyzes the first step of mitochondrial fatty acid biosynthesis (mtFAS).•Mutations in ACSF3 cause CMAMMA an inborn error of metabolism.•Hypofunctional mtFAS impairs mitochondrial flexibility and energetics.•ACSF3 deficient fibroblasts show altered mitochondrial fission.•Dysfunctional fissio...
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| Veröffentlicht in: | Biochimica et biophysica acta. Molecular and cell biology of lipids 2025-03, Vol.1870 (2), p.159582, Article 159582 |
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| Hauptverfasser: | , , , , |
| Format: | Artikel |
| Sprache: | eng |
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| Online-Zugang: | Volltext |
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| Zusammenfassung: | •ACSF3 catalyzes the first step of mitochondrial fatty acid biosynthesis (mtFAS).•Mutations in ACSF3 cause CMAMMA an inborn error of metabolism.•Hypofunctional mtFAS impairs mitochondrial flexibility and energetics.•ACSF3 deficient fibroblasts show altered mitochondrial fission.•Dysfunctional fission process may play a role in the onset of neurodegeneration. |
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| ISSN: | 1388-1981 1879-2618 1879-2618 |
| DOI: | 10.1016/j.bbalip.2024.159582 |