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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| container_title | Biochimica et biophysica acta. Molecular and cell biology of lipids |
| container_volume | 1870 |
| creator | Alatibi, Khaled Sumser, Kathrin Christopoulou, Maria Elpida Hug, Martin J. Tucci, Sara |
| description | •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. |
| doi_str_mv | 10.1016/j.bbalip.2024.159582 |
| format | Article |
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| identifier | ISSN: 1388-1981 |
| ispartof | Biochimica et biophysica acta. Molecular and cell biology of lipids, 2025-03, Vol.1870 (2), p.159582, Article 159582 |
| issn | 1388-1981 1879-2618 1879-2618 |
| language | eng |
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| source | Elsevier ScienceDirect Journals Complete |
| subjects | ACSF3 Energy metabolism Mitochondrial dynamics Neurodegeneration |
| title | Dysregulated mitochondrial fission and neurodegeneration proteomic signature in ACSF3-deficient cells |
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