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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Bibliographic Details
Published in:Biochimica et biophysica acta. Molecular and cell biology of lipids 2025-03, Vol.1870 (2), p.159582, Article 159582
Main Authors: Alatibi, Khaled, Sumser, Kathrin, Christopoulou, Maria Elpida, Hug, Martin J., Tucci, Sara
Format: Article
Language:English
Subjects:
ACSF3
Energy metabolism
Mitochondrial dynamics
Neurodegeneration
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Summary:•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.
ISSN:1388-1981
1879-2618
1879-2618
DOI:10.1016/j.bbalip.2024.159582