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 fission process may play a role in the onset of neurodegeneration.
Authors
- Sara Tucci (ORCID: https://orcid.org/0000-0003-3851-4843)
- Martin Johannes Hug (ORCID: https://orcid.org/0000-0003-2305-2746)
- Maria Elpida Christopoulou
- Amr S. Khaled
- Kathrin Sumser
Institutions
- University Medical Center Freiburg (DE)
Publication Details
- Journal
- Biochimica et Biophysica Acta (BBA) - Molecular and Cell Biology of Lipids
- Published
- 2024-11-23
- DOI
- https://doi.org/10.1016/j.bbalip.2024.159582
- Citations
- 1
- Primary Topic
- Mitochondrial Function and Pathology
- Type
- article
- Field-Weighted Citation Impact
- 0.23