ArticleThe Journal of neuroscience : the official journal of the Society for Neuroscience2018
Impaired Mitochondrial Fatty Acid Synthesis Leads to Neurodegeneration in Mice.
Article in The Journal of neuroscience : the official journal of the Society for Neuroscience, 2018. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 14 papers.
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Who cites it
14 citing papers in PubMed, 36 citations in OpenAlex.
- The mitochondrial trans-2-enoyl-CoA reductase is necessary for mitochondrial homeostasis in C. elegans.Genetics · 2025Article
- A mouse model of MEPAN demonstrates a role for mitochondrial fatty acid synthesis in iron-sulfur cluster and supercomplex formation.Proceedings of the National Academy of Sciences of the United States of America · 2025Article
- Fatty acid metabolism related gene MECR contributes to the progression of prostate cancer.Cancer cell international · 2025Article
- Ucp4 Knockdown of Cerebellar Purkinje Cells Induces Bradykinesia.Molecular neurobiology · 2024Article
- Mitochondrial fatty acid synthesis is an emergent central regulator of mammalian oxidative metabolism.Cell metabolism · 2024Review
- Expert Panel Curation of 113 Primary Mitochondrial Disease Genes for the Leigh Syndrome Spectrum.Annals of neurology · 2023Article
- A defect in mitochondrial fatty acid synthesis impairs iron metabolism and causes elevated ceramide levels.Nature metabolism · 2023Article
- Understanding Long COVID; Mitochondrial Health and Adaptation-Old Pathways, New Problems.Biomedicines · 2022Review
- Genetic variants in ALDH1L1 and GLDC influence the serine-to-glycine ratio in Hispanic children.The American journal of clinical nutrition · 2022Article
- Lipid metabolism and storage in neuroglia: role in brain development and neurodegenerative diseases.Cell & bioscience · 2022Review
- Fatty Acid Synthesis in Glial Cells of the CNS.International journal of molecular sciences · 2021Review
- Mitochondrial fatty acid synthesis coordinates oxidative metabolism in mammalian mitochondria.eLife · 2020Article
- Mitochondrial biogenesis is altered in HIV+ brains exposed to ART: Implications for therapeutic targeting of astroglia.Neurobiology of disease · 2019Article
- Polioencephalopathy in Eurasier dogs.Journal of veterinary internal medicineArticle
Corrections and comments
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Authors and funding
10 authors at 4 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
There has been a growing interest toward mitochondrial fatty acid synthesis (mtFAS) since the recent discovery of a neurodegenerative human disorder termed MEPAN (mitochondrial enoyl reductase protein associated neurodegeneration), which is caused by mutations in the mitochondrial enoyl-CoA/ACP (acyl carrier protein) reductase (MECR) carrying out the last step of mtFAS. We show here that MECR protein is highly expressed in mouse Purkinje cells (PCs). To elucidate mtFAS function in neural tissue, here, we generated a mouse line with a PC-specific knock-out (KO) of
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