Homozygous Expression of Mutant ELOVL4 Leads to Seizures and Death in a Novel Animal Model of Very Long-Chain Fatty Acid Deficiency

Lipids are essential components of the nervous system. However, the functions of very long-chain fatty acids (VLC-FA; ≥ 28 carbons) in the brain are unknown. The enzyme ELOngation of Very Long-chain fatty acids-4 (ELOVL4) catalyzes the rate-limiting step in the biosynthesis of VLC-FA (Agbaga et al.,...

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Udgivet i:Mol Neurobiol
Main Authors: Hopiavuori, Blake R., Deák, Ferenc, Wilkerson, Joseph L., Brush, Richard S., Rocha-Hopiavuori, Nicole A., Hopiavuori, Austin R., Ozan, Kathryn G., Sullivan, Michael T., Wren, Jonathan D., Georgescu, Constantin, Szweda, Luke, Awasthi, Vibhudutta, Towner, Rheal, Sherry, David M., Anderson, Robert E., Agbaga, Martin-Paul
Format: Artigo
Sprog:Inglês
Udgivet: Springer US 2017
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Article
Online adgang:https://ncbi.nlm.nih.gov/pmc/articles/PMC5820379/
https://ncbi.nlm.nih.gov/pubmed/29168048
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1007/s12035-017-0824-8
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