Article
Homozygous Expression of Mutant ELOVL4 Leads to Seizures and Death in a Novel Animal Model of Very Long-Chain Fatty Acid Deficiency.
Molecular neurobiology - 1 Feb 2018
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
Abstract excerpt
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., Proc Natl Acad Sci USA 105(35): 12843-12848, 2008; Logan et al., J Lipid Res 55(4): 698-708, 2014), which we...
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