Fig. 3.

W246G mutant makes significantly higher amounts of 32 carbon VLC-PUFA in SCA34 homozygous mutant retinas. A: Relative mole % of VLC-PUFA levels in 12-month-old WT, heterozygous (HET), and homozygous (Mut) rat retinas with knock-in of the W246G mutation, analyzed by GC. B: ELOVL4 protein expression in 12-month-old WT, heterozygous (HET), and homozygous (Mut) rats with knock-in of the W246G mutation. Tissue lysates were collected for Western blot analysis with beta-tubulin as loading control. Results are the mean ± SD (n = 3). Statistical significance was assessed by ANOVA with Tukey’s post hoc test. ∗P < 0.05; ∗∗P < 0.01, ns not significant in comparison with WT. ELOVL, elongation of very long-chain fatty acid; SCA, spinocerebrellar ataxia; VLC-PUFA, very long chain-PUFA.