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. 2020 Aug 11;57(11):4735–4753. doi: 10.1007/s12035-020-02052-8

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© The Author(s) 2020

Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/.

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Fig. 3 — W246G ELOVL4 retains the ability to synthesize VLC-PUFA. Analysis of retinal glycerophospholipids from WT, HET, and MUT SCA34-KI rats shows that the W246G mutant form of ELOVL4 retains the capacity to synthesize VLC-PUFA at normal levels. No differences in total retinal levels of VLC-PUFA were present among WT, HET, and MUT rats. a VLC-PUFA were detected specifically in the phosphatidylcholine fraction (PC), but total VLC-PUFA levels showed no differences among WT, HET, and MUT rat retinas. However, significant differences were detected in non-VLC-FA (PC 34:01 and PC 40:06) among genotypes. b No VLC-PUFA were detected in the phosphatidylethanolamine (PE) fraction of WT, HET, or MUT rat retina. However, statistically significant differences were detected in PE 40:06 and PC 44:12. c No VLC-PUFA were detected in the phosphatidylserine (PS) fraction of WT, HET, or MUT rat retina. No significant differences were detected in any lipid species in the PS fraction. (Analysis by 1-way ANOVA with Tukey’s post hoc test. Data shown as mean ± St. Dev. *, MUT differs from WT; $, HET differs from WT; #, MUT differs from WT. See Supplemental Tables 1–3 for details)