ELOVL4 and retinal degeneration: Based on the tissue-specific distribution of VLC-FA, with VLC-PUFA enriched in the retina and VLC-SFA predominantly in the brain and skin (7, 8, 10, 17), patients with SCA34 pathology without any reported retinal degeneration, as in those carrying the L168F and W246G ELOVL4 mutations (30, 31), may have an alteration in the quantities and types of VLC-SFAs produced.