To better understand the role of ELOVL4 and its VLC-fatty acid products in the retina, we generated a novel knock-in rat model expressing the 736T>G (p.W246G) form of ELOVL4 that causes human SCA34 [35] and examined lipid composition, retinal function, and photoreceptor degeneration. The gene discussed is ELOVL4; the disease is spinocerebellar ataxia type 34.