Mutations in ELOVL4 are known to cause mislocalization and aggregation of ELOVL4 from the endoplasmic reticulum to other organelles in photoreceptors leading to STGD3.275,276 ELOVL4 is a fatty acid elongase that preferentially uses eicosapentaenoic acid (EPA) as a substrate to generate very-long-chain polyunsaturated fatty acids (VLC-PUFAs).277 To model STGD3, researchers identified a 5 base pair deletion in ELOVL4 of a STGD3 patient195 and generated transgenic mice expressing ELOVL4 with either the same 5 base pair deletion in human ELOVL4175 or mouse Elovl4. The gene discussed is ELOVL4; the disease is Stargardt disease 3.