Heterozygous inheritance of several different ELOVL4 mutations in exon 6 that cause early truncation of the ELOVL4 protein and loss of the ER-retention signal in the C-terminus leads to autosomal dominant Stargardt-like macular dystrophy (STGD3), an aggressive juvenile-onset macular degeneration [8–11]. This evidence concerns the gene ELOVL4 and Stargardt disease.