Lysinuric protein intolerance (LPI) (OMIM #222700) is caused by mutations in the gene coding for solute carrier family 7A member 7 (SLC7A7) located at chromosome 14q11.2, resulting in defective dibasic amino acid (lysine, arginine, ornithine) transport at the epithelial cells in the kidney and intestine [128]. Here, SLC7A7 is linked to lysinuric protein intolerance.