Inherited mutations of ARSB are the cause of Mucopolysaccharidosis (MPS) VI (Maroteaux-Lamy-Syndrome; phenotype MIM number is 253200) [6,7,8,9,10,11], which is characterized by the accumulation of dermatan sulfate and chondroitin 4-sulfate (C4S) throughout the body. This evidence concerns the gene ARSB and mucopolysaccharidosis type 6.