MPS VI, also known as Maroteaux-Lamy syndrome, is caused by complete or partial loss of activity of the enzyme N-acetyl-galactosamine-4-sulfatase (arylsulfatase B, ARSB), which is involved in the breakdown of two different GAGs: dermatan sulfate and chondroitin 4-sulfate [6, 7]. This evidence concerns the gene ARSB and mucopolysaccharidosis type 6.