To better understand skeletal compromise in MPS, we used as a model mucopolysaccharidosis type VI (MPS VI) or Maroteaux–Lamy syndrome, an MPS subtype caused by the deficiency of N-acetylgalactosamine 4-sulfatase (or Arylsulfatase B), a lysosomal enzyme involved in dermatan sulfate (DS) and chondroitin 4 sulfate (C4S) catabolism [1,17]. Here, ARSB is linked to mucopolysaccharidosis type 6.