Intriguingly, mutations in ARSB, encoding the lysosomal enzyme Arylsulfatase B, are associated with the recessive lysosome disorder, Mucopolysaccharidosis type VI (MPS VI, also called Maroteaux-Lamy syndrome), in which the glycosaminoglycan, dermatan sulfate, accumulates causing skeletal dysplasia and other heterogeneous manifestations [70]. Here, ARSB is linked to lysosomal storage disease.