Deficiency of the enzyme arylsulfatase B (ARSB; N-acetylgalactosamine-4-sulfatase) leads to the lysosomal storage disease mucopolysaccharidosis (MPS) VI (Maroteaux-Lamy-Syndrome), which is associated with accumulation of the sulfated glycosoaminoglycans chondroitin-4-sulfate (C4S) and dermatan sulfate (DS). The gene discussed is ARSB; the disease is lysosomal storage disease.