Mucopolysaccharidosis type VI [OMIM:253200], also known as Maroteaux–Lamy syndrome, is a progressive AR LSD with an estimated prevalence of 1/43,000–1,500,000 [624,625], caused by mutations affecting the ARSB gene on chromosome 5q14.1. The gene discussed is ARSB; the disease is mucopolysaccharidosis type 6.