mucopolysaccharidosis vi

Maroteaux–Lamy syndrome, or Mucopolysaccharidosis Type VI (MPS-VI), is an inherited disease caused by a deficiency in the enzyme ASRB (arylsulfatase B). ASRB is responsible for the breakdown of large sugar molecules called glycosaminoglycans (AKA GAGs, or mucopolysaccharides). In particular, ARSB breaks down dermatan sulfate and chondroitin sulfate. Because people with MPS-VI lack the ability to break down these GAGs, these chemicals build up in the lysosomes of cells. MPS-VI is therefore a type ...