What is MPS VI?
MPS VI, known as Maroteaux-Lamy disease, is one of the mucopolysaccharide storage diseases. MPS VI was first identified by Dr Maroteaux and Dr Lamy in 1963.
Mucopolysaccharides are long chains of sugar molecules used in the building of bones, cartilage, skin, tendons and many other tissues in the body. In the course of normal life there is a continuous recycling process of building new mucopolysaccharides and breaking down old ones. The breakdown and recycling process requires a series of special biochemical tools called enzymes.
People with MPS VI are missing or are low in an enzyme called N-acetylgalactosamine-4-sulfatase, which is essential in breaking down mucopolysaccharides dermatan sulphate. When dermatan sulphate is not completely broken down it remains stored in the body and the symptoms of MPS VI occur.