Mucopolysaccharidosis VI (MPS VI) - overview

Overview | FAQs | Lysosomal storage disorders

Mucopolysaccharidosis VI, also known as Maroteaux-Lamy syndrome, is the deficiency of an enzyme necessary for the processing of certain complex carbohydrates known as glycosaminoglycans (GAG). Without that enzyme, cells are unable to excrete waste carbohydrates, which then accumulate in the lysosomes of the cell. MPS VI is inherited and occurs equally among males and females. The progression of the disease varies between individuals, and different organs may be affected. Unlike MPS I, MPS VI doesn’t usually erode intelligence.

Standard treatment is with Naglazyme®.