Maroteaux-Lamy syndrome

Other Names:
Mucopolysaccharidosis VI

Maroteaux–Lamy syndrome, or Mucopolysaccharidosis Type VI (MPS-VI), is an inherited disease caused by a deficiency in the enzyme arylsulfatase B (ARSB). ASRB is responsible for the breakdown of large sugar molecules called glycosaminoglycans (GAGs, also known as 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 of lysosomal storage disease.

Broader Problems:
Mucopolysaccharide diseases
Medicine Pathology
Problem Type:
G: Very specific problems
Date of last update
03.02.2021 – 17:30 CET