Problem
Maroteaux-Lamy syndrome
Other Names:
Mucopolysaccharidosis VI Nature:
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 Problem Type:
G: Very specific problems Date of last update
03.02.2021 – 17:30 CET