Catalog

Mucopolysaccharidosis type 3

No MMS code

Link to WHO Definition

Mucopolysaccharidosis type 3 – http://id.who.int/icd/entity/1477250013

Mucopolysaccharidosis type III (MPS III) is a lysosomal storage disease belonging to the group of mucopolysaccharidoses and characterised by severe and rapid intellectual deterioration. Deficiencies in one of the four enzymes required for heparan sulphate degradation are responsible for each of the MPS III subtypes: heparan sulfamidase for MPS IIIA, alpha-N-acetylglucosaminidase for MPS IIIB, alpha-glucosaminide N-acetyltransferase for MPS IIIC, and N-acetylglucosamine-6-sulfate sulfatase for MPS IIID.