The SGSH gene provides instructions for producing an enzyme called sulfamidase. This enzyme is located in lysosomes, compartments within cells that digest and recycle different types of molecules. Sulfamidase is involved in the step-wise breakdown of large molecules called glycosaminoglycans (GAGs). GAGs are composed of sugar molecules that are linked together to form a long string. To break down these large molecules, individual sugars are removed one at a time from one end of the molecule. Sulfamidase removes a chemical group known as a sulfate from a sugar called glucosamine when it is at the end of the GAG chain.
Other Names
heparan N-sulfatase; heparan sulfate sulfatase; HSS; N-sulphoglucosamine sulphohydrolase; N-sulphoglucosamine sulphohydrolase precursor; SFMD; SPHM_HUMAN; sulfamidase; sulfoglucosamine sulfamidase; sulphamidase
Related Genetics Topics
Identifiers
- NCBI Gene: 6448
- OMIM: 605270
Source: MedlinePlus Genetics, U.S. National Library of Medicine.
