The GUSB gene provides instructions for producing an enzyme called beta-glucuronidase (β-glucuronidase). This enzyme is located in lysosomes, compartments within cells that digest and recycle different types of molecules. β-glucuronidase is involved in the 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. β-glucuronidase is involved in the break down of three types of GAGs: dermatan sulfate, heparan sulfate, and chondroitin sulfate. This enzyme removes a sugar called glucuronic acid when it is at the end of the GAG chain.
Other Names
beta-D-glucuronidase; beta-G1; beta-glucuronidase; beta-glucuronidase precursor; BG; BGLR_HUMAN; FLJ39445; glucuronidase, beta; MPS7
Related Genetics Topics
Identifiers
- NCBI Gene: 2990
- OMIM: 611499
Source: MedlinePlus Genetics, U.S. National Library of Medicine.
