The GUSB gene encodes beta-glucuronidase (EC 3.2.1.31), a lysosomal hydrolase involved in the stepwise degradation of glucuronic acid-containing glycosaminoglycans (Shipley et al., 1993 [PubMed 7680524]). It is a tetrameric glycoprotein composed of identical subunits (Oshima et al., 1987 [PubMed 3468507]). The GUSB gene is mutated in mucopolysaccharidosis type VII (MPS7; MIM 253220).[supplied by OMIM]