The GUSB gene encodes beta-glucuronidase, a lysosomal hydrolase involved in the stepwise degradation of glucuronic acid-containing glycosaminoglycans (Shipley et al., 1993). It is a tetrameric glycoprotein composed of identical subunits (Oshima et al., 1987). The GUSB gene is mutated in mucopolysaccharidosis type VII.
