Human beta-Glucuronidase/GUSB PharmaGenie ELISA Kit (SBRS0302)

Built around a human-specific capture antibody, the Assay Genie kit quantitatively measures beta-glucuronidase (GUSB), a lysosomal acid hydrolase that cleaves glucuronic acid residues from glycosaminoglycans during extracellular matrix turnover. Loss-of-function mutations in GUSB underlie mucopolysaccharidosis type VII (Sly syndrome), and the assay is widely used to track enzyme replacement therapy responses, characterize patient-derived fibroblasts releasing enzyme into cell culture supernatant, and benchmark recombinant GUSB activity in plasma and serum specimens from treated individuals. Each kit ships with a pre-coated microplate, biotinylated detection antibody, streptavidin-HRP conjugate, recombinant human GUSB standard, assay diluent, wash buffer, and TMB chromogenic substrate. Pipette 50 uL of sample and standards in duplicate, incubate, wash, develop color, and read absorbance to interpolate concentration from the standard curve. Reconstituted reagents and unused strip wells should be stored refrigerated at 4 degrees C, protected from light, and not subjected to more than two freeze-thaw cycles.