Human SGSH ELISA Kit (HUFI08483)

The SGSH (sulfoglucosamine sulfohydrolase) gene encodes a lysosomal sulfatase that removes sulfate groups from non-reducing-end glucosamine residues of heparan sulfate during glycosaminoglycan catabolism. Loss-of-function mutations cause Mucopolysaccharidosis type IIIA (Sanfilippo syndrome A), a progressive neurodegenerative lysosomal storage disorder. This Assay Genie kit enables quantitative measurement of human SGSH protein in serum or plasma, supporting research into MPS IIIA pathophysiology, enzyme replacement therapy monitoring, gene therapy efficacy, and biomarker development in patient cohorts.

Each kit contains a pre-coated microplate, biotinylated detection antibody, streptavidin-HRP conjugate, TMB substrate, wash buffer, and serial-dilution standards sufficient for 96 Assays. The protocol uses approximately 100 µl of human sample per well, with a total run time of roughly 4 hours after preparation. Component storage is 2-8°C; sealed plates must be kept dry with the desiccant pouch, the streptavidin-HRP should be protected from light, and reconstituted standards should be aliquoted to limit freeze-thaw cycles. The capture antibody is human-specific, with no significant cross-reactivity reported with homologous sulfatases or with rodent SGSH in validation work. For research use only; the kit is not validated for clinical diagnostic or therapeutic decision-making.