Anti-SGSH Antibody

The SGSH gene encodes N-sulfoglucosamine sulfohydrolase, a lysosomal enzyme essential for the stepwise degradation of heparan sulfate. Loss-of-function mutations in SGSH are the molecular cause of mucopolysaccharidosis type IIIA (Sanfilippo syndrome type A), a progressive neurodegenerative lysosomal storage disorder marked by childhood-onset cognitive decline and behavioral disturbance.

This rabbit polyclonal antibody from Antibodies.com is raised against SGSH and validated for Western blot and immunohistochemistry workflows on human samples. Supplied as an unconjugated reagent in a 100 µl volume, it supports detection of endogenous sulfamidase expression in cell and tissue lysate preparations, aiding research into lysosomal storage disease biology, enzyme replacement therapy development, and the cellular pathophysiology of glycosaminoglycan accumulation.