MyBioSource.com's Rabbit anti-human SGSH polyclonal Antibody is a Rabbit Polyclonal antibody. This antibody has been shown to work in applications such as: ELISA, and Immunohistochemistry. The Rabbit anti-human SGSH polyclonal Antibody was generated using SGSH as the antigen and it reacts with Human.
Description
SGSH(N-sulphoglucosamine sulphohydrolase) is also named as HSS, sulphamidase and belongs to the sulfatase family. Sulfamidase is synthesized as a 62 kDa precursor protein, which is modified with mannose 6-phosphate (M6P) residues, allowing their recognition by mannose-6-phosphate receptors in the Golgi complex and ensuring transport to the endosomal/lysosomal system. It catalyzes the third step of degradation of glucosaminoglycans and is required for the degradation of heparan sulphate. Defects in SGSH are the cause of mucopolysaccharidosis type 3A (MPS3A), also known as Sanfilippo syndrome A