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Antigenic Specificity | Human SGSH |
Clone | polyclonal |
Host Species | Rabbit |
Reactive Species | human |
Isotype | n/a |
Format | antigen affinity purified |
Size | 0.05 mL, 0.1 mL |
Concentration | n/a |
Applications | ELISA (EIA), Immunohistochemistry (IHC) |
Reviews / Ratings | If you have used this antibody, please help fellow researchers by submitting reviews to pAbmAbs and antYbuddY. |
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. |
Immunogen | Immunogen: Recombinant human N-sulphoglucosamine sulphohydrolase protein (273-502aa) Santa Cruz Alternative: Potential replacement for Santa Cruz Biotechnology antibody catalog# sc-79741 / sc-79742 |
Other Names | [Sulfoglucosamine sulfamidase;Sulphamidase;SGSH;HSS], [SGSH; SGSH; HSS; SFMD; MPS3A; HSS] |
Gene, Accession # | [SGSH], Gene ID: 6448, NCBI: NP_000190.1, UniProt: P51688 |
Catalog # | MBS1497890 |
Price | $120, $160 |
Order / More Info | Human SGSH Antibody from MYBIOSOURCE INC. |
Product Specific References | n/a |