Active Recombinant Human SGSH protein, His-tagged

• Cat.No.: SGSH-22H
• Product Overview: Recombinant Human SGSH(Arg23-Leu502) fused with His tag at C-terminal was expressed in Insect cells.

Specification

• Description: Also known as N-sulfoglucosamine sulfohydrolase and heparan N-sulfatase, Sulfamidase/SGSH is an important member of the sulfatase family involved in the degradation of heparan sulfate (HS). Different from the HS specific endosulfatases that remove sulfate from internal GlcNAc residues, SGSH removes sulfate group from the non-reducing end glucosamine residues on HS. The SGSH deficiency results in mucopolysaccharidosis type IIIA (MPS IIIA, Sanfilippo A syndrome), an autosomal recessive lysosomal storage disease characterized by neurological dysfunction but relatively mild somatic manifestations. Human SGSH shows 88.6% sequence identity with that of mouse sequence.
• Source: Insect cells
• Species: Human
• Tag: His
• Predicted N Terminal: Arg23
• Form: Supplied as a 0.2 μm filtered solution in Tris and NaCl.
• Bio-activity: Measured by its ability to hydrolyze the substrate 4-Nitrocatechol Sulfate (PNCS). The specific activity is >0.3 pmol/min/μg.
• Molecular Mass: Predicted Molecular Mass: 55 kDa;SDS-PAGE: 55-63 kDa, reducing conditions.
• Endotoxin: <1.0 EU per 1 μg of the protein by the LAL method.
• Purity: >90%, by SDS-PAGE under reducing conditions and visualized by Colloidal Coomassie® Blue stain at 5 μg per lane.
• Storage: Avoid repeated freeze-thaw cycles.6 months from date of receipt, -20 to -70 centigrade as supplied.3 months, -20 to -70 centigrade under sterile conditions after opening.

Gene Information

• Gene Name: SGSH N-sulfoglucosamine sulfohydrolase [ Homo sapiens ]
• Official Symbol: SGSH
• Synonyms: SGSH; N-sulfoglucosamine sulfohydrolase; N-sulphoglucosamine sulphohydrolase; HSS; MPS3A; mucopolysaccharidosis type IIIA; SFMD; sulfamidase; sulphamidase; heparan sulfate sulfatase; sulfoglucosamine sulfamidase
• Gene ID: 6448
• mRNA Refseq: NM_000199
• Protein Refseq: NP_000190
• MIM: 605270
• UniProt ID: P51688
• Chromosome Location: 17q25.3
• Pathway: Glycosaminoglycan degradation, organism-specific biosystem; Glycosaminoglycan degradation, conserved biosystem; Heparan sulfate degradation, organism-specific biosystem; Heparan sulfate degradation, conserved biosystem; Lysosome, organism-specific biosystem; Lysosome, conserved biosystem; Metabolic pathways, organism-specific biosystem
• Function: N-sulfoglucosamine sulfohydrolase activity; catalytic activity; hydrolase activity; metal ion binding; sulfuric ester hydrolase activity