Recombinant Human SGSH

• Cat.No.: SGSH-30971TH
• Product Overview: Recombinant full length Human SGSH with proprietary N terminal tag; Predicted MW 81.29 kDa.

Specification

• Description: This gene encodes one of several enzymes involved in the lysosomal degradation of heparan sulfate. Mutations in this gene are associated with Sanfilippo syndrome A, one type of the lysosomal storage disease mucopolysaccaridosis III, which results from impaired degradation of heparan sulfate. Transcripts of varying sizes have been reported but their biological validity has not been determined.
• Protein length: 502 amino acids
• Molecular Weight: 81.290kDa inclusive of tags
• Source: Wheat germ
• Form: Liquid
• Purity: Proprietary Purification
• Storage buffer: pH: 8.00Constituents:0.3% Glutathione, 0.79% Tris HCl
• Storage: Shipped on dry ice. Upon delivery aliquot and store at -80oC. Avoid freeze / thaw cycles.
• Sequences of amino acids: MSCPVPACCALLLVLGLCRARPRNALLLLADDGGFESGAY NNSAIATPHLDALARRSLLFRNAFTSVSSCSPSRASLLTG LPQHQNGMYGLHQDVHHFNSFDKVRSLPLLLSQAGVRTGI IGKKHVGPETVYPFDFAYTEENGSVLQVGRNITRIKLLVR KFLQTQDDRPFFLYVAFHDPHRCGHSQPQYGTFCEKFGNG ESGMGRIPDWTPQAYDPLDVLVPYFVPNTPAARADLAAQY TTVGRMDQGVGLVLQELRDAGVLNDTLVIFTSDNGIPFPS GRTNLYWPGTAEPLLVSSPEHPKRWGQVSEAYVSLLDLTP TILDWFSIPYPSYAIFGSKTIHLTGRSLLPALEAEPLWAT VFGSQSHHEVTMSYPMRSVQHRHFRLVHNLNFKMPFPIDQ DFYVSPTFQDLLNRTTAGQPTGWYKDLRHYYYRARWELYD RSRDPHETQNLATDPRFAQLLEMLRDQLAKWQWETHDPWV CAPDGVLEEKLSPQCQPLHNEL
• Sequence Similarities: Belongs to the sulfatase family.

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
• 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
• Function: N-sulfoglucosamine sulfohydrolase activity; catalytic activity; hydrolase activity; metal ion binding; sulfuric ester hydrolase activity