Recombinant Human SGSH, GST-tagged

• Cat.No.: SGSH-280H
• Product Overview: Recombinant Human SGSH (1 a.a. - 502 a.a.) fused with GST-tag at N-terminal, was expressed in vitro wheat germ expression system.

Specification

• Cat. No.: SGSH-280H
• 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.
• Source: wheat germ
• Molecular Mass: 83.1 kDa
• Sequence: MSCPVPACCALLLVLGLCRARPRNA LLLLADDGGFESGAYNNSAIATPHL DALARRSLLFRNAFTSVSSCSPSRA SLLTGLPQHQNGMYGLHQDVHHFNS FDKVRSLPLLLSQAGVRTGIIGKKH VGPETVYPFDFAYTEENGSVLQVGR NITRIKLLVRKFLQTQDDRPFFLYV AFHDPHRCGHSQPQYGTFCEKFGNG ESGMGRIPDWTPQAYDPLDVLVPYF VPNTPAARADLAAQYTTVGRMDQGV GLVLQELRDAGVLNDTLVIFTSDNG IPFPSGRTNLYWPGTAEPLLVSSPE HPKRWGQVSEAYVSLLDLTPTILDW FSIPYPSYAIFGSKTIHLTGRSLLP ALEAEPLWATVFGSQSHHEVTMSYP MRSVQHRHFRLVHNLNFKMPFPIDQ DFYVSPTFQDLLNRTTAGQPTGWYK DLRHYYYRARWELYDRSRDPHETQN LATDPRFAQLLEMLRDQLAKWQWET HDPWVCAPDGVLEEKLSPQCQPLHN EL
• Storagebuffer: 50 mM Tris-HCI, 10 mM reduced Glutathione, pH 8.0 in the elution buffer.
• Applications: ELISA; WB
• Storage: Store at -80°C. Aliquot to avoid repeated freezing and thawing.
• OfficialSymbol: SGSH

Gene Information

• Gene Name: SGSH N-sulfoglucosamine sulfohydrolase [ Homo sapiens ]
• Synonyms: SGSH; N-sulfoglucosamine sulfohydrolase; HSS; MPS3A; mucopolysaccharidosis type IIIA; SFMD; sulfamidase; heparan sulfate sulfatase; sulfoglucosamine sulfamidase; N-sulphoglucosamine sulphohydrolase; EC 3.10.1.1; Sulfoglucosamine sulfamidase; Sulphamidase
• Gene ID: 6448
• mRNA Refseq: NM_000199
• Protein Refseq: NP_000190
• MIM: 605270
• UniProt ID: P51688
• Chromosome Location: 17q25.3
• Pathway: Glycosaminoglycan degradation; Heparan sulfate degradation; Lysosome; MPS I - Hurler syndrome
• Function: N-sulfoglucosamine sulfohydrolase activity; catalytic activity; metal ion binding; sulfuric ester hydrolase activity