Recombinant Full Length Human SGSH Protein, GST-tagged

• Cat.No.: SGSH-474HF
• Product Overview: Recombinant full length Human SGSH (1 a.a. - 502 a.a.) fused with GST-tag at N-terminal, was expressed in vitro wheat germ expression system.

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.
• Source: In Vitro Cell Free System
• Species: Human
• Tag: GST
• Molecular Mass: 83.1 kDa
• Protein Length: 502 amino acids
• AA Sequence: MSCPVPACCA LLLVLGLCRA RPRNALLLLA DDGGFESGAY NNSAIATPHL DALARRSLLF RNAFTSVSSC SPSRASLLTG LPQHQNGMYG LHQDVHHFNS FDKVRSLPLL LSQAGVRTGI IGKKHVGPET VYPFDFAYTE ENGSVLQVGR NITRIKLLVR KFLQTQDDRP FFLYVAFHDP HRCGHSQPQY GTFCEKFGNG ESGMGRIPDW TPQAYDPLDV LVPYFVPNTP AARADLAAQY TTVGRMDQGV GLVLQELRDA GVLNDTLVIF TSDNGIPFPS GRTNLYWPGT AEPLLVSSPE HPKRWGQVSE AYVSLLDLTP TILDWFSIPY PSYAIFGSKT IHLTGRSLLP ALEAEPLWAT VFGSQSHHEV TMSYPMRSVQ HRHFRLVHNL NFKMPFPIDQ DFYVSPTFQD LLNRTTAGQP TGWYKDLRHY YYRARWELYD RSRDPHETQN LATDPRFAQL LEMLRDQLAK WQWETHDPWV CAPDGVLEEK LSPQCQPLHN EL
• Applications: ELISA; WB
• Storage: Store at -80 centigrade. Aliquot to avoid repeated freezing and thawing.
• Storage Buffer: 50 mM Tris-HCl, 10 mM reduced Glutathione, pH=8.0 in the elution buffer.

Gene Information

• Gene Name: SGSH N-sulfoglucosamine sulfohydrolase [ Homo sapiens ]
• Official Symbol: SGSH
• 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