"SGSH" Related Products

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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.
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: MSCPVPACCALLLVLGLCRARPRNALLLLADDGGFESGAYNNSAIATPHLDALARRSLLFRNAFTSVSSCSPSRASLLTGLPQHQNGMYGLHQDVHHFNSFDKVRSLPLLLSQAGVRTGIIGKKHVGPETVYPFDFAYTEENGSVLQVGRNITRIKLLVRKFLQTQDDRPFFLYVAFHDPHRCGHSQPQYGTFCEKFGNGESGMGRIPDWTPQAYDPLDVLVPYFVPNTPAARADLAAQYTTVGRMDQGVGLVLQELRDAGVLNDTLVIFTSDNGIPFPSGRTNLYWPGTAEPLLVSSPEHPKRWGQVSEAYVSLLDLTPTILDWFSIPYPSYAIFGSKTIHLTGRSLLPALEAEPLWATVFGSQSHHEVTMSYPMRSVQHRHFRLVHNLNFKMPFPIDQDFYVSPTFQDLLNRTTAGQPTGWYKDLRHYYYRARWELYDRSRDPHETQNLATDPRFAQLLEMLRDQLAKWQWETHDPWVCAPDGVLEEKLSPQCQPLHNEL
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 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

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