|Other Names||N-sulphoglucosamine sulphohydrolase, Sulfoglucosamine sulfamidase, Sulphamidase, SGSH, HSS|
|Format||Synthetic peptide was lyophilized with 100% acetonitrile and is supplied as a powder. Reconstitute with 0.1 ml DI water for a final concentration of 1 mg/ml.|
|Storage||Maintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C.|
|Precautions||This product is for research use only. Not for use in diagnostic or therapeutic procedures.|
|Function||Catalyzes a step in lysosomal heparan sulfate degradation.|
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Provided below are standard protocols that you may find useful for product applications.
SGSH is 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.
??leat, D.E., et al. Mol. Cell Proteomics 5(4):686-701(2006)??ekri, S., et al. J. Inherit. Metab. Dis. 28(4):601-602(2005)??uschol, N., et al. Hum. Mutat. 23(6):559-566(2004)??hang, H., et al. Nat. Biotechnol. 21(6):660-666(2003)
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