|Application ||WB, E|
|Calculated MW||56695 Da|
|Antigen Region||420-449 aa|
|Other Names||N-sulphoglucosamine sulphohydrolase, Sulfoglucosamine sulfamidase, Sulphamidase, SGSH, HSS|
|Target/Specificity||This SGSH antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 420-449 amino acids from the C-terminal region of human SGSH.|
|Format||Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification.|
|Storage||Maintain refrigerated at 2-8°C for up to 2 weeks. For long term storage store at -20°C in small aliquots to prevent freeze-thaw cycles.|
|Precautions||SGSH Antibody (C-Term) is for research use only and 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.
?Sleat, D.E., et al. Mol. Cell Proteomics 5(4):686-701(2006)
?Bekri, S., et al. J. Inherit. Metab. Dis. 28(4):601-602(2005)
?Muschol, N., et al. Hum. Mutat. 23(6):559-566(2004)
?Zhang, H., et al. Nat. Biotechnol. 21(6):660-666(2003)
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