SGSH/Sulphamidase Rabbit Polyclonal Antibody
No Size Price Availability  
YRP11761-01 50ul 1500.00 In Stock
YRP11761-01 100ul 2000.00 In Stock
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Source: Rabbit Gene Id: 6448
Isotype: IgG Swiss Prot: P51688
purity: Purified by Protein A.
Background:
Sulfatases are enzymes that hydrolyse a diverse range of sulfate esters. Deficiency of lysosomal sulfatases leads to human diseases characterized by the accumulation of either GAGs (glycosaminoglycans) or sulfolipids. Sulfamidase, also known as HSS, SFMD, MPS3A or SGSH, is a 502 amino acid lysosome that belongs to the sulfatase family. It has been suggested that sulfamidase may be involved in the lysosomal degradation of heparan sulfate. Defects in the gene encoding sulfamidase are the cause of Sanfilippo syndrome A, an autosomal recessive lysosomal storage disease caused by impaired degradation of heparan sulfate. Sanfilippo syndrome A is characterized by severe central nervous system degeneration but relatively mild somatic manifestations.
Reactivity Human, Mouse, Rat
Tested applications WB IHC IF
Clonality Polyclonal Antibody
Calculated MW /
Recommended Dilutions
WB 1:100-1:1000
IHC 1:100-1:500
IF 1:50-1:200
Immunogen KLH conjugated synthetic peptide derived from human Sulphamidase
Storage Aqueous buffered solution containing 100ug/ml BSA, 50% glycerol and 0.09% sodium azide. Store at -20℃ for 12 months
Synonym HSS, SFMD, MPS3A, N-sulphoglucosamine sulphohydrolase, Sulfoglucosamine sulfamidase, Sulphamidase, SGSH
This product is for research use only, not for diagnostic or therapeutic use!

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