| Overview |
| bs-11756R |
| SGSH/Sulphamidase Polyclonal Antibody |
| WB, ELISA, IHC-P, IHC-F, IF(IHC-P), IF(IHC-F), IF(ICC), ICC |
| Rat |
| Human, Mouse, Dog |
| Specifications |
| Unconjugated |
| Rabbit |
| KLH conjugated synthetic peptide derived from human Sulphamidase |
| 301-388/502 |
| Polyclonal |
| IgG |
| 1ug/ul |
| Purified by Protein A. |
| 0.01M TBS(pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| Shipped at 4°C. Store at -20°C for one year. Avoid repeated freeze/thaw cycles. |
| Target |
| 6448 |
| P51688 |
| Cytoplasm |
| HSS; SFMD; MPS3A; N-sulphoglucosamine sulphohydrolase; Sulfoglucosamine sulfamidase; Sulphamidase; SGSH |
| 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. |
| Application Dilution |
| WB |
1:300-5000 |
| ELISA |
1:500-1000 |
| IHC-P |
1:200-400 |
| IHC-F |
1:100-500 |
| IF(IHC-P) |
1:50-200 |
| IF(IHC-F) |
1:50-200 |
| IF(ICC) |
1:50-200 |
| ICC |
1:100-500 |
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