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Specifications
- Antibody Type:Primary
- Antigen Name:N-sulfoglucosamine sulfohydrolase
- Antigen Symbol:SGSH
- Clonality:Polyclonal
- Conjugation:Cy5®
- Host:Rabbit
- ImmunoChemistry:Yes
- Isotype:IgG
- Reactivity:Human,Rat,Mouse
- Size:100 µL
- Environmentally Preferable:
- Cross Adsorption:No
- Form:liquid
- Gene ID:6448
- Antigen Synonyms:Sulphamidase|HSS|SFMD|MPS3A
- Storage Buffer:Aqueous buffered solution containing 100ug/ml BSA, 50% glycerol and 0.09% sodium azide. Store at 4°C for 12 months.
- Storage Temperature:Store at 4°C for 12 months
- Concentration:1 μg/μl
- Shipping Temperature:4°C
- Immunogen:310-360/502
- Purification:Purified by Protein A
- Cat. No.:10266-444
- Supplier no.:BS-11756R-CY5
Specifications
About this item
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.
Recommended Dilutions: IF(IHC-P): 1:50-200
Type: Primary
Antigen: SGSH
Clonality: Polyclonal
Clone:
Conjugation: Cy5®
Epitope:
Host: Rabbit
Isotype: IgG
Reactivity: Human, Mouse, Rat