Anti-SGSH/Sulphamidase Rabbit Polyclonal Antibody (Alexa Fluor® 680)

Catalog # BOSSBS-11756R-A680

Supplier: Bioss

Catalog # BOSSBS-11756R-A680

Supplier: Bioss

Specifications

Antibody type:
Primary
Antigen name:
SGSH/Sulphamidase
Clonality:
Polyclonal
Conjugation:
Alexa Fluor® 680
Host:
Rabbit
ImmunoChemistry:
Yes
ImmunoFluorescence:
Yes
Isotype:
IgG
Reactivity:
Rat
Western blot:
Yes
Epitope:
301-388/502
Form:
Liquid
Gene ID:
6448
Antigen synonyms:
Sulphamidase|N-sulphoglucosamine sulphohydrolase|Sulfoglucosamine sulfamidase|SGSH|HSS|SFMD|MPS3A
Modification:
Unmodified
Storage buffer:
Aqueous buffered solution containing 0,01 M TBS (pH 7,4) with 1% BSA, 0,03% Proclin300 and 50% Glycerol
Molecular weight:
55 kDa
Storage temperature:
Store at −20 °C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.
Concentration:
1 µg/µl
Shipping temperature:
4 °C
Immunogen:
KLH conjugated synthetic peptide derived from human Sulphamidase
Tested applications:
ICC
Purification:
Purified by Protein A
Pack type:
Vial
Pk:
100 µl

Specifications

About this item

Sulfatases are enzymes that hydrolyse a diverse range of sulfate esters. Deficiency of lysosomal sulfatases leads to human diseases characterised 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 characterised by severe central nervous system degeneration but relatively mild somatic manifestations.

Type: Primary
Antigen: SGSH/Sulphamidase
Clonality: Polyclonal
Clone:
Conjugation: ALEXA FLUOR® 680
Public Immunogen Range: 301-388/502
Host: Rabbit
Isotype: IgG
Reactivity: Rat