- Tel: 858.663.9055
-
Email: info@nsjbio.com
- Tel: 858.663.9055
- Email: info@nsjbio.com
SGSH Antibody / N-sulphoglucosamine sulphohydrolase / HSS (RQ7802)
Email: info@nsjbio.com
| Catalog No | Formulation | Size | Price (USD) | ||
|---|---|---|---|---|---|
|
RQ7802 | 0.5mg/ml if reconstituted with 0.2ml sterile DI water | 100 ug | 449 |
| Availability | 1-3 business days |
| Species Reactivity | Human, Mouse, Rat |
| Format | Antigen affinity purified |
| Host | Rabbit |
| Clonality | Polyclonal (rabbit origin) |
| Isotype | Rabbit IgG |
| Purity | Antigen affinity purified |
| Buffer | Lyophilized from 1X PBS with 2% Trehalose |
| UniProt | P51688 |
| Localization | Cytoplasm (Lysosome) |
| Applications | Western Blot : 0.5-1ug/ml Immunohistochemistry (FFPE) : 2-5ug/ml Flow Cytometry : 1-3ug/million cells Direct ELISA : 0.1-0.5ug/ml |
| Limitations | This SGSH antibody is available for research use only. |
|
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SGSH antibody detects N-sulphoglucosamine sulphohydrolase (SGSH), also known as Heparan sulfate sulfatase (HSS), a lysosomal enzyme essential for the stepwise degradation of heparan sulfate glycosaminoglycans. The UniProt recommended name is N-sulfoglucosamine sulfohydrolase (SGSH). This enzyme catalyzes the hydrolysis of N-sulfate groups from glucosamine residues, representing an early and critical step in the lysosomal catabolism of heparan sulfate, a key component of the extracellular matrix and cell surface proteoglycans.
Functionally, SGSH antibody identifies a 502-amino-acid lysosomal hydrolase synthesized as a precursor that undergoes proteolytic processing into an active form. The enzyme functions optimally at acidic pH within lysosomes and requires post-translational modifications, including glycosylation, for correct folding and trafficking. SGSH is a member of the sulfatase family, whose coordinated activity prevents the accumulation of sulfated glycosaminoglycans. In healthy cells, SGSH ensures proper turnover of heparan sulfate, supporting normal cell signaling, endocytosis, and extracellular matrix maintenance.
The SGSH gene is located on chromosome 17q25.3 and is expressed in nearly all tissues, with particularly high activity in the brain, liver, and kidney. In the central nervous system, SGSH contributes to the clearance of heparan sulfate from neuronal and glial lysosomes, preserving normal neural function. Deficiency or mutation of the SGSH gene disrupts this degradation process, leading to the accumulation of undegraded heparan sulfate and lysosomal dysfunction.
Pathologically, mutations in SGSH cause Sanfilippo syndrome type A (Mucopolysaccharidosis type IIIA, MPS IIIA), a rare autosomal recessive lysosomal storage disorder characterized by progressive neurodegeneration, behavioral disturbances, and cognitive decline. Over 100 SGSH gene mutations have been identified, leading to partial or complete loss of enzymatic activity. The resulting buildup of heparan sulfate in neurons and other tissues triggers widespread cellular stress, inflammation, and impaired autophagy. There is currently no cure for MPS IIIA, although experimental therapies targeting gene replacement and enzyme delivery are under investigation.
Research using SGSH antibody supports studies in lysosomal function, glycosaminoglycan metabolism, and the molecular basis of MPS IIIA. SGSH antibody is validated for use in relevant research applications to detect N-sulphoglucosamine sulphohydrolase expression and study lysosomal degradation pathways and enzyme deficiencies. NSJ Bioreagents provides this antibody optimized for use in glycobiology, neurology, and metabolic disease research.
Optimal dilution of the SGSH antibody should be determined by the researcher.
E. coli-derived recombinant human protein (amino acids R206-L502) was used as the immunogen for the SGSH antibody.
After reconstitution, the SGSH antibody can be stored for up to one month at 4oC. For long-term, aliquot and store at -20oC. Avoid repeated freezing and thawing.
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