Anti-HSS/SGSH Antibody Picoband®

SKU: A04608-1

Size: 100 μg

Storage: At -20℃ for one year from date of receipt. After reconstitution, at 4℃ for one month. It can also be aliquotted and stored frozen at -20℃ for six months. Avoid repeated freezing and thawing.

Form: Lyophilized

Reactivity: Human,Mouse,Rat

Applications: WB,IHC,Flow Cytometry,ELISA

Application Details: Western blot, 0.25-0.5 μg/ml, Human, Mouse, Rat
Immunohistochemistry(Paraffin-embedded Section), 2-5 μg/ml, Human
Flow Cytometry (Fixed), 1-3 μg/1x10⁶ cells, Human
ELISA, 0.1-0.5 μg/ml, -

Gene Name: SGSH

Specificity:

Background: N-sulphoglucosamine sulphohydrolase is an enzyme that in humans is encoded by the SGSH gene. This gene encodes the enzyme sulfamidase; one of several enzymes involved in the lysosomal degradation of heparan sulfate. Mutations in this gene are associated with the lysosomal storage disease mucopolysaccaridosis IIIA, also known as Sanfilippo syndrome A, which results from impaired degradation of heparan sulfate. Transcripts of varying sizes have been reported but their biological validity has not been determined.

Immunogen: E.coli-derived human HSS/SGSH recombinant protein (Position: R206-L502).

Clonality: Polyclonal

Contents: Each vial contains 4 mg Trehalose, 0.9 mg NaCl, 0.2 mg Na2HPO4.

Purification: Immunogen affinity purified.

Reconstitution: Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.

Reference: 1. Bhattacharyya, R., Gliddon, B., Beccari, T., Hopwood, J. J., Stanley, P. A novel missense mutation in lysosomal sulfamidase is the basis of MPS IIIA in a spontaneous mouse mutant. Glycobiology 11: 99-103, 2001. 2. Bielicki, J., Hopwood, J. J., Melville, E. L., Anson, D. S. Recombinant human sulphamidase: expression, amplification, purification and characterization. Biochem. J. 329: 145-150, 1998. 3. Blanch, L., Weber, B., Guo, X.-H., Scott, H. S., Hopwood, J. J. Molecular defects in Sanfilippo syndrome type A. Hum. Molec. Genet. 6: 787-791, 1997.

Uniprot ID: P51688

Host: Rabbit

Concentration: Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.

Conjugate:

Cross Reactivity: No cross-reactivity with other proteins.

Isotype: Rabbit IgG

Phospho_site:

Clone Number:

Observed Molecular Weight: 57 kDa

Calculated Molecular Weight: 57 kDa

Gene ID: 6448

Protein Name: N-sulphoglucosamine sulphohydrolase

Gene Full Name: N-sulfoglucosamine sulfohydrolase

Synonyms: HSS; MPS3A; SFMD; SGSH; Sulfoglucosamine sulfamidase; Sulphamidase