Sulfamidase/SGSH Antibody (1018387) [Unconjugated]

Sulfamidase/SGSH was detected in immersion fixed HT-29 human colon adenocarcinoma cell line (positive stain) and U937 human histiocytic lymphoma cell line (negative stain) using Mouse Anti-Human Sulfamidase/SGSH Monoclonal Antibody (Catalog # MAB8380) at 8 µg/mL for 3 hours at room temperature. Cells were stained using the NorthernLights™ 557-conjugated Anti-Mouse IgG Secondary Antibody (red; Catalog # NL007) and counterstained with DAPI (blue). Specific staining was localized to cytoplasm. View our protocol for Fluorescent ICC Staining of Cells on Coverslips.

• Reactivity: Hu
• Applications: ICC/IF
• Clone: 1018387
• Clonality: Monoclonal
• Host: Mouse
• Conjugate: Unconjugated

Sulfamidase/SGSH Antibody (1018387) [Unconjugated] Summary

• Immunogen: Spodoptera frugiperda, Sf 21 (baculovirus)-derived human Sulfamidase/SGSH protein
  Arg23-Leu502
  Accession # P51688
• Specificity: Detects human Sulfamidase/SGSH in direct ELISAs.
• Source: N/A
• Isotype: IgG2a
• Clonality: Monoclonal
• Host: Mouse
• Purity Statement: Protein A or G purified from hybridoma culture supernatant

Applications/Dilutions

• Dilutions:
  • Immunocytochemistry 8-25 ug/mL

Packaging, Storage & Formulations

• Storage: Use a manual defrost freezer and avoid repeated freeze-thaw cycles.
  • 12 months from date of receipt, -20 to -70 °C as supplied.
  • 1 month, 2 to 8 °C under sterile conditions after reconstitution.
  • 6 months, -20 to -70 °C under sterile conditions after reconstitution.
• Buffer: Lyophilized from a 0.2 μm filtered solution in PBS with Trehalose. *Small pack size (SP) is supplied either lyophilized or as a 0.2 µm filtered solution in PBS.
• Reconstitution Instructions: Reconstitute at 0.5 mg/mL in sterile PBS.

Notes

This product is produced by and ships from R&D Systems, Inc., a Bio-Techne brand.

Alternate Names for Sulfamidase/SGSH Antibody (1018387) [Unconjugated]

• heparan sulfate sulfatase
• HSS
• HSSN-sulphoglucosamine sulphohydrolase
• MPS3A
• MPS3Asulphamidase
• N-sulfoglucosamine sulfohydrolase
• SFMD
• SFMDEC 3.10.1.1
• SGSH
• Sulfamidase
• Sulfoglucosamine sulfamidase
• Sulphamidase

Background

Also known as N-sulfoglucosamine sulfohydrolase and heparan N-sulfatase, Sulfamidase/SGSH is an important member of the sulfatase family involved in the degradation of heparan sulfate (HS) (1). Different from the HS specific endosulfatases that remove sulfate from internal GlcNAc residues (2), SGSH removes sulfate group from the non-reducing end glucosamine residues on HS. The SGSH deficiency results in mucopolysaccharidosis type IIIA (MPS IIIA, Sanfilippo A syndrome), an autosomal recessive lysosomal storage disease characterized by neurological dysfunction but relatively mild somatic manifestations (3). Human SGSH shows 88.6% sequence identity with that of mouse sequence.

1. DiezRoux, G. and Ballabio, A. et al. (2005) Annu. Rev. Genomics Hum. Genet. 6:355.
2. Morimoto-Tomita, M. et al. (2002) J. Biol. Chem. 277:49175.
3. Blanch, L. et al. (1997) Hum. Mol. Genet. 6:787.

Limitations

This product is for research use only and is not approved for use in humans or in clinical diagnosis. Primary Antibodies are guaranteed for 1 year from date of receipt.

Bioinformatics

• Uniprot:
  • P51688()