Sulfamidase/SGSH Antibody (1018331) [Alexa Fluor® 350]

• Reactivity: Hu, Mu
• Applications: WB, IHC
• Clone: 1018331
• Clonality: Monoclonal
• Host: Mouse
• Conjugate: Alexa Fluor 350

Sulfamidase/SGSH Antibody (1018331) [Alexa Fluor® 350] Summary

• Immunogen: Spodoptera frugiperda, Sf 21 (baculovirus)-derived human Sulfamidase/SGSH
  Arg23-Leu502
  Accession # P51688
• Specificity: Detects human Sulfamidase/SGSH in direct ELISAs. Detects human and mouse Sulfamidase/SGSH in Western blots.
• Isotype: IgG2a
• Clonality: Monoclonal
• Host: Mouse
• Purity Statement: Protein A or G purified

Applications/Dilutions

• Dilutions:
  • Immunohistochemistry
  • Western Blot

Packaging, Storage & Formulations

• Storage: Protect from light. Do not freeze. 12 months from date of receipt, 2 to 8 °C as supplied
• Buffer: Supplied 0.2mg/ml in 1X PBS with RDF1 and 0.09% Sodium Azide

Notes

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

Alternate Names for Sulfamidase/SGSH Antibody (1018331) [Alexa Fluor® 350]

• 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.

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.