Sulfamidase/SGSH Antibody [Alexa Fluor™ Plus 594]

• Reactivity: Mu
• Clonality: Polyclonal
• Host: Goat
• Conjugate: Alexa Fluor Plus 594

Sulfamidase/SGSH Antibody [Alexa Fluor™ Plus 594] Summary

• Specificity: Detects mouse Sulfamidase/SGSH in direct ELISAs and Western blots.
• Isotype: IgG
• Clonality: Polyclonal
• Host: Goat

Packaging, Storage & Formulations

• Storage: Protect from light. Do not freeze. 12 months from date of receipt, 2 to 8 °C as supplied
• Buffer: Supplied 0.2 mg/mL in a saline solution containing BSA and 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 [Alexa Fluor™ Plus 594]

• 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 (1). 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 (2). The deduced amino acid sequence of mouse SGSH consists of a signal peptide (residues 1‑22) and a mature chain (residues 23‑502) (1). rmSGSH corresponds to the mature chain and has sulfatase activity.

1. Diez-Roux, G. and Ballabio, A. (2005) Annu. Rev. Genomics Hum. Genet. 6:355.
2. Gliddon, B.L. et al. (2004) Mol. Genet. Metab. 83:239.

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.