alpha-N-acetylgalactosaminidase/NAGA Antibody [Alexa Fluor® 700]

• Reactivity: Hu
• Applications: WB
• Clonality: Polyclonal
• Host: Sheep
• Conjugate: Alexa Fluor 700

alpha-N-acetylgalactosaminidase/NAGA Antibody [Alexa Fluor® 700] Summary

• Immunogen: Chinese hamster ovary cell line recombinant human α‑N‑acetylgalactosaminidase/NAGA
  Leu18-Gln411
  Accession # P17050
• Specificity: Detects human alpha ‑N‑acetylgalactosaminidase/NAGA in direct ELISAs and Western blots. In direct ELISAs, less than 2% cross-reactivity with recombinant human alpha -Galactosidase A is observed.
• Isotype: IgG
• Clonality: Polyclonal
• Host: Sheep
• Purity: Antigen Affinity-purified

Applications/Dilutions

• Dilutions:
  • 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
• Purity: Antigen Affinity-purified

Notes

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

Alternate Names for alpha-N-acetylgalactosaminidase/NAGA Antibody [Alexa Fluor® 700]

• alpha-N- (alpha-galactosidase B)
• alphaNacetylgalactosaminidase
• alpha-N-acetylgalactosaminidase
• EC 3.2.1
• EC 3.2.1.49
• GALB
• N-acetylgalactosaminidase, alpha-
• NAGA

Background

NAGA is a lysosomal alpha -N-acetylgalactosaminidase that cleaves non-reducing alpha -N-acetylgalactosaminyl moieties from glycoconjugates (1). Mature NAGA has 394 amino acids and is trafficked to the lysosome via the mannose-6-phosphate receptor‑mediated pathway (2). The enzyme is a retaining exoglycosidase, where both the substrate and product of the enzymatic reaction have the same anomeric configuration (3). Deficiency in NAGA results in increased urinary excretion and tissue accumulation of glycopeptides and oligosaccharides containing terminal alpha ‑N‑acetylgalactosaminyl moieties (4), manifesting as Schindler’s disease, an autosomal recessive disease with neuroaxonal dystrophy and other neurological symptoms (5). The enzyme can be used to remove alpha ‑N‑acetylgalactosaminyl residues present on red blood cells thus converting blood type A to blood type O (6, 7, 8).

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.