Human alpha ‑N‑acetylgalactosaminidase/NAGA Alexa Fluor® 488-conjugated Antibody

Catalog # Availability Size / Price Qty
AF6717G-100UG

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Human alpha ‑N‑acetylgalactosaminidase/NAGA Alexa Fluor® 488-conjugated Antibody Summary

Species Reactivity
Human
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.
Source
Polyclonal Sheep IgG
Purification
Antigen Affinity-purified
Immunogen
Chinese hamster ovary cell line recombinant human α‑N‑acetylgalactosaminidase/NAGA
Leu18-Gln411
Accession # P17050
Formulation
Supplied 0.2mg/ml in 1X PBS with RDF1 and 0.09% Sodium Azide
Label
Alexa Fluor 488 (Excitation= 488 nm, Emission= 515-545 nm)

Applications

Recommended Concentration
Sample
Western Blot
Optimal dilution of this antibody should be experimentally determined.
 

Please Note: Optimal dilutions should be determined by each laboratory for each application. General Protocols are available in the Technical Information section on our website.

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Preparation and Storage

Shipping
The product is shipped with polar packs. Upon receipt, store it immediately at the temperature recommended below.
Stability & Storage
Protect from light. Do not freeze. 12 months from date of receipt, 2 to 8 °C as supplied

Background: alpha-N-acetylgalactosaminidase/NAGA

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).

Entrez Gene IDs
4668 (Human); 17939 (Mouse); 315165 (Rat)
Alternate Names
alpha-N- (alpha-galactosidase B); alphaNacetylgalactosaminidase; alpha-N-acetylgalactosaminidase; EC 3.2.1; EC 3.2.1.49; GALB; N-acetylgalactosaminidase, alpha-; NAGA

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