Human alpha ‑N‑acetylgalactosaminidase/NAGA Antibody

Catalog #: AF6717 Datasheet / COA / SDS

Catalog #	Availability	Size / Price	Qty
AF6717
AF6717-SP

Detection of Human alpha ‑N‑acetylgalactosaminidase/NAGA by Western Blot.

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Human alpha ‑N‑acetylgalactosaminidase/NAGA 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 alpha ‑N‑acetylgalactosaminidase/NAGA
Leu18-Gln411
Accession # P17050

Formulation

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.

Label

Unconjugated

Applications

Recommended Concentration

Sample

Western Blot

1 µg/mL

See below

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.

Scientific Data

Western Blot

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Detection of Human alpha ‑N‑acetylgalactosaminidase/NAGA by Western Blot. Western blot shows lysates of human placenta tissue. PVDF membrane was probed with 1 µg/mL of Sheep Anti-Human a-N-acetylgalactosaminidase/NAGA Antigen Affinity-purified Polyclonal Antibody (Catalog # AF6717) followed by HRP-conjugated Anti-Sheep IgG Secondary Antibody (Catalog # HAF016). A specific band was detected for a-N-acetylgalactosaminidase/NAGA at approximately 45 kDa (as indicated). This experiment was conducted under reducing conditions and using Immunoblot Buffer Group 1.

Reconstitution Calculator

Preparation and Storage

Reconstitution

Sterile PBS to a final concentration of 0.2 mg/mL.

Shipping

Lyophilized product is shipped at ambient temperature. Liquid small pack size (-SP) is shipped with polar packs. Upon receipt, store immediately at the temperature recommended below.

Stability & 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.

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).

References

Wang, A.M. et al. (1990) J. Biol. Chem. 265:21859.
Sweeley, C.C. et al. (1983) Arch. Biochem. Biophys. 223:158.
Garman, S.C. et al. (2002) Structure. 10:425.
Eng, C.M. et al. (2001) N. Engl. J. Med. 345:9.
Wang, A.M. et al. (1990) J. Clin. Invest. 86:1752.
Liu, Q.P. et al. (2007) Nature Biotechnol. 25:454.
Calcutt, M. J. et al. (2002) FEMS Microbiol. Lett. 214:77.
Zhu, A. et al. (1996) Arch. Biochem. Biophys. 327:324.

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