N-Acetylglucosaminidase, alpha (NAGLU) antibody

Antigen: N-Acetylglucosaminidase, alpha (NAGLU)

Synonyms: NAG, MPS3B, UFHSD, MPS-IIIB, NAGLU

Clonality: Monoclonal (1B7)

Host: Mouse

Reactivity: Human

Application: ELISA, Western Blotting (WB)

Certificates: ISO 9001:2008

Catalog no.: ABIN394860

Additional Information

Alternative name: NAGLU

Gene ID: 4669

UniProt: NP_000254

Immunogen: NAGLU (NP_000254, 644 a.a. ~ 743 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 K

Isotype: IgG2a kappa

Clone: 1B7

Description: Other names: NAG, UFHSD N-acetylglucosaminidase, alpha- (Sanfilippo disease IIIB)

Characteristics: Purified Mouse Monoclonal Antibody (Mab)

Specificity: NAGLU (NP_000254, 644 a.a. ~ 743 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.

Molecular Weight: 82167 DA

Comments:

Background: This gene encodes an enzyme that degrades heparan sulfate by hydrolysis of terminal N-acetyl-D-glucosamine residues in N-acetyl-alpha-D-glucosaminides. Defects in this gene are the cause of mucopolysaccharidosis type IIIB (MPS-IIIB), also known as Sanfilippo syndrome B. This disease is characterized by the lysosomal accumulation and urinary excretion of heparan sulfate.

Application Details

Application Notes: ELISA ~~ 1ug/ml~3ng/ml Western blot ~~ 1:500~1000

Purification: Purified

Buffer: Clear, colorless solution in phosphate buffered saline, pH 7.2 .

Storage: Maintain refrigerated at 2-8 deg C for up to 6 months. For long term storage store at -20 deg C in small aliquots to prevent freeze-thaw cycles

Research Area: Cell Structure

Restrictions: For Research Use only

Images

NAGLU monoclonal antibody (M02), clone 1B7. Western Blot analysis of NAGLU expression in Jurkat ( Cat # L017V1 ).

Publications

Product: Clark, Glanowski, Nielsen et al.: "Inferring nonneutral evolution from human-chimp-mouse orthologous gene trios." in: Science (New York, N.Y.), Vol. 302, Issue 5652, pp. 1960-3, 2003 (PubMed).

Gerhard, Wagner, Feingold et al.: "The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)." in: Genome research, Vol. 14, Issue 10B, pp. 2121-7, 2004 (PubMed).

Chinen, Tohma, Izumikawa et al.: "Sanfilippo type B syndrome: five patients with an R565P homozygous mutation in the alpha-N-acetylglucosaminidase gene from the Okinawa islands in Japan." in: Journal of human genetics, Vol. 50, Issue 7, pp. 357-9, 2005 (PubMed).

Mangas, Nogueira, Prata et al.: "Molecular analysis of mucopolysaccharidosis type IIIB in Portugal: evidence of a single origin for a common mutation (R234C) in the Iberian Peninsula." in: Clinical genetics, Vol. 73, Issue 3, pp. 251-6, 2008 (PubMed).

Champion, Basehore, Wood et al.: "Identification and characterization of a novel homozygous deletion in the alpha-N-acetylglucosaminidase gene in a patient with Sanfilippo type B syndrome (mucopolysaccharidosis IIIB)." in: Molecular genetics and metabolism, Vol. 100, Issue 1, pp. 51-6, 2010 (PubMed).