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N-Acetylglucosaminidase, alpha (NAGLU) (AA 644-743) antibody

Details for Product No. ABIN394860, Supplier: Log in to see
Antigen
  • MPS-IIIB
  • MPS3B
  • NAG
  • UFHSD
Epitope
AA 644-743
4
4
2
2
2
1
Reactivity
Human
7
5
4
Host
Mouse
10
5
Clonality (Clone)
Monoclonal ()
Conjugate
Un-conjugated
2
2
Application
Western Blotting (WB)
12
9
4
4
3
1
Supplier
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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
Clone 1B7
Isotype IgG2a kappa
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.
Purification This antibody is purified through a protein G column, eluted with high and low pH buffers and neutralized immediately, followed by dialysis against PBS.
Alternative Name NAGLU (NAGLU Antibody Abstract)
Background Synonyms: Serine protease inhibitor J6, 47 kDa heat shock protein, Collagen-binding protein, Colligin, Serpin H1,Hsp47, Cbp1, Serpinh1
Molecular Weight 82167 DA
Gene ID 12406
NCBI Accession NP_000254
Research Area Cell Structure
Application Notes Western blot = 1:500-1000
Comment

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.

Restrictions For Research Use only
Buffer PBS with 0.09% (w/v) sodium azide.
Preservative Sodium azide
Precaution of Use This product contains sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
Storage 4 °C/-20 °C
Storage Comment Maintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C in small aliquots to prevent freeze-thaw cycles
Expiry Date 6 months
Supplier Images
Western Blotting (WB) image for anti-N-Acetylglucosaminidase, alpha (NAGLU) (AA 644-743) antibody (ABIN394860) NAGLU monoclonal antibody (M02), clone 1B7. Western Blot analysis of NAGLU expression...
Background publications 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).

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).

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).

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).

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).