NAGLU Antibody (monoclonal) (M02)
Mouse monoclonal antibody raised against a partial recombinant NAGLU.
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- 实验流程
- 背景知识
Application
| WB |
|---|---|
| Primary Accession | P54802 |
| Other Accession | NM_000263 |
| Reactivity | Human, Mouse |
| Host | mouse |
| Clonality | monoclonal |
| Isotype | IgG2a Kappa |
| Clone Names | 1B7 |
| Calculated MW | 82266 Da |
| Gene ID | 4669 |
|---|---|
| Other Names | Alpha-N-acetylglucosaminidase, N-acetyl-alpha-glucosaminidase, NAG, Alpha-N-acetylglucosaminidase 82 kDa form, Alpha-N-acetylglucosaminidase 77 kDa form, NAGLU, UFHSD1 |
| Target/Specificity | NAGLU (NP_000254, 644 a.a. ~ 742 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 KDa. |
| Dilution | WB~~1:500~1000 |
| Format | Clear, colorless solution in phosphate buffered saline, pH 7.2 . |
| Storage | Store at -20°C or lower. Aliquot to avoid repeated freezing and thawing. |
| Precautions | NAGLU Antibody (monoclonal) (M02) is for research use only and not for use in diagnostic or therapeutic procedures. |
For Research Use Only. Not For Use In Diagnostic Procedures.
Provided below are standard protocols that you may find useful for product applications.
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.
REFERENCES
Identification and characterization of a novel homozygous deletion in the alpha-N-acetylglucosaminidase gene in a patient with Sanfilippo type B syndrome (mucopolysaccharidosis IIIB). Champion KJ, et al. Mol Genet Metab, 2010 May. PMID 20138557.Molecular analysis of mucopolysaccharidosis type IIIB in Portugal: evidence of a single origin for a common mutation (R234C) in the Iberian Peninsula. Mangas M, et al. Clin Genet, 2008 Mar. PMID 18218046.Sanfilippo type B syndrome: five patients with an R565P homozygous mutation in the alpha-N-acetylglucosaminidase gene from the Okinawa islands in Japan. Chinen Y, et al. J Hum Genet, 2005. PMID 15933803.The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC). Gerhard DS, et al. Genome Res, 2004 Oct. PMID 15489334.Inferring nonneutral evolution from human-chimp-mouse orthologous gene trios. Clark AG, et al. Science, 2003 Dec 12. PMID 14671302.
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