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NAGLU Antibody (monoclonal) (M02)

NAGLU Antibody (monoclonal) (M02)

ArtNr	ABC-AT2970a
Hersteller	Abcepta
Menge	100 ug
Kategorie	Neuroscience Targets for Rodent Neurosciences Antibodies Western Blot Primary Antibodies
Typ	Antibody Monoclonal
Format	Liquid
Applikationen	WB
Clon	1B7
Specific against	Human (Homo sapiens), Mouse (Murine, Mus musculus)
Host	Mouse
Isotype	IgG2a Kappa
Citations	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.
NCBI	NP_000254.2
ECLASS 10.1	42030590
ECLASS 11.0	42030590
UNSPSC	12352203
Alias	Alpha-N-acetylglucosaminidase,N-acetyl-alpha-glucosaminidase,NAG,Alpha-N-acetylglucosaminidase 82 kDa form,Alpha-N-acetylglucosaminidase 77 kDa form,NAGLU,UFHSD1
Similar products	NAG, NAGLU, N-acetyl-alpha-glucosaminidase, Alpha-N-acetylglucosaminidase, UFHSD1, Alpha-N-acetylglucosaminidase 82 kDa form, Alpha-N-acetylglucosaminidase 77 kDa form
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Manufacturer - Targets	NAGLU (NP_000254, 644 a.a. ~ 742 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.
Bio 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.
Clonality	Monoclonal
Gene Name	NAGLU
Subtitle	Mouse monoclonal antibody raised against a partial recombinant NAGLU.
Reactivity	H, M
Antigen Source	Human: ~~ Positive
Formulation	Clear, colorless solution in phosphate buffered saline, pH 7.2 .
Dilution	WB: 1:500~1000 WB: 1:500~1000
Org Accession	NP_000254
Gene Description	N-acetylglucosaminidase, alpha-
Gene Summary	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. [provided by RefSeq
Antigen Type	Recombinant Protein

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ABC-AT2970a-datasheet.pdf

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Menge: 100 ug

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