Product Description

ANAG Rabbit Polyclonal Antibody

Sizes: 50μL, 100μL

Catalogue Numbers: ES18360-50, ES18360-100

Lead times: 1-2 weeks, if manufacturer has product in stock

Manufacturer/Ship Location: China

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. [provided by RefSeq, Jul 2008],

Reactivity: Human, Rat, Mouse,

Host: Rabbit

Applications: WB

Clonality: Polyclonal

Immunogen: Synthesized peptide derived from human ANAG AA range: 146-196

Isotype: IgG

Target: ANAG

Specificity: This antibody detects endogenous levels of ANAG at Human

Dilution: WB 1：500-2000

Purification: The antibody was affinity-purified from rabbit serum by affinity-chromatography using specific immunogen.

Concentration: 1 mg/ml,

Formlation: Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.

Expression: Liver, ovary, peripheral blood leukocytes, testis, prostate, spleen, colon, lung, placenta and kidney,

Molecular Weight (Da): 82kD

Fields: Glycosaminoglycan degradation, Metabolic pathways, Lysosome

Function: Catalytic Activity: Hydrolysis of terminal non-reducing N-acetyl-D-glucosamine residues in N-acetyl-alpha-D-glucosaminides
Caution: A MPS3B mutation at position 100 was erroneously reported (PubMed: 9950362) as an amino acid change from Arg to His. The right amino acid change is from His to Arg
Disease: Defects in NAGLU are the cause of mucopolysaccharidosis type 3B (MPS3B) [MIM: 252920], also known as Sanfilippo syndrome B. MPS3B is a form of mucopolysaccharidosis type 3, an autosomal recessive lysosomal storage disease due to impaired degradation of heparan sulfate. MPS3 is characterized by severe central nervous system degeneration, but only mild somatic disease. Onset of clinical features usually occurs between 2 and 6 years, severe neurologic degeneration occurs in most patients between 6 and 10 years of age, and death occurs typically during the second or third decade of life
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Subcellular Location: Lysosome

Storage and Stability: -20°C/1 year

Gene Name: NAGLU UFHSD1

Protein Name: ANAG

Research Use Only