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anti-NAGLU antibody :: Rabbit anti-Human N-Acetyl Alpha-D-Glucosaminidase Polyclonal Antibody

Scan QR to view Datasheet Catalog #    MBS2027566 (3 units are in stock [0.1 mg] for immediate shipment)
Unit / Price
0.01 mg  /  $110 +1 FREE 8GB USB
0.02 mg  /  $130 +1 FREE 8GB USB
0.05 mg  /  $175 +1 FREE 8GB USB
0.1 mg  /  $225 +1 FREE 8GB USB
0.2 mg  /  $340 +1 FREE 8GB USB
1 mg  /  $800 +1 FREE 8GB USB
 
 Go to:   rightarrow  Product Names   rightarrow Product Info   rightarrow Accession #s   rightarrow Product Desc   rightarrow Diseases/Tissues/Pathways   rightarrow Applications   rightarrow References 
 Product Name   

N-Acetyl Alpha-D-Glucosaminidase (NAGLU), Polyclonal Antibody

★Popular Item★
 Also Known As   

Polyclonal Antibody to N-Acetyl Alpha-D-Glucosaminidase (NAGLU)

 Product Gene Name   

anti-NAGLU antibody

[Similar Products]
 Research Use Only    For Research Use Only. Not for use in diagnostic procedures.
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 OMIM    U43572 Genomic DNA
 3D Structure    ModBase 3D Structure for P54802
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 Clonality    Polyclonal
 Isotype    IgG
 Host    Rabbit
 Species Reactivity    Human
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 Specificity    The antibody is a Rabbit polyclonal antibody raised against NAGLU. It has been selected for its ability to recognize NAGLU in immunohistochemical staining and western blotting.
 Purity/Purification    Antigen-specific Affinity Chromatography
 Form/Format    Supplied as solution form in 0.01M PBS, pH7.4, containing 0.05% Proclin-300, 50% glycerol
 Concentration    200ug/ml (lot specific)
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 Source    Polyclonal antibody preparation
 Traits    Liquid
 Immunogen    Recombinant NAGLU (Val485~Trp743) expressed in E.coli
    
 Conjugated Antibody    The APC conjugated antibody version of this item is also available as catalog #MBS2070317
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 Preparation and Storage    Storage: Avoid repeated freeze/thaw cycles.
Store at 4 degree C for frequent use.
Aliquot and store at -20 degree C for two years.
Stability Test: The thermal stability is described by the loss rate. The loss rate was determined by accelerated thermal degradation test, that is, incubate the protein at 37 degree C for 48h, and no obvious degradation and precipitation were observed.The loss rate is less than 5% within the expiration date under appropriate storage condition.
 Other Notes    Small volumes of anti-NAGLU antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.
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 Applications Tested/Suitable for anti-NAGLU antibody   

WB; ICC; IHC-P; IHC-F; ELISA; IP; IF; FCM

 Application Notes for anti-NAGLU antibody    Western blotting: 0.5-2ug/ml
Immunocytochemistry in formalin fixed cells: 5-20ug/ml
Immunohistochemistry in formalin fixed frozen section: 5-20ug/ml
Immunohistochemistry in paraffin section: 5-20ug/ml
Enzyme-linked Immunosorbent Assay: 0.05-2ug/ml

Optimal working dilutions must be determined by end user
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 Western Blot (WB) of anti-NAGLU antibody    (Figure. Western Blot; Sample: Recombinant protein.)
anti-NAGLU antibody Western Blot (WB) (WB) image
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 Immunohistochemistry (IHC) of anti-NAGLU antibody    (DAB staining on IHC-P; Samples: Human Kidney Tissue)
anti-NAGLU antibody Immunohistochemistry (IHC) (IHC) image
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 Immunohistochemistry (IHC) of anti-NAGLU antibody    (Used in DAB staining on formalin fixed paraffin-embedded kidney tissue)
anti-NAGLU antibody Immunohistochemistry (IHC) (IHC) image
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NCBI/Uniprot data below describe general gene information for NAGLU. It may not necessarily be applicable to this product.
 NCBI GI #    66346698
 NCBI GeneID    4669
 NCBI Accession #    NP_000254.2 [Other Products]
 NCBI GenBank Nucleotide #    NM_000263.3 [Other Products]
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 UniProt Primary Accession #    P54802 [Other Products]
 UniProt Related Accession #    P54802 [Other Products]
 Molecular Weight    82,266 Da
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 NCBI Official Full Name    alpha-N-acetylglucosaminidase
 NCBI Official Synonym Full Names    N-acetyl-alpha-glucosaminidase
 NCBI Official Symbol    NAGLU [Similar Products]
 NCBI Official Synonym Symbols   
NAG; CMT2V; MPS3B; UFHSD; MPS-IIIB
[Similar Products]
 NCBI Protein Information    alpha-N-acetylglucosaminidase
 UniProt Protein Name    Alpha-N-acetylglucosaminidase
 UniProt Synonym Protein Names   
N-acetyl-alpha-glucosaminidase; NAG
 Protein Family    Alpha-N-acetylglucosaminidase
 UniProt Gene Name    NAGLU [Similar Products]
 UniProt Synonym Gene Names    UFHSD1; NAG [Similar Products]
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 NCBI Summary for NAGLU    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]
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 UniProt Comments for NAGLU    NAGLU: Involved in the degradation of heparan sulfate. Defects in NAGLU are the cause of mucopolysaccharidosis type 3B (MPS3B); 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.

Protein type: EC 3.2.1.50; Glycan Metabolism - glycosaminoglycan degradation; Hydrolase

Chromosomal Location of Human Ortholog: 17q21.2

Cellular Component: extracellular exosome; lysosomal lumen; lysosome

Molecular Function: alpha-N-acetylglucosaminidase activity

Biological Process: cerebellar Purkinje cell layer development; glycosaminoglycan catabolic process; inner ear receptor cell development; locomotor rhythm; lysosome organization and biogenesis; middle ear morphogenesis; nervous system development; retinal rod cell development

Disease: Charcot-marie-tooth Disease, Axonal, Type 2v; Mucopolysaccharidosis, Type Iiib
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 Research Articles on NAGLU    1. CSF enzyme activity levels for either SGSH (in MPS IIIA subjects) or NAGLU (in MPS IIIB) significantly differed from normal controls. Several other behavioral or functional measures were found to be uninformative in this population, including timed functional motor tests.
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 Precautions    All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.
 Disclaimer    While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.
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Products associated with anti-NAGLU antibodyPathways associated with anti-NAGLU antibody
 Reference Product  PubMed Publications
 SGSH antibody  >6 publications with NAGLU and SGSH
 HGSNAT antibody  >3 publications with NAGLU and HGSNAT
 GNS antibody  >2 publications with NAGLU and GNS
 HSD17B1 antibody  >1 publications with NAGLU and HSD17B1
 IDUA antibody  >1 publications with NAGLU and IDUA
 IDS antibody  >1 publications with NAGLU and IDS
 Products by Pathway  Pathway Diagram
 Defective B3GAT3 Causes JDSSDHD Pathway antibodies  Defective B3GAT3 Causes JDSSDHD Pathway Diagram
 Defective B4GALT1 Causes B4GALT1-CDG (CDG-2d) Pathway antibodies  Defective B4GALT1 Causes B4GALT1-CDG (CDG-2d) Pathway Diagram
 Defective B4GALT7 Causes EDS, Progeroid Type Pathway antibodies  Defective B4GALT7 Causes EDS, Progeroid Type Pathway Diagram
 Defective CHST14 Causes EDS, Musculocontractural Type Pathway antibodies  Defective CHST14 Causes EDS, Musculocontractural Type Pathway Diagram
 Defective CHST3 Causes SEDCJD Pathway antibodies  Defective CHST3 Causes SEDCJD Pathway Diagram
 Defective CHST6 Causes MCDC1 Pathway antibodies  Defective CHST6 Causes MCDC1 Pathway Diagram
 Defective CHSY1 Causes TPBS Pathway antibodies  Defective CHSY1 Causes TPBS Pathway Diagram
 Defective EXT1 Causes Exostoses 1, TRPS2 And CHDS Pathway antibodies  Defective EXT1 Causes Exostoses 1, TRPS2 And CHDS Pathway Diagram
 Defective EXT2 Causes Exostoses 2 Pathway antibodies  Defective EXT2 Causes Exostoses 2 Pathway Diagram
 Defective PAPSS2 Causes SEMD-PA Pathway antibodies  Defective PAPSS2 Causes SEMD-PA Pathway Diagram
Diseases associated with anti-NAGLU antibodyOrgans/Tissues associated with anti-NAGLU antibody
 Disease Name  Pubmed Publications
 Mucopolysaccharidosis III Antibodies  >80 publications with NAGLU and Mucopolysaccharidosis III
 Disease Models, Animal Antibodies  >25 publications with NAGLU and Disease Models, Animal
 Abnormalities, Multiple Antibodies  >6 publications with NAGLU and Abnormalities, Multiple
 Skin Diseases Antibodies  >5 publications with NAGLU and Skin Diseases
 Intellectual Disability Antibodies  >5 publications with NAGLU and Intellectual Disability
 Inflammation Antibodies  >3 publications with NAGLU and Inflammation
 Liver Diseases Antibodies  >3 publications with NAGLU and Liver Diseases
 Kidney Diseases Antibodies  >2 publications with NAGLU and Kidney Diseases
 Myocardial Infarction Antibodies  >1 publications with NAGLU and Myocardial Infarction
 Blood Coagulation Disorders Antibodies  >1 publications with NAGLU and Blood Coagulation Disorders
 Organ/Tissue Name  Pubmed Publications
 Brain Antibodies  >44 publications with NAGLU and Brain
 Liver Antibodies  >20 publications with NAGLU and Liver
 Kidney Antibodies  >9 publications with NAGLU and Kidney
 Ovary Antibodies  >8 publications with NAGLU and Ovary
 Skin Antibodies  >6 publications with NAGLU and Skin
 Lung Antibodies  >5 publications with NAGLU and Lung
 Bone Antibodies  >4 publications with NAGLU and Bone
 Eye Antibodies  >3 publications with NAGLU and Eye
 Bone Marrow Antibodies  >3 publications with NAGLU and Bone Marrow
 Intestine Antibodies  >3 publications with NAGLU and Intestine
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