Price range: $268.00 through $328.00
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] ,
| Size | 50μL/100μL |
| Target | ANAG |
| Reactivity | Human, Mouse, Rat |
| Applications | WB, IHC, IF, ELISA |
| MW(Calculated) | 82kD |
| MW(Observed) | 82kD |
| Host Species | Rabbit |
| Isotype | IgG, Kappa |
| Conjugate/Modification | Unmodified |
| Recommended Dilution Ratio | IHC 1:200-1:1000; WB 1:2000-1:10000; IF 1:200-1:1000; ELISA 1:5000-1:20000 / Note: For IHC, we suggest antigen retrieval with TE buffer pH 9.0 (Cat#WT1004) |
| Formulation | PBS, 50% glycerol, 0.05% Proclin 300, 0.05%BSA |
| Purification | Recombinant Antibody expressed in animal component-free (ACF) media, purified via Protein A affinity chromatography. |
| Storage | -15°C to -25°C/1 year (Do not lower than -25°C) |
| Concentration | |
| Clonality | Monoclonal |
| Clone Number | RM2024R |
| Immunogen | The specific immunogen used to produce this antibody is proprietary information. |
| Specificity | Endogenous |
| Gene Name | NAGLU UFHSD1 |
| Protein Name | ANAG |
| Other Name | |
| Species | Human |
| Gene ID | 4669 |
| Uniprot | P54802 |
| Species.1 | |
| Gene ID.1 | |
| Uniprot.1 | |
| Species.2 | |
| Gene ID.2 | |
| Uniprot.2 | |
| Cellular Localization | Lysosome. |
| Tissue Expression | Liver , ovary , peripheral blood leukocytes , testis , prostate , spleen , colon , lung , placenta and kidney. |
| Signaling_pathway | Cellular Processes >> Transport and catabolism >> Lysosome |
| Research Areas | >>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. ,Function:Involved in the degradation of heparan sulfate. ,subunit:Monomer and homodimer. ,tissue specificity:Liver , ovary , peripheral blood leukocytes , testis , prostrate , spleen , colon , lung , placenta and kidney. , |
