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Antibody Product Overview
| ELK.No | ES14571 |
| Product name | NAGS rabbit pAb |
| Reactivity | Human; Mouse |
| Applications | WB;IHC |
| Other name | |
| Size | 50μL |
| Unit price ($) | 148 |
| Human gene ID | 162417 |
| Human Swiss-Prot | Q8N159 |
| Source | Rabbit |
| Isotype | IgG |
| Target | NAGS |
| Fields | >>Arginine biosynthesis;>>Metabolic pathways;>>2-Oxocarboxylic acid metabolism;>>Biosynthesis of amino acids |
| Gene name | NAGS |
| Protein name | NAGS |
| Human gene link | View Human Gene |
| Human Swiss link | View Human Swiss-Prot |
| Mouse gene ID | 217214 |
| Mouse gene link | View Mouse Gene |
| Mouse Swiss-Prot | Q8R4H7 |
| Mouse Swiss link | View Mouse Swiss-Prot |
| Rat gene ID | |
| Rat gene link | |
| Rat Swiss-Prot | |
| Rat Swiss link | |
| Immunogen | Synthesized peptide derived from human NAGS AA range: 361-411 |
| Specificity | This antibody detects endogenous levels of NAGS at Human/Mouse |
| Formulation | Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide. |
| Clonality | Polyclonal |
| Dilution | WB 1:500-2000;IHC-p 1:50-300 |
| Purification | The antibody was affinity-purified from rabbit serum by affinity-chromatography using specific immunogen. |
| Concentration | 1 mg/ml |
| Storage stability | -20°C/1 year |
| Molecular Weight (Da) | 59kD |
| Observed band (KD) | |
| Background | The N-acetylglutamate synthase gene encodes a mitochondrial enzyme that catalyzes the formation of N-acetylglutamate (NAG) from glutamate and acetyl coenzyme-A. NAG is a cofactor of carbamyl phosphate synthetase I (CPSI), the first enzyme of the urea cycle in mammals. This gene may regulate ureagenesis by altering NAG availability and, thereby, CPSI activity. Deficiencies in N-acetylglutamate synthase have been associated with hyperammonemia. [provided by RefSeq, Jul 2008], |
| Function | catalytic activity:Acetyl-CoA + L-glutamate = CoA + N-acetyl-L-glutamate.,disease:Defects in NAGS are the cause of N-acetylglutamate synthase deficiency (NAGSD) [MIM:237310]. NAGSD is a rare autosomal recessively inherited metabolic disorder leading to severe neonatal or late onset hyperammonemia without increased excretion of orotic acid. Clinical symptoms are somnolence, tachypnea, feeding difficulties, a severe neurologic presentation characterized by uncontrollable movements, developmental delay, visual impairment, failure to thrive and hyperammonemia precipitated by the introduction of high-protein diet or febrile illness.,enzyme regulation:Increased by L-arginine.,function:Plays a role in the regulation of ureagenesis by producing variable amounts of N-acetylglutamate (NAG), thus modulating carbamoylphosphate synthase I (CPSI) activity.,online information:N-acetylglutamate synthase |
| Subcellular location | Mitochondrion matrix . |
| Expression | Highly expressed in the adult liver, kidney and small intestine. Weakly expressed in the fetal liver, lung, pancreas, placenta, heart and brain tissue. |
Additional Images
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Western blot analysis of lysates from HpeG2 cells, primary antibody was diluted at 1:1000, 4°over night
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Immunohistochemical analysis of paraffin-embedded human Gastric adenocarcinoma. 1, Antibody was diluted at 1:200(4° overnight). 2, Tris-EDTA,pH9.0 was used for antigen retrieval. 3,Secondary antibody was diluted at 1:200(room temperature, 45min).
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: AO-06-ES14571-50
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