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Antibody Product Overview
| ELK.No | ES14359 |
| Product name | OTC rabbit pAb |
| Reactivity | Human; Mouse;Rat |
| Applications | WB |
| Other name | Ornithine carbamoyltransferase, mitochondrial (EC 2.1.3.3) (Ornithine transcarbamylase) (OTCase) |
| Size | 100μL |
| Unit price ($) | 248 |
| Human gene ID | 5009 |
| Human Swiss-Prot | P00480 |
| Source | Rabbit |
| Isotype | IgG |
| Target | OTC |
| Fields | >>Arginine biosynthesis;>>Metabolic pathways;>>Biosynthesis of amino acids |
| Gene name | OTC |
| Protein name | OTC |
| Human gene link | View Human Gene |
| Human Swiss link | View Human Swiss-Prot |
| Mouse gene ID | 18416 |
| Mouse gene link | View Mouse Gene |
| Mouse Swiss-Prot | P11725 |
| Mouse Swiss link | View Mouse Swiss-Prot |
| Rat gene ID | 25611 |
| Rat gene link | View Rat Gene |
| Rat Swiss-Prot | P00481 |
| Rat Swiss link | View Rat Swiss-Prot |
| Immunogen | Synthesized peptide derived from human OTC AA range: 275-325 |
| Specificity | This antibody detects endogenous levels of OTC at Human/Mouse/Rat |
| Formulation | Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.60% sodium azide. |
| Clonality | Polyclonal |
| Dilution | WB 1:500-2000 |
| 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) | |
| Observed band (KD) | 38kD |
| Background | This nuclear gene encodes a mitochondrial matrix enzyme. Missense, nonsense, and frameshift mutations in this enzyme lead to ornithine transcarbamylase deficiency, which causes hyperammonemia. Since the gene for this enzyme maps close to that for Duchenne muscular dystrophy, it may play a role in that disease also. [provided by RefSeq, Jul 2008], |
| Function | catalytic activity:Carbamoyl phosphate + L-ornithine = phosphate + L-citrulline.,disease:Defects in OTC are the cause of ornithine carbamoyltransferase deficiency (OTCD) [MIM:311250]. OTCD is an X-linked disorder of the urea cycle which causes a form of hyperammonemia. Mutations with no residual enzyme activity are always expressed in hemizygote males by a very severe neonatal hyperammonemic coma that generally proves to be fatal. Heterozygous females are either asymptomatic or express orotic aciduria spontaneously or after protein intake. The disorder is treatable with supplemental dietary arginine and low protein diet. The arbitrary classification of patients into the "neonatal" group (clinical hyperammonemia in the first few days of life) and "late" onset (clinical presentation after the neonatal period) has been used to differentiate severe from mild forms.,online information:OTCase |
| Subcellular location | Mitochondrion matrix . |
| Expression | Mainly expressed in liver and intestinal mucosa. |
Additional Images
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Western blot analysis of lysates from U2OS cells, primary antibody was diluted at 1:1000, 4°over night
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: AO-06-ES14359-100
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