OTC/Ornithine Carbamoyltransferase Rabbit pAb
- 产品详情
- 实验流程
Application
| WB, IHC-P, IHC-F, IF |
|---|---|
| Primary Accession | P00480 |
| Other Accession | P00480 |
| Host | Rabbit |
| Clonality | Polyclonal |
| Calculated MW | 39935 Da |
| Physical State | Liquid |
| Immunogen | KLH conjugated synthetic peptide derived from human OTC/Ornithine Carbamoyltransferase |
| Epitope Specificity | 31-130/354 |
| Isotype | IgG |
| Purity | affinity purified by Protein A |
| Buffer | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| SIMILARITY | Belongs to the ATCase/OTCase family. |
| SUBUNIT | Homotrimer. |
| Post-translational modifications | Acetylation at Lys-88 negatively regulates ornithine carbamoyltransferase activity in response to nutrient signals. |
| 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. |
| Important Note | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| Background Descriptions | 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] |
| Gene ID | 5009 |
|---|---|
| Other Names | OCTD; OTC1; OTCD; OTCase; Sf; spf; OTC_BOVIN; OTC; Ornithine carbamoyltransferase, mitochondrial; 2.1.3.3; OTC_HUMAN; OTC_MOUSE; OTC_PIG; OTC_RAT; OTC_SHEEP; ornithine transcarbamylase; ornithine carbamoyltransferase; ornithine transcarbamylase deficiency |
| Dilution | WB=1:500-2000,IHC-P=1:100-500,IHC-F=1:100-500,IF=1:100-500,Flow-Cyt=1ug/Test |
| Storage | Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C. |
For Research Use Only. Not For Use In Diagnostic Procedures.
| Name | OTC (HGNC:8512) |
|---|---|
| Function | Catalyzes the second step of the urea cycle, the condensation of carbamoyl phosphate with L-ornithine to form L-citrulline (PubMed:2556444, PubMed:6372096, PubMed:8112735). The urea cycle ensures the detoxification of ammonia by converting it to urea for excretion (PubMed:2556444). |
| Cellular Location | Mitochondrion matrix |
| Tissue Location | Mainly expressed in liver and intestinal mucosa. |
Research Areas
Application Protocols
Provided below are standard protocols that you may find useful for product applications.
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