Anti-OTC Picoband Antibody
- SPECIFICATION
- CITATIONS
- PROTOCOLS
- BACKGROUND
Application
| WB |
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Primary Accession | P00480 |
Host | Rabbit |
Reactivity | Human, Mouse, Rat |
Clonality | Polyclonal |
Format | Lyophilized |
Description | Rabbit IgG polyclonal antibody for Ornithine carbamoyltransferase, mitochondrial(OTC) detection. Tested with WB in Human;Mouse;Rat. |
Reconstitution | Add 0.2ml of distilled water will yield a concentration of 500ug/ml. |
Gene ID | 5009 |
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Other Names | Ornithine carbamoyltransferase, mitochondrial, 2.1.3.3, Ornithine transcarbamylase, OTCase, OTC |
Calculated MW | 39935 MW KDa |
Application Details | Western blot, 0.1-0.5 µg/ml, Mouse, Rat, Human |
Subcellular Localization | Mitochondrion matrix. |
Tissue Specificity | Mainly expressed in liver and intestinal mucosa. |
Protein Name | Ornithine carbamoyltransferase, mitochondrial |
Contents | Each vial contains 5mg BSA, 0.9mg NaCl, 0.2mg Na2HPO4, 0.05mg NaN3. |
Immunogen | A synthetic peptide corresponding to a sequence at the N-terminus of human OTC (33-70aa NKVQLKGRDLLTLKNFTGEEIKYMLWLSADLKFRIKQK), different from the related mouse and rat sequences by three amino acids. |
Purification | Immunogen affinity purified. |
Cross Reactivity | No cross reactivity with other proteins. |
Storage | At -20˚C for one year. After r˚Constitution, at 4˚C for one month. It˚Can also be aliquotted and stored frozen at -20˚C for a longer time.Avoid repeated freezing and thawing. |
Name | OTC (HGNC:8512) |
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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. |
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Provided below are standard protocols that you may find useful for product applications.
Background
Ornithine transcarbamylase (OTC) (also called ornithine carbamoyltransferase) is an enzyme that catalyzes the reaction between carbamoyl phosphate (CP) and ornithine (Orn) to form citrulline (Cit) and phosphate (Pi). 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 also play a role in that disease.
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