The OTC gene encodes ornithine carbamoyltransferase, a mitochondrial enzyme also referred to as ornithine transcarbamylase. It mediates the conversion of ornithine and carbamoyl phosphate to citrulline, a core reaction in the urea cycle that eliminates toxic ammonia from the body.
Mutations in OTC cause X-linked ornithine transcarbamylase deficiency (OTCD), the most prevalent urea cycle disorder, leading to life-threatening hyperammonemia. Standard treatments include ammonia-scavenging drugs like sodium phenylbutyrate; adeno-associated virus (AAV)-based gene therapy targeting liver cells is under clinical evaluation for severe OTCD cases to restore enzyme function.
Recombinant Human Ornithine transcarbamylase, mitochondrial (OTC) (CSB-EP017270HUc7)
Validated Data
(Tris-Glycine gel) Discontinuous SDS-PAGE (reduced) with 5% enrichment gel and 15% separation gel.
OTC Antibody (CSB-PA017270LA01HU)
Validated Data
IHC image of CSB-PA017270LA01HU diluted at 1:400 and staining in paraffin-embedded human liver tissue performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a biotinylated secondary antibody and visualized using an HRP conjugated SP system.
IHC image of CSB-PA017270LA01HU diluted at 1:400 and staining in paraffin-embedded human liver cancer performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a biotinylated secondary antibody and visualized using an HRP conjugated SP system.
Human Ornithine carbamoyltransferase, mitochondrial(OTC) ELISA kit (CSB-EL017270HU)
Validated Data
Code: CSB-EL017270HU
Size: 96T,5×96T,10×96T
Sensitivity: 0.312 mU/mL
Detection Range: 1.25 mU/mL-80 mU/mL
These standard curves are provided for demonstration only. A standard curve should be generated for each set of samples assayed.
The following OTC reagents supplied by CUSABIO are manufactured under a strict quality control system. Multiple applications have been validated and solid technical support is offered.
OTC Antibodies for Homo sapiens (Human)
| Code | Product Name | Species Reactivity | Application |
|---|---|---|---|
| CSB-PA017270LA01HU | OTC Antibody | Human | ELISA, IHC |
| CSB-PA017270LB01HU | OTC Antibody, HRP conjugated | Human | ELISA |
| CSB-PA017270LC01HU | OTC Antibody, FITC conjugated | Human | |
| CSB-PA189809 | OTC Antibody | Human,Mouse,Rat | ELISA,WB,IHC |
| CSB-PA033931 | OTC Antibody | Human,Mouse,Rat | ELISA,WB,IHC |
OTC Proteins for Homo sapiens (Human)
| Code | Product Name | Source |
|---|---|---|
| CSB-YP017270HU CSB-BP017270HU CSB-MP017270HU CSB-EP017270HU-B |
Recombinant Human Ornithine carbamoyltransferase, mitochondrial (OTC) | Yeast Baculovirus Mammalian cell In Vivo Biotinylation in E.coli |
| CSB-EP017270HU | Recombinant Human Ornithine transcarbamylase, mitochondrial (OTC) | E.coli |
| CSB-EP017270HUc7 | Recombinant Human Ornithine transcarbamylase, mitochondrial (OTC) | E.coli |
OTC ELISA Kit for Homo sapiens (Human)
| Code | Product Name | Sample Types | Sensitivity |
|---|---|---|---|
| CSB-EL017270HU | Human Ornithine carbamoyltransferase, mitochondrial(OTC) ELISA kit | serum, plasma, tissue homogenates, cell lysates | 0.312 mU/mL |