Datasheet
| Product Name | MTCO2 Rabbit mAb |
| Background | Cofactor: Copper A. Disease: Defects in MT-CO2 are a cause of cytochrome c oxidase deficiency (COX deficiency) [MIM:220110]; also called mitochondrial complex IV deficiency. COX deficiency is a clinically heterogeneous disorder. The clinical features are ranging from isolated myopathy to severe multisystem disease, with onset from infancy to adulthood. Disease: Defects in MT-CO2 are associated with tumor formation. Function: Cytochrome c oxidase is the component of the respiratory chain that catalyzes the reduction of oxygen to water. Subunits 1-3 form the functional core of the enzyme complex. Subunit 2 transfers the electrons from cytochrome c via its binuclear copper A center to the bimetallic center of the catalytic subunit 1. Similarity: Belongs to the cytochrome c oxidase subunit 2 family. |
| Reactivity | Human |
| Source | Rabbit, IgG Kappa |
| Applications | WB; IHC; IF; IP; ELISA |
| Dilutions | WB: 1:1000-1:5000; IHC: 1:200-1:1000; IF: 1:200-1:1000; IP: 1:50-1:200; ELISA: 1:5000-1:20000 |
| Purification | Protein A |
| Formulation | PBS, 50% glycerol, 0.05% Proclin 300, 0.05%BSA |
| Storage and Stability | -20°C/1 year |
| UniProt Accession # | P00403 |
| Protein Name | MTCO2 |
| Gene Name | MT-CO2 COII COXII MTCO2 |
| Gene ID | 4513 |
| Other Names |