Overview
| 别名 | Ubiquinone biosynthesis protein COQ9; mitochondrial |
| 基因名 | COQ9 |
| UniProt ID | O75208 |
| 反应种属 | Human |
| 应用 | WB,IHC-P |
| 宿主 | Mouse |
| 偶联物 | Unconjugated |
| 修饰 | Unmodified |
| 亚型 | IgG2a |
| 克隆号 | 9R4-Z6-E5 |
| 克隆性 | Monoclonal Antibody |
| 分子量 | Calculated MW: 35 kDa |
| 纯化方式 | Affinity Purified |
| 产品形式 | Liquid |
| 推荐稀释比 | WB-1:1000; IHC-1:200 |
| 存储缓冲液 | Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.09% sodium azide |
| 保存温度 | Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles. |
| 背景信息 | Coenzyme Q9 (COQ9) is a mitochondrial membrane-associated protein essential for the biosynthesis of coenzyme Q (ubiquinone). It functions non-catalytically by binding aromatic isoprene intermediates with high specificity and presenting them to the hydroxylase COQ7. This interaction is critical for stabilizing the CoQ-synthome, a multi-protein complex required for efficient Q10 production, which supports mitochondrial respiration and antioxidant defense. COQ9 exists as a homodimer with a specialized lipid-binding pocket and is regulated under metabolic stress. Clinically, mutations in COQ9 cause primary coenzyme Q10 deficiency 5 (COQ10D5), an autosomal-recessive disorder. This condition manifests as neonatal-onset encephalopathy, seizures, hypotonia, and renal dysfunction. The pathology is driven by impaired oxidative phosphorylation and mitochondrial energy failure resulting from the loss of CoQ-synthome stability. |
检测原理