Overview
| 别名 | NADPH-dependent 3-demethoxyubiquinone 3-hydroxylase; mitochondrial; 3-demethoxyubiquinone 3-hydroxylase; NADH); Timing protein clk-1 homolog; Ubiquinone biosynthesis monooxygenase COQ7 |
| 基因名 | COQ7 |
| UniProt ID | Q99807 |
| 反应种属 | Human |
| 应用 | IHC-P |
| 宿主 | Mouse |
| 偶联物 | Unconjugated |
| 修饰 | Unmodified |
| 亚型 | IgG1 |
| 克隆号 | 2B7-W4-H5 |
| 克隆性 | Monoclonal Antibody |
| 分子量 | Calculated MW: 24 kDa |
| 纯化方式 | Affinity Purified |
| 产品形式 | Liquid |
| 推荐稀释比 | IHC-1:100-1:200 |
| 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 Q7, hydroxylase (COQ7) is a mitochondrial di-iron monooxygenase essential for the biosynthesis of ubiquinone (coenzyme Q). It catalyzes the hydroxylation of 5-demethoxyubiquinone to 5-hydroxyubiquinone and serves a structural role in stabilizing the COQ multi-protein complex. While primarily localized to the mitochondria, a nuclear pool of COQ7 participates in mitochondrial-nuclear retrograde signaling to modulate stress responses and longevity. Clinically, pathogenic variants in COQ7 cause primary coenzyme Q10 deficiency type 8, a condition characterized by impaired mitochondrial respiration and energy metabolism. It is also associated with distal hereditary motor neuronopathy, reflecting the critical requirement for COQ7-mediated ubiquinone production in neuromuscular health and oxidative phosphorylation. |
检测原理