Overview
| 别名 | Leucine-rich PPR motif-containing protein; mitochondrial; 130 kDa leucine-rich protein; LRP 130; GP130 |
| 基因名 | LRPPRC |
| UniProt ID | P42704 |
| 反应种属 | Human |
| 应用 | WB,IHC-P |
| 宿主 | Mouse |
| 偶联物 | Unconjugated |
| 修饰 | Unmodified |
| 亚型 | IgG1 |
| 克隆号 | 3V8-A1-Y7 |
| 克隆性 | Monoclonal Antibody |
| 分子量 | Calculated MW: 157 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 | Leucine rich pentatricopeptide repeat containing (LRPPRC) is a mitochondrial RNA-binding protein that plays a central role in both nuclear and mitochondrial RNA metabolism. In the nucleus, it facilitates the export of EIF4E-sensitive mRNAs through interactions with EIF4E and CRM1. Within the mitochondria, LRPPRC forms a ribonucleoprotein complex with SLIRP to regulate the stability and translation of poly(A) mRNAs, particularly those encoding cytochrome c oxidase (COX) subunits. This complex is essential for delivering mitochondrial mRNAs to the mitoribosome. Pathogenically, biallelic mutations in LRPPRC cause French-Canadian type Leigh syndrome, a severe neurodegenerative disorder characterized by mitochondrial complex IV deficiency, lactic acidosis, and early mortality. Beyond its role in energy metabolism, LRPPRC also modulates mitochondrial potential and suppresses basal autophagy. |
检测原理