人 LRPPRC 抗体对
01AB02497
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.
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技术参数
- 靶点基因名
- LRPPRC
- 基因别名
- LRP130
- 蛋白别名
- leucine rich pentatricopeptide repeat containing; LRPPRC
- UniProt
- P42704
- 物种
- Human
产品详情
- 靶点名称
- LRPPRC
- 蛋白名称
- leucine rich pentatricopeptide repeat containing
- 蛋白别名
- Leucine-rich PPR motif-containing protein; mitochondrial; 130 kDa leucine-rich protein; LRP 130; GP130
- 靶点基因名
- LRPPRC
- 基因别名
- LRP130
- UniProt
- P42704
- 来源物种
- Homo sapiens (Human)
- 物种反应性
- Human
背景介绍
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.