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- Bioss
- bs-8719P
- 2025年10月16日
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- 规格：
500ug
| 产品编号 | bs-8719P |
| 英文名称 | LRPPRC Antibody Blocking Peptide |
| 中文名称 | 富含亮氨酸蛋白LRP130封闭多肽 |
| 英文别名 | LRPPRC; 130 kDa leucine-rich protein; GP130; Leucine-rich PPR-motif containing protein; LRP 130; LPPRC_HUMAN. |
| 纯化方法 | HPLC |
| 研究领域 | Epigenetics and Nuclear Signaling > DNA / RNA > RNA Processing Epigenetics and Nuclear Signaling > DNA / RNA > Translation > Mito. Translation Neuroscience > Neurology process > Neurodegenerative disease Neuroscience > Neurology process > Neurogenesis |
| 亚基 | Interacts with INPP5D/SHIP1 (By similarity). Forms heterodimers composed of LIPR and IL6ST (type I OSM receptor). Also forms heterodimers composed of OSMR and IL6ST (type II OSM receptor). Homodimer. The homodimer binds two molecules of herpes virus IL6. Component of a hexamer of two molecules each of IL6, IL6R and IL6ST. Interacts with HCK. |
| 亚细胞定位 | Mitochondrion. Nucleus; nucleoplasm. Nucleus inner membrane. Nucleus outer membrane. Note: Seems to be predominantly mitochondrial. |
| 组织特异性 | Found in all the tissues and cell lines examined. Expression not restricted to IL6 responsive cells. |
| 相似性 | Contains 20 PPR (pentatricopeptide) repeats. |
| 功能 | LPPRC is thought to play a role in RNA metabolism in both nuclei and mitochondria. In the nucleus, LPPRC binds to HNRPA1-associated poly(A) mRNAs and is part of nmRNP complexes at late stages of mRNA maturation, which are possibly associated with nuclear mRNA export. In mitochondria, LPPRC binds to poly(A) mRNA. LPPRC may play a role in translation or stability of mitochondrially encoded cytochrome c oxidase (COX) subunits. Defects in LRPPRC are the cause of Leigh syndrome French-Canadian type (LSFC). Leigh syndrome is a severe neurological disorder characterized by bilaterally symmetrical necrotic lesions in subcortical brain regions that is commonly associated with systemic cytochrome c oxidase (COX) deficiency. In the Saguenay-Lac Saint Jean region of Quebec province in Canada, a biochemically distinct form of Leigh syndrome with COX deficiency has been described. Patients have been observed to have a developmental delay, hypotonia, mild facial dysmorphism, chronic well-compensated metabolic acidosis, and high mortality due to episodes of severe acidosis and coma. Enzyme activity was close to normal in kidney and heart, 50% of normal in fibroblasts and skeletal muscle, and nearly absent in brain and liver. |
| 保存条件 | Shipped at 4℃. Stored at -20℃ for one year. Avoid repeated freeze/thaw cycles. |
| 注意事项 | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| 背景资料 | Leucine-rich protein (LRP130) is a cytoplasmic mRNA-binding protein likely to be involved in the processing of mitochondrial DNA transcripts. Defects in the LRPPRC gene that encodes LRP130 result in the French-Canadian type of Leigh syndrome, a severe neurological disorder characterized by lesions in the subcortical region of the brain. LRP130 also interacts with the low-affinity receptor for leukemia inhibitory factor to produce an intracelluar signal cascade. |
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LRPPRC Antibody Blocking Peptide(bs-8719P)-500ug
￥880
