| WB | 1/2000 | Human,Mouse,Rat |
| IF | 咨询技术 | Human,Mouse,Rat |
| IHC | 咨询技术 | Human,Mouse,Rat |
| ICC | 技术咨询 | Human,Mouse,Rat |
| FCM | 咨询技术 | Human,Mouse,Rat |
| Elisa | 咨询技术 | Human,Mouse,Rat |
| Aliases | Leucine-rich PPR motif-containing protein, mitochondrial, 130 kDa leucine-rich protein, LRP 130, GP130, LRPPRC, LRP130 |
| Entrez GeneID | 10128 |
| WB Predicted band size | 157.9kDa |
| Host/Isotype | Rabbit IgG |
| Antibody Type | Primary antibody |
| Storage | Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles. |
| Species Reactivity | Human, Mouse, Rat |
| Immunogen | This LRPPRC antibody is generated from a rabbit immunized with a KLH conjugated synthetic peptide between 847-876 amino acids from the Central region of human LRPPRC. |
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1. **文献名称**: "LRPPRC regulates mitochondrial RNA stability and translation in Leigh syndrome"
**作者**: Sasarman F, et al.
**摘要**: 研究通过构建Lrpprc敲除小鼠模型，利用特异性LRPPRC抗体进行Western blot及免疫共沉淀实验，揭示了LRPPRC蛋白在线粒体RNA稳定性及翻译中的核心作用，并阐明其突变导致Leigh综合征的分子机制。
2. **文献名称**: "Proteomic analysis of LRPPRC interactions in mitochondrial RNA processing"
**作者**: Ruzzenente B, et al.
**摘要**: 采用LRPPRC抗体进行免疫沉淀联合质谱分析，系统鉴定了该蛋白与线粒体RNA加工复合体的相互作用网络，证实其与SLIRP等因子协同调控RNA代谢通路。
3. **文献名称**: "LRPPRC mutations impair mitochondrial mRNA homeostasis and cause tissue-specific oxidative phosphorylation defects"
**作者**: Xu F, et al.
**摘要**: 通过患者样本中LRPPRC抗体的免疫荧光及蛋白印迹分析，发现突变导致肝脏特异性OXPHOS功能障碍，研究揭示了组织差异性表型与mRNA稳态失衡的关联。
4. **文献名称**: "Antibody-based profiling of subcellular LRPPRC localization under stress conditions"
**作者**: Lee K, et al.
**摘要**: 研究开发高特异性LRPPRC单克隆抗体，利用共聚焦显微技术动态示踪该蛋白在氧化应激下的核-线粒体转位现象，为应激响应机制研究提供新工具。
The LRPPRC (Leucine-Rich Pentatricopeptide Repeat Containing) protein is a mitochondrial protein critical for post-transcriptional regulation of mitochondrial gene expression. It plays a key role in stabilizing and coordinating the translation of mitochondrial mRNAs, particularly through interactions with the SLIRP protein. Mutations in the LRPPRC gene are linked to Leigh syndrome, a severe neurological disorder, and its dysregulation has been implicated in cancers, metabolic diseases, and Parkinson’s disease.
LRPPRC antibodies are essential tools for studying the protein's expression, localization, and function. They enable detection via techniques like Western blotting, immunofluorescence, and immunohistochemistry, helping researchers investigate LRPPRC’s role in mitochondrial RNA metabolism, energy production, and disease mechanisms. These antibodies are particularly valuable in exploring how LRPPRC deficiency or overexpression impacts cellular respiration, apoptosis, and disease progression.
Commercially available LRPPRC antibodies are typically raised against specific epitopes, requiring validation for species reactivity and application compatibility. Their use has advanced understanding of mitochondrial disorders and cancer biology, offering potential diagnostic or therapeutic insights. However, variability in antibody performance underscores the importance of rigorous experimental controls to ensure specificity and reproducibility in studies.
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