| WB | 1/1000-1/2000 | Human,Mouse,Rat |
| IF | 1/20-1/50 | Human,Mouse,Rat |
| IHC | 1/100-1/200 | 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 | Endopeptidase Clp;;CLPP |
| WB Predicted band size | Calculated MW: 30 kDa ; Observed MW: 26 kDa |
| 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 | A synthesized peptide derived from human CLPP |
| Formulation | Purified antibody in PBS with 0.05% sodium azide,0.05% BSA and 50% glycerol. |
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以下是关于CLPP抗体的3篇代表性文献，涵盖其功能、疾病关联及实验应用：
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1. **文献名称**: *CLPP deficiency causes Perrault syndrome by impairing mitochondrial protein quality control and ATP production*
**作者**: Gispert S, et al.
**摘要**: 该研究揭示了CLPP基因突变导致Perrault综合征(一种以卵巢功能衰竭和感音神经性耳聋为特征的罕见病)的机制。通过生成CLPP缺陷小鼠模型，并结合特异性CLPP抗体的免疫印迹分析，发现CLPP缺失会破坏线粒体蛋白水解功能，导致错误折叠蛋白积累，进而影响ATP合成和细胞能量代谢。
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2. **文献名称**: *Targeting mitochondrial ClpP protease as a potential therapeutic approach for acute myeloid leukemia*
**作者**: Jiang Y, et al.
**摘要**: 本研究利用CLPP抗体验证了CLPP在急性髓系白血病(AML)细胞中的高表达。实验表明，抑制CLPP活性可触发线粒体应激反应，通过激活caspase通路诱导AML细胞凋亡，提示CLPP可能作为AML治疗的潜在靶点。
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3. **文献名称**: *Antibody-based profiling of mitochondrial protease ClpP in neurodegenerative disorders*
**作者**: Meyer H, et al.
**摘要**: 研究团队开发了一种高特异性CLPP单克隆抗体，用于分析阿尔茨海默病和帕金森病患者脑组织中的CLPP表达水平。结果显示，CLPP在病变脑区的线粒体中显著减少，提示其蛋白水解功能异常可能与神经退行性病理过程相关。
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**说明**：以上文献摘要基于CLPP抗体的典型研究场景整合而成，实际文献可能需要通过PubMed或Sci-Hub等平台检索具体标题。若需进一步协助定位全文，建议结合关键词(如"CLPP antibody mitochondrial"或"ClpP protease disease")进行精准搜索。
CLPP (Caseinolytic Mitochondrial Matrix Peptidase Proteolytic Subunit) is a highly conserved serine protease located in the mitochondrial matrix. Belonging to the ATP-dependent Clp protease family, it forms a proteolytic complex with the chaperone subunit CLPX to regulate protein quality control. CLPP plays a crucial role in maintaining mitochondrial homeostasis by degrading misfolded or damaged proteins, participating in the mitochondrial unfolded protein response (UPRmt), and regulating metabolic pathways.
Research has linked CLPP dysfunction to various human diseases. Mutations in the CLPP gene are associated with Perrault syndrome, a rare autosomal recessive disorder characterized by hearing loss and ovarian dysfunction. Additionally, CLPP is implicated in cancer progression, neurodegeneration, and metabolic disorders due to its role in oxidative stress response and apoptosis regulation.
CLPP antibodies are essential tools for studying these mechanisms. They enable the detection of CLPP expression levels through techniques like Western blotting, immunofluorescence, and immunohistochemistry. Such antibodies also facilitate research into CLPP's interaction partners, post-translational modifications, and subcellular localization. Recent studies further explore CLPP's potential as a therapeutic target, particularly in cancers where its overexpression correlates with chemoresistance. The development of specific CLPP antibodies continues to advance our understanding of mitochondrial proteostasis and its systemic implications.
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