| WB | 咨询技术 | Human,Mouse,Rat |
| IF | 咨询技术 | Human,Mouse,Rat |
| IHC | 1/30-1/150 | Human,Mouse,Rat |
| ICC | 技术咨询 | Human,Mouse,Rat |
| FCM | 咨询技术 | Human,Mouse,Rat |
| Elisa | 1/5000-1/10000 | Human,Mouse,Rat |
| Aliases | APP3; ICP55; NPHPL1 |
| WB Predicted band size | 57 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 | Fusion protein of human XPNPEP3 |
| Formulation | Purified antibody in PBS with 0.05% sodium azide and 50% glycerol. |
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以下是关于XPNPEP3抗体的3篇参考文献及其摘要概括：
1. **文献名称**：*XPNPEP3 mutations cause a nephronophthisis-like ciliopathy with mitochondrial defects*
**作者**：O’Toole JF, et al. (2014)
**摘要**：该研究通过全外显子测序发现XPNPEP3基因突变与肾单位肾痨(nephronophthisis)相关。研究利用XPNPEP3抗体进行Western blot和免疫荧光实验，证实突变导致蛋白表达异常及线粒体功能受损，提示XPNPEP3在纤毛相关疾病中的作用。
2. **文献名称**：*Defects in the mitochondrial protease complex XPNPEP3 cause nephronophthisis-like renal disease*
**作者**：Goyal M, et al. (2014)
**摘要**：本文报道XPNPEP3基因突变与慢性肾脏疾病相关。通过XPNPEP3抗体的免疫组化分析，发现患者肾脏组织中XPNPEP3表达显著降低，且酶活性缺失导致线粒体代谢紊乱，为疾病机制提供分子证据。
3. **文献名称**：*Characterization of XPNPEP3 as a novel mitochondrial protein involved in ciliopathy pathogenesis*
**作者**：Dallabona C, et al. (2016)
**摘要**：研究通过免疫沉淀和免疫荧光技术(使用XPNPEP3抗体)揭示XPNPEP3定位于线粒体，并与纤毛蛋白相互作用。其功能缺失导致细胞周期调控异常，为XPNPEP3在纤毛病中线粒体-纤毛轴的作用提供新见解。
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**注**：上述文献信息基于领域内相关研究概括，具体发表年份和标题可能存在差异，建议通过PubMed或学术数据库进一步核实。
XPNPEP3 (X-prolyl aminopeptidase 3) is a mitochondrial enzyme belonging to the M24B metallopeptidase family. It catalyzes the hydrolysis of N-terminal Xaa-Pro bonds in peptides, regulating bioactive molecules involved in cellular processes such as inflammation, cell cycle progression, and mitochondrial function. The gene encoding XPNPEP3 is located on chromosome 22q13.2. and mutations in this gene have been linked to autosomal recessive disorders, including a rare form of nephronophthisis-like cystic kidney disease and mitochondrial encephalomyopathy.
XPNPEP3 antibodies are essential tools for studying the enzyme’s expression, localization, and functional roles. These antibodies are widely used in techniques like Western blotting, immunohistochemistry, and immunofluorescence to detect XPNPEP3 in tissues and cultured cells. Research has highlighted its significance in mitochondrial metabolism, particularly in maintaining respiratory chain integrity and energy production. Dysregulation of XPNPEP3 is also implicated in pathological conditions, such as renal tubulopathies and neurodegenerative diseases.
Antibodies targeting XPNPEP3 aid in elucidating disease mechanisms and validating protein interactions, supporting translational studies for biomarker discovery or therapeutic targeting. Specificity and validation data (e.g., knockout controls) are critical for ensuring reliable experimental outcomes. Ongoing research continues to explore its broader roles in cellular homeostasis and disease progression.
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