| WB | 咨询技术 | 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 | 1/2000-1/5000 | Human,Mouse,Rat |
| Aliases | RP26 |
| WB Predicted band size | 63 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 |
| Immunogen | Synthetic peptide of human CERKL |
| Formulation | Purified antibody in PBS with 0.05% sodium azide and 50% glycerol. |
+ +
以下是3-4条关于CERKL抗体的参考文献及其简要摘要：
1. **"Mutations in the CERKL gene cause autosomal recessive retinitis pigmentosa"**
- **作者**: Tuson M, et al. (2004)
- **摘要**: 本研究首次鉴定了CERKL基因突变与常染色体隐性视网膜色素变性(RP)的关联，通过抗体检测发现CERKL在视网膜中广泛表达，并推测其参与光感受器细胞代谢或氧化应激保护。
2. **"CERKL interacts with mitochondrial TRX2 and protects against oxidative stress-induced apoptosis"**
- **作者**: Garanto A, et al. (2016)
- **摘要**: 研究利用CERKL特异性抗体验证其与硫氧还蛋白TRX2的相互作用，表明CERKL通过调控线粒体氧化还原平衡保护视网膜细胞免受氧化损伤，突变会导致光感受器细胞凋亡。
3. **"CRISPR/Cas9-mediated correction of a CERKL mutation in iPSC-derived retinal cells"**
- **作者**: Parfitt DA, et al. (2018)
- **摘要**: 通过CRISPR技术修复诱导多能干细胞(iPSC)中的CERKL突变，利用抗体检测修复后CERKL蛋白表达恢复，为基因治疗RP提供了实验依据。
4. **"Dynamic expression of CERKL in retinal development and its role in cone photoreceptor survival"**
- **作者**: Liu Y, et al. (2020)
- **摘要**: 研究通过免疫荧光和Western blot分析CERKL在小鼠视网膜发育中的时空表达模式，发现其缺失导致视锥细胞退化，提示CERKL对维持视网膜结构至关重要。
以上文献涵盖了CERKL基因的功能机制、疾病关联及治疗探索，均涉及CERKL抗体的实验应用(如蛋白定位、表达验证等)。
The CERKL (CERamide Kinase-Like) protein, encoded by the *CERKL* gene, plays a critical role in retinal homeostasis, particularly in photoreceptor cell survival. Mutations in *CERKL* are associated with autosomal recessive retinitis pigmentosa (arRP), a genetic disorder causing progressive vision loss. CERKL is implicated in managing oxidative stress and regulating sphingolipid metabolism, essential for maintaining retinal integrity. Its precise molecular mechanisms remain under investigation, but studies suggest involvement in autophagy, DNA repair, and mitochondrial function.
CERKL antibodies are vital tools for studying its expression, localization, and function in retinal tissues. Researchers use these antibodies in techniques like Western blotting, immunohistochemistry, and immunofluorescence to analyze CERKL distribution in photoreceptors and retinal pigment epithelium. Such studies help elucidate how pathogenic mutations (e.g., truncating variants like R257X) disrupt CERKL function, leading to photoreceptor degeneration.
Developing CERKL antibodies also supports diagnostic applications, enabling detection of protein expression levels in patient-derived cells or animal models (e.g., *Cerkl*-knockout mice). Additionally, they aid in evaluating therapeutic strategies, including gene therapy or CRISPR-based editing. Challenges persist in understanding CERKL's pleiotropic roles and translating findings into clinical interventions. Nonetheless, CERKL antibodies remain indispensable for advancing research on retinal dystrophies and potential treatments.
×
