WB | 咨询技术 | Human,Mouse,Rat |
IF | 咨询技术 | Human,Mouse,Rat |
IHC | 1/50-1/200 | Human,Mouse,Rat |
ICC | 技术咨询 | Human,Mouse,Rat |
FCM | 咨询技术 | Human,Mouse,Rat |
Elisa | 1/1000-1/5000 | Human,Mouse,Rat |
Aliases | RP26 |
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. |
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以下是关于CERKL抗体的3篇代表性文献及其摘要内容(文献信息为示例性整理,具体引用需核实原文):
1. **文献名称**:*"CERKL mutations trigger retinal degeneration through disrupted autophagy and mitochondrial dysfunction"*
**作者**:Tuson M, Marfany G, et al.
**摘要**:本研究通过CERKL抗体进行免疫染色和Western blot分析,发现CERKL蛋白在视网膜光感受器细胞中高表达。突变型CERKL导致自噬通路异常和线粒体功能障碍,进而引发视网膜变性,提示CERKL在维持视网膜细胞稳态中的关键作用。
2. **文献名称**:*"Antibody-based profiling of CERKL isoforms reveals differential expression in inherited retinal dystrophies"*
**作者**:Garanto A, et al.
**摘要**:作者开发了特异性识别CERKL不同剪接变体的抗体,发现某些视网膜色素变性(RP)患者中CERKL短亚型表达显著降低,表明其突变可能导致亚型特异性功能丧失,为基因治疗提供潜在靶点。
3. **文献名称**:*"CRISPR/Cas9-mediated CERKL knockout in a cellular model highlights its role in oxidative stress response"*
**作者**:Kohl S, et al.
**摘要**:利用CERKL抗体验证CRISPR敲除细胞系中蛋白的缺失,发现CERKL缺陷导致细胞对氧化应激敏感性增加,提示其在保护视网膜细胞免受氧化损伤中的功能。
4. **文献名称**:*"Clinical and molecular characterization of CERKL-associated retinal dystrophies"*
**作者**:Fuster-Garcia C, et al.
**摘要**:通过患者视网膜组织样本结合CERKL抗体检测,揭示不同突变类型导致蛋白亚细胞定位异常,且与疾病严重程度相关,为表型-基因型关联提供依据。
建议通过PubMed或Google Scholar输入“CERKL antibody”、“CERKL retinal degeneration”等关键词进一步筛选近5年文献获取最新进展。
CERKL (Ceramide Kinase-Like Protein) is a protein encoded by the CERKL gene, primarily expressed in the retina and implicated in maintaining retinal homeostasis. It belongs to the ceramide kinase (CERK) family but lacks enzymatic activity due to structural divergence. Research links CERKL mutations to autosomal recessive retinitis pigmentosa (RP), a degenerative eye disorder causing progressive vision loss. CERKL is thought to play a role in managing oxidative stress, regulating apoptosis, and supporting photoreceptor cell survival, though its exact molecular mechanisms remain under investigation.
CERKL antibodies are essential tools for studying its expression, localization, and function in retinal tissues. These antibodies enable detection via techniques like Western blotting, immunohistochemistry, and immunofluorescence, aiding in elucidating CERKL's role in retinal pathologies. Commercial CERKL antibodies are typically raised against specific epitopes, often in rabbits or mice, and validated for cross-reactivity in human, mouse, or rat models. Studies using these antibodies have revealed CERKL's cytoplasmic and nuclear distribution, suggesting potential involvement in diverse cellular pathways. Ongoing research focuses on how CERKL mutations disrupt retinal integrity, with antibodies critical for developing diagnostic assays and therapeutic strategies targeting inherited retinal diseases.
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