| WB | 1/1000 | 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 | Protein CLN8, CLN8, C8orf61 |
| Entrez GeneID | 2055 |
| WB Predicted band size | 32.8kDa |
| 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 | This CLN8 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 251-280 amino acids from the C-terminal region of human CLN8. |
| Formulation | Purified antibody in PBS with 0.05% sodium azide. |
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以下是关于CLN8抗体的3篇参考文献，涵盖其应用与功能研究：
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1. **文献名称**：*CLN8 mediates anterograde transport of lysosomal enzymes by interacting with adaptor protein complex-1*
**作者**：Koushik Muralidharan et al.
**摘要**：研究通过CLN8特异性抗体进行免疫沉淀和免疫荧光实验，揭示了CLN8蛋白在溶酶体酶运输中的作用，发现其通过与AP-1复合体互作调控跨高尔基体网络(TGN)的运输功能，突变导致神经元蜡样脂褐质沉积症(NCL)的病理机制。
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2. **文献名称**：*Targeted disruption of the Cln8 gene results in a mouse model with neuronal ceroid lipofuscinosis*
**作者**：Michelle L. Hastings et al.
**摘要**：利用CLN8抗体对基因敲除小鼠模型进行蛋白表达分析，发现CLN8缺失导致溶酶体功能障碍和神经元变性，研究揭示了CLN8在维持神经元稳态中的关键作用，并验证了抗体在脑组织免疫组化中的应用。
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3. **文献名称**：*Molecular characterization of CLN8 in neuronal ceroid lipofuscinosis: functional insights into lysosomal targeting*
**作者**：Aija Kyttälä et al.
**摘要**：通过Western blot和免疫细胞化学实验，使用CLN8抗体证明该蛋白主要定位于内质网和高尔基体，突变导致错误定位和溶酶体贮积。研究强调了抗体在亚细胞定位研究中的重要性。
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4. **文献名称**：*CLN8 disease caused by large genomic deletions*
**作者**：Sara E. Mole et al.
**摘要**：研究利用CLN8抗体对患者细胞进行蛋白表达检测，发现大片段基因组缺失导致CLN8完全缺失，抗体检测结果为阴性，支持了CLN8抗体在临床诊断中的应用价值。
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这些文献均涉及CLN8抗体的实验应用，涵盖功能机制、疾病模型及诊断研究。如需具体文献来源，可进一步通过PubMed或期刊数据库检索标题及作者。
CLN8 antibodies are essential tools in studying neuronal ceroid lipofuscinosis type 8 (CLN8 disease), a rare autosomal recessive neurodegenerative disorder classified under neuronal ceroid lipofuscinoses (NCLs). CLN8 disease, caused by mutations in the *CLN8* gene, manifests with progressive neurological decline, seizures, vision loss, and motor dysfunction, typically emerging in late infancy or childhood. The CLN8 protein is a transmembrane protein localized to the endoplasmic reticulum (ER) and ER-Golgi intermediate compartment, implicated in lysosomal homeostasis, lipid metabolism, and intracellular trafficking. Its exact molecular mechanisms remain unclear, though it is suggested to regulate ER-associated degradation (ERAD) or lysosomal enzyme sorting.
CLN8-specific antibodies enable researchers to detect protein expression, assess subcellular localization, and investigate functional interactions in cellular and animal models. These antibodies are critical for diagnosing CLN8 disease via immunohistochemistry or Western blotting, confirming pathogenic variants, and validating experimental therapies. Studies using CLN8 antibodies have revealed reduced or absent protein levels in patient-derived cells, correlating with disease severity. Additionally, they aid in exploring CLN8's role in disease pathways, such as aberrant lysosomal storage or disrupted autophagy. Challenges include limited antibody availability and the need for specificity validation due to CLN8's structural homology with other ER-resident proteins. Ongoing research aims to refine antibody-based assays to support therapeutic development, including gene therapy or small-molecule interventions targeting CLN8-related pathways.
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