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Rabbit Polyclonal CLN3 Antibody

  • 中文名: CLN3抗体
  • 别    名: Battenin, Batten disease protein, Protein CLN3, CLN3, BTS
货号: IPDX31261
Price: ¥1180
数量:
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验证与应用

应用及物种
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

产品详情

AliasesBattenin, Batten disease protein, Protein CLN3, CLN3, BTS
Entrez GeneID1201
WB Predicted band size47.6kDa
Host/IsotypeRabbit IgG
Antibody TypePrimary antibody
StorageStore at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles.
Species ReactivityHuman
ImmunogenThis CLN3 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 235-263 amino acids from the Central region of human CLN3.
FormulationPurified antibody in PBS with 0.05% sodium azide.

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参考文献

以下是关于CLN3抗体的3篇参考文献的简要信息,涵盖其在不同研究中的应用及发现:

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1. **文献名称**: *"Characterization of a Novel Antibody to CLN3 Reveals Its Role in Autophagy and Neuronal Ceroid Lipofuscinosis"*

**作者**: Smith J, et al.

**摘要**: 该研究开发了一种特异性识别CLN3蛋白的高效抗体,证实CLN3在自噬溶酶体途径中起关键作用。通过免疫荧光和Western blot分析,发现CLN3缺失导致溶酶体功能异常,提示其与神经元蜡样脂褐质沉积症的病理机制相关。

2. **文献名称**: *"CLN3 Protein Localization and Interaction Networks in Murine Brain Tissue Using Immunohistochemistry"*

**作者**: Chen L, et al.

**摘要**: 利用新型CLN3抗体对小鼠脑组织进行免疫组化分析,发现CLN3广泛表达于皮层和海马神经元中,并定位于内体和线粒体膜。研究揭示了CLN3与多种膜转运蛋白的相互作用,为理解其在神经退行中的功能提供线索。

3. **文献名称**: *"Antibody-Based Detection of CLN3 in Patient-Derived Cells: Implications for Diagnostic Assays"**

**作者**: Gonzalez R, et al.

**摘要**: 研究评估了CLN3抗体在Batten病患者细胞中的诊断潜力。通过流式细胞术和ELISA,发现患者细胞中CLN3蛋白表达显著降低,证明该抗体可作为快速筛查CLN3基因突变相关疾病的工具。

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**备注**:以上文献信息为示例性质,实际引用时需根据具体论文内容调整。建议通过PubMed或Google Scholar以关键词“CLN3 antibody”搜索最新研究获取准确文献。

背景信息

CLN3 antibodies are immunological tools used to study the CLN3 protein, which is encoded by the *CLN3* gene located on human chromosome 16. Mutations in this gene are linked to Batten disease (neuronal ceroid lipofuscinosis type 3. CLN3 disease), a rare, fatal autosomal recessive neurodegenerative disorder affecting children. The CLN3 protein is a transmembrane protein predominantly localized to lysosomes and endosomes, though its exact function remains unclear. It is hypothesized to play roles in lysosomal acidification, autophagy, vesicle trafficking, and apoptosis regulation.

CLN3 antibodies are critical for detecting and quantifying the protein in research, aiding in understanding disease mechanisms. They are used in techniques like Western blotting, immunohistochemistry, and immunofluorescence to study CLN3 expression, localization, and interactions in cellular and animal models. Commercially available CLN3 antibodies are typically raised against specific epitopes of the human protein, often validated in knockout controls to ensure specificity. However, challenges persist due to the protein’s low abundance and hydrophobic nature, which can affect antibody performance. Research using these antibodies has highlighted CLN3’s involvement in lysosomal dysfunction and neuronal degeneration, though therapeutic strategies remain under investigation. Reliable CLN3 antibodies remain vital for advancing Batten disease research and potential biomarker development.

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