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Rabbit Polyclonal BBS10 Antibody

  • 中文名: BBS10抗体
  • 别    名: C12orf58
货号: IPDX09352
Price: ¥1180
数量:
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验证与应用

应用及物种
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

产品详情

AliasesC12orf58
WB Predicted band size81 kDa
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
ImmunogenFusion protein of human BBS10
FormulationPurified antibody in PBS with 0.05% sodium azide and 50% glycerol.

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参考文献

以下是关于BBS10抗体的参考文献示例(注:以下内容为基于典型研究的概括性示例,具体文献请通过学术数据库核实):

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1. **文献名称**: *Mutations in BBS10 cause Bardet-Biedl syndrome with variable phenotypic penetrance*

**作者**: Stoetzel C, et al.

**摘要**: 该研究通过Western blot和免疫荧光技术,使用BBS10抗体检测患者细胞中蛋白表达水平,发现BBS10突变导致蛋白稳定性下降,与纤毛功能障碍及多器官异常相关。

2. **文献名称**: *BBS10 interacts with chaperonin complexes to modulate ciliogenesis*

**作者**: Zhang Q, et al.

**摘要**: 利用BBS10抗体进行共免疫沉淀实验,揭示BBS10与分子伴侣复合物的相互作用,表明其在纤毛形成中通过调控蛋白折叠发挥作用,突变可导致细胞定位异常。

3. **文献名称**: *Tissue-specific expression of BBS10 in murine models*

**作者**: Davis EE, et al.

**摘要**: 通过免疫组化结合BBS10抗体,系统分析小鼠模型中该蛋白的分布,发现其在肾脏、视网膜等高纤毛密度组织中高表达,提示与Bardet-Biedl综合征表型的关联性。

4. **文献名称**: *Functional characterization of BBS10 variants using CRISPR/Cas9 and antibody-based assays*

**作者**: Wei Q, et al.

**摘要**: 研究利用BBS10抗体进行流式细胞术和亚细胞定位分析,发现致病突变破坏蛋白-蛋白相互作用网络,导致纤毛信号传导缺陷。

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**建议**:如需具体文献,可通过PubMed或Google Scholar搜索关键词“BBS10 antibody”、“BBS10 protein analysis”等,并筛选方法学中提及抗体应用的论文。

背景信息

The BBS10 antibody is a crucial tool for studying Bardet-Biedl syndrome (BBS), a rare autosomal recessive ciliopathy characterized by retinal degeneration, obesity, polydactyly, renal anomalies, and cognitive impairment. BBS10. a gene located on chromosome 12q21.2. encodes a protein component of the BBSome complex, which regulates ciliary function and intracellular trafficking. Mutations in BBS10 account for ~20% of BBS cases, making it one of the most frequently implicated genes in the disorder. The BBS10 antibody specifically targets the BBS10 protein, enabling researchers to investigate its expression, localization, and interactions within cellular pathways. It is widely used in techniques like Western blotting, immunofluorescence, and immunohistochemistry to analyze tissue-specific expression patterns, validate disease models (e.g., knockout mice or zebrafish), and explore molecular mechanisms underlying ciliary dysfunction. Commercially available BBS10 antibodies are typically polyclonal or monoclonal, raised against specific epitopes of human or murine BBS10. Validation includes testing in BBS10-deficient cells or tissues to confirm specificity. Research utilizing this antibody has advanced understanding of BBS pathogenesis, cilia-related signaling, and potential therapeutic targets for syndromic ciliopathies.

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