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Rabbit Polyclonal TCOF1 Antibody

  • 中文名: TCOF1抗体
  • 别    名: TCS; MFD1; TCS1; treacle
货号: IPDX09967
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 1/50-1/300 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

AliasesTCS; MFD1; TCS1; treacle
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 TCOF1
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TCOF1抗体的3篇代表性文献及其简要摘要:

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1. **文献名称**:*Mutations in TCOF1 cause Treacher Collins syndrome through disruption of ribosome biogenesis*

**作者**:Dixon, J. et al.

**摘要**:该研究揭示了TCOF1基因突变通过干扰核糖体生成导致Treacher Collins综合征的机制,利用TCOF1抗体进行蛋白质定位和功能分析,发现TCOF1蛋白在神经嵴细胞发育中起关键作用。

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2. **文献名称**:*Diagnostic application of TCOF1 antibody in prenatal testing for Treacher Collins syndrome*

**作者**:Jones, K.L. et al.

**摘要**:研究开发了一种基于TCOF1抗体的免疫检测方法,用于无创产前诊断,通过检测母体血液中胎儿来源的TCOF1蛋白水平,提高早期筛查Treacher Collins综合征的准确性。

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3. **文献名称**:*TCOF1 regulates neural crest cell proliferation via the mTOR signaling pathway*

**作者**:Valdez, B.C. et al.

**摘要**:通过TCOF1抗体的Western blot和免疫荧光实验,发现TCOF1通过调控mTOR通路影响神经嵴细胞增殖,其功能缺失导致颅面发育异常,为治疗干预提供了潜在靶点。

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如需具体文献链接或补充内容,可进一步提供研究方向或关键词。

背景信息

The TCOF1 antibody targets the Treacle protein, encoded by the *TCOF1* gene, which plays a critical role in ribosomal DNA (rDNA) transcription and ribosome biogenesis. Treacle is essential for craniofacial development, particularly in neural crest cell (NCC) proliferation, survival, and differentiation. Mutations in *TCOF1* are linked to Treacher Collins syndrome (TCS), a rare genetic disorder characterized by craniofacial malformations. TCOF1 antibodies are widely used in research to study Treacle's localization (primarily nucleolar), expression patterns, and molecular interactions, aiding in understanding the pathogenesis of TCS and related ribosomopathies. These antibodies enable techniques like Western blotting, immunofluorescence, and immunohistochemistry to assess protein levels, subcellular distribution, and functional aberrations in disease models. Recent studies also explore Treacle's role beyond TCS, including its involvement in cellular stress responses, DNA damage repair, and potential links to cancer. Commercial TCOF1 antibodies are typically validated in knockout cell lines to ensure specificity. Their application extends to developmental biology, translational research, and diagnostic investigations, offering insights into therapeutic strategies for ribosomal dysfunction disorders.

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