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Rabbit Monoclonal CHMP2B Antibody

  • 中文名: CHMP2B抗体
  • 别    名: ALS17; CHMP2b; DMT1; hVps2-2; VPS2B;;CHMP2B
货号: IPDX18812
Price: ¥1280
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 1/20-1/50 Human,Mouse,Rat
IHC 咨询技术 Human,Mouse,Rat
ICC 1/50-1/200 Human,Mouse,Rat
FCM 1/20-1/100 Human,Mouse,Rat
Elisa 咨询技术 Human,Mouse,Rat

产品详情

AliasesALS17; CHMP2b; DMT1; hVps2-2; VPS2B;;CHMP2B
WB Predicted band sizeCalculated MW: 24 kDa ; Observed MW: 28 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,Mouse,Rat
ImmunogenA synthesized peptide derived from human CHMP2B
FormulationPurified antibody in PBS with 0.05% sodium azide,0.05% BSA and 50% glycerol.

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参考文献

以下是关于CHMP2B抗体的3篇参考文献及其摘要内容:

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1. **文献名称**: *Mutations in the endosomal ESCRTIII-complex subunit CHMP2B in frontotemporal dementia*

**作者**: Skibinski, G. et al. (2005)

**摘要**: 该研究首次发现CHMP2B基因突变与家族性额颞叶痴呆(FTD)的关联。通过抗体检测,发现突变导致ESCRT-III复合体功能异常,引发神经元内溶酶体功能障碍和自噬体积累,为神经退行性疾病的机制提供了新见解。

2. **文献名称**: *CHMP2B regulates autophagy and lysosomal function in neurodegenerative disease models*

**作者**: Lee, J.A. et al. (2008)

**摘要**: 研究利用CHMP2B特异性抗体分析突变蛋白在细胞和小鼠模型中的表达,发现CHMP2B缺失导致自噬体-溶酶体融合障碍,加剧神经细胞死亡,揭示了其在维持溶酶体功能中的关键作用。

3. **文献名称**: *Characterization of a polyclonal antibody against human CHMP2B for neurodegenerative disease research*

**作者**: Clayton, E.L. et al. (2010)

**摘要**: 本文详细描述了一种多克隆CHMP2B抗体的开发与验证。通过免疫印迹和免疫荧光实验,证明该抗体可特异性识别内源性和过表达的CHMP2B蛋白,适用于神经退行性疾病模型的病理机制研究。

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以上文献涵盖了CHMP2B在疾病关联、功能机制及抗体开发中的应用,均为该领域的关键研究。如需扩展或补充其他研究,请随时告知!

背景信息

The CHMP2B (Charged Multivesicular Body Protein 2B) antibody is a tool used to study the CHMP2B protein, a key component of the ESCRT-III (Endosomal Sorting Complex Required for Transport-III) complex. ESCRT-III mediates critical membrane remodeling processes, including cytokinesis, multivesicular body (MVB) formation, and viral budding. CHMP2B plays a role in membrane scission and cargo sorting, particularly in endosomal-lysosomal trafficking.

Mutations in the CHMP2B gene are linked to neurodegenerative disorders, notably frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS). These mutations, such as the pathogenic truncating variant (e.g., p.Thr104Asnfs*5), disrupt ESCRT-III function, leading to aberrant endosomal-lysosomal trafficking, accumulation of autophagosomes, and protein aggregates. CHMP2B-related FTD is characterized by neuronal inclusions positive for ubiquitin, p62. and TDP-43. though distinct from other FTD subtypes.

CHMP2B antibodies are essential in research to detect protein expression, localization, and aggregation in cellular and animal models. They are used in techniques like Western blotting, immunohistochemistry, and immunofluorescence to explore disease mechanisms, such as impaired autophagy and lysosomal dysfunction. Additionally, these antibodies aid in studying the interplay between CHMP2B mutations and other ALS/FTD-associated proteins (e.g., C9orf72. TDP-43). Understanding CHMP2B pathology contributes to developing therapeutic strategies targeting endosomal-lysosomal pathways in neurodegeneration.

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