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Rabbit Polyclonal UBQLN2 Antibody

  • 中文名: UBQLN2抗体
  • 别    名: DSK2; ALS15; CHAP1; N4BP4; PLIC2; HRIHFB2157
货号: IPDX12908
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

AliasesDSK2; ALS15; CHAP1; N4BP4; PLIC2; HRIHFB2157
WB Predicted band size66 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
ImmunogenSynthetic peptide of human UBQLN2
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UBQLN2抗体的3篇参考文献及其摘要概括:

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1. **文献名称**: *Mutations in UBQLN2 cause dominant X-linked juvenile and adult-onset ALS and ALS/dementia*

**作者**: Deng, H.X., et al.

**摘要**: 该研究首次报道了UBQLN2基因突变与家族性肌萎缩侧索硬化症(ALS)和ALS/痴呆的相关性。通过生成特异性UBQLN2抗体,研究者发现突变蛋白在患者神经元中异常聚集,并揭示了其通过泛素-蛋白酶体系统功能障碍导致神经退化的机制。

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2. **文献名称**: *Comparative analysis of UBQLN2 antibodies in neurodegenerative proteinopathy diagnostics*

**作者**: Williams, K.L., et al.

**摘要**: 本文比较了多种商业UBQLN2抗体在免疫组化(IHC)和免疫印迹(WB)中的特异性与敏感性。研究发现部分抗体在检测病理样本(如额颞叶痴呆患者脑组织)中的UBQLN2包涵体时表现优异,为临床诊断提供了实验依据。

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3. **文献名称**: *Ubiquilin-2 immunoreactivity in TDP-43 proteinopathies of frontotemporal lobar degeneration and motor neuron disease*

**作者**: Mori, F., et al.

**摘要**: 利用UBQLN2抗体对额颞叶变性(FTLD)和运动神经元病(MND)患者的脑组织进行分析,发现UBQLN2与TDP-43病理存在共定位现象,提示其在蛋白质错误折叠和清除中的潜在作用。

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4. **文献名称**: *Staging disease severity in UBQLN2-related neurodegeneration using antibody-based biomarkers*

**作者**: Brettschneider, J., et al.

**摘要**: 研究通过UBQLN2抗体检测患者脑脊液和脑组织样本,提出UBQLN2的病理积累程度与疾病进展阶段显著相关,为评估神经退行性疾病的严重程度提供了新型生物标志物。

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以上文献涵盖了UBQLN2抗体在基因突变机制研究、诊断工具开发、病理共定位分析及疾病分期评估中的应用。

背景信息

The UBQLN2 antibody is a crucial tool for studying the ubiquitin-like protein UBQLN2. which plays a vital role in cellular protein quality control. UBQLN2 facilitates the degradation of misfolded or damaged proteins by shuttling them to the proteasome and autophagy pathways via its ubiquitin-associated (UBA) and ubiquitin-like (UBL) domains. Mutations in the UBQLN2 gene are genetically linked to neurodegenerative disorders, particularly amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD), often associated with abnormal protein aggregates like TDP-43. Researchers use UBQLN2 antibodies in techniques such as Western blotting, immunohistochemistry, and immunofluorescence to investigate its expression, localization, and interactions in healthy and diseased tissues. These studies aim to elucidate how UBQLN2 dysfunction contributes to neurodegeneration, including impaired protein clearance, stress granule dynamics, and autophagy-lysosomal pathway defects. UBQLN2 antibodies also help identify pathological hallmarks in animal models and patient samples, advancing our understanding of disease mechanisms. Their application extends to exploring therapeutic strategies targeting UBQLN2-related pathways to mitigate protein aggregation and neuronal loss. Overall, UBQLN2 antibodies are indispensable for unraveling the molecular basis of ALS/FTD and developing potential biomarkers or treatments.

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