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Rabbit Polyclonal COL17A1 Antibody

  • 中文名: COL17A1抗体
  • 别    名: ERED; BP180; BPA-2; BPAG2; LAD-1; BA16H23.2
货号: IPDX13107
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 1/50-1/200 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

AliasesERED; BP180; BPA-2; BPAG2; LAD-1; BA16H23.2
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 COL17A1
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COL17A1抗体的3篇参考文献及其摘要概括:

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1. **文献名称**: "Autoantibodies against collagen XVII in bullous pemphigoid"

**作者**: Nishie W. et al.

**摘要**: 该研究发现大疱性类天疱疮(BP)患者的血清中存在靶向COL17A1(胶原XVII)的自身抗体,揭示了这些抗体通过破坏半桥粒结构导致表皮-真皮分离的分子机制,为BP的诊断和治疗提供了依据。

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2. **文献名称**: "Collagen XVII coordinates epidermal differentiation through regulation of laminin-332 expression"

**作者**: Tasanen K. et al.

**摘要**: 研究通过COL17A1抗体标记发现,胶原XVII通过调控层粘连蛋白-332的表达,在表皮分化和基底膜完整性中起关键作用,其缺失或抗体介导的破坏可能引发皮肤脆性和水疱形成。

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3. **文献名称**: "Mutation analysis and characterization of COL17A1 mutations in junctional epidermolysis bullosa"

**作者**: McGrath J.A. et al.

**摘要**: 本文利用COL17A1抗体进行免疫荧光检测,鉴定了交界型大疱性表皮松解症(JEB)患者的COL17A1基因突变,显示突变导致胶原XVII表达缺失或功能异常,强调了抗体在疾病分子诊断中的应用价值。

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4. **文献名称**: "Targeted disruption of the collagen XVII gene causes skin fragility and basement membrane defects"

**作者**: Heinonen S. et al.

**摘要**: 通过构建COL17A1基因敲除小鼠模型并结合抗体染色,研究发现胶原XVII缺失导致皮肤基底膜结构异常,证实其在维持表皮-真皮连接中的核心作用,为相关遗传性皮肤病的研究提供了模型基础。

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以上研究均聚焦于COL17A1抗体在疾病机制、诊断或功能研究中的应用。

背景信息

COL17A1. a gene encoding collagen type XVII alpha 1 chain (also known as BP180 or BPAG2), is a transmembrane protein critical for maintaining epidermal-dermal adhesion. As a key component of hemidesmosomes, it stabilizes the basement membrane zone by anchoring epithelial cells to the underlying extracellular matrix. COL17A1 is expressed in basal keratinocytes and plays roles in tissue integrity, wound healing, and stem cell niche regulation. Antibodies targeting COL17A1 are primarily studied in autoimmune blistering disorders, notably bullous pemphigoid (BP) and mucous membrane pemphigoid, where autoantibodies disrupt COL17A1 function, leading to subepidermal blistering. These antibodies are detected via immunofluorescence, ELISA, or immunoblotting in clinical diagnostics. Beyond autoimmune contexts, COL17A1 antibodies are used in research to explore its roles in epithelial-mesenchymal transition, cancer progression, and aging-related tissue degeneration. Mutations in COL17A1 also cause junctional epidermolysis bullosa, a genetic disorder characterized by fragile skin. Recent studies highlight COL17A1's involvement in stem cell maintenance, making its antibodies valuable tools for investigating regenerative mechanisms. Therapeutic strategies targeting COL17A1. such as antibody-blocking agents, are under exploration for autoimmune and degenerative conditions.

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