| WB | 咨询技术 | Human,Mouse,Rat |
| IF | 1/20-1/50 | Human,Mouse,Rat |
| IHC | IHC:1/100-1/200;IHF:1/50-1/200 | Human,Mouse,Rat |
| ICC | 1/50-1/200 | Human,Mouse,Rat |
| FCM | 咨询技术 | Human,Mouse,Rat |
| Elisa | 咨询技术 | Human,Mouse,Rat |
| Aliases | BP180; BPA 2; BPAG2; COL17A1; Collagen 17; CollagenXVII; LAD1;Collagen XVII alpha 1 |
| WB Predicted band size | Calculated MW: 150 kDa ; Observed MW: 130,180 kDa |
| Host/Isotype | Rabbit IgG |
| Antibody Type | Primary antibody |
| Storage | Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles. |
| Species Reactivity | Human,Mouse,Rat |
| Immunogen | A synthesized peptide derived from human Collagen XVII alpha 1 |
| Formulation | Purified antibody in PBS with 0.05% sodium azide,0.05% BSA and 50% glycerol. |
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以下是关于Collagen XVII抗体的3篇代表性文献：
1. **文献名称**: *Autoantibodies to collagen XVII in bullous pemphigoid*
**作者**: van den Bergh F, Giudice GJ
**摘要**: 该研究揭示了大疱性类天疱疮(BP)患者血清中的自身抗体主要靶向Collagen XVII(BP180)的胞外结构域NC16A，并证实这些抗体通过激活补体通路导致表皮下水疱形成。
2. **文献名称**: *Collagen XVII deficiency induces epidermal aging in mice*
**作者**: Nishie W, Sawamura D, Goto M, et al.
**摘要**: 通过构建Col17a1基因敲除小鼠模型，研究发现Collagen XVII缺失导致表皮干细胞耗竭和皮肤老化表型，表明该蛋白在维持表皮稳态中起关键作用。
3. **文献名称**: *The role of collagen XVII in autoimmune blistering diseases*
**作者**: Thoma-Uszynski S, Uter W, Schwietzke S, et al.
**摘要**: 本文综述了Collagen XVII在自身免疫性水疱病(如黏膜类天疱疮)中的病理机制，强调其抗体可通过阻断蛋白功能或诱导炎症反应导致表皮-真皮分离。
如需更多文献，可进一步补充。
Collagen XVII, also known as BP180 or BPAG2. is a transmembrane protein primarily expressed in hemidesmosomes of basal keratinocytes, where it plays a critical role in maintaining dermal-epidermal adhesion. It consists of a collagenous extracellular domain and a cytoplasmic N-terminal domain, functioning as a structural component and signaling mediator. Autoantibodies targeting Collagen XVII are central to the pathogenesis of autoimmune blistering diseases, particularly bullous pemphigoid (BP) and mucous membrane pemphigoid. In BP, IgG autoantibodies bind to the NC16A domain of Collagen XVII, triggering inflammatory cascades that lead to blister formation.
Diagnostically, Collagen XVII antibodies are detected via immunofluorescence, ELISA, or immunoblotting, aiding in disease confirmation and monitoring. Research also explores their role in rare genetic disorders like junctional epidermolysis bullosa, where Collagen XVII mutations cause skin fragility. Therapeutic strategies targeting these antibodies, such as immunoadsorption or biologics, are under investigation. Recent studies focus on epitope mapping, antibody subclasses, and their correlation with disease severity, enhancing understanding of pathophysiology and treatment optimization. Additionally, Collagen XVII-deficient animal models have been instrumental in studying skin homeostasis and repair mechanisms.
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