| WB | 咨询技术 | Human,Mouse,Rat |
| IF | 咨询技术 | Human,Mouse,Rat |
| IHC | 咨询技术 | Human,Mouse,Rat |
| ICC | 技术咨询 | Human,Mouse,Rat |
| FCM | 咨询技术 | Human,Mouse,Rat |
| Elisa | 1/1000-1/2000 | Human,Mouse,Rat |
| Aliases | DG1; DSG; CDHF4; PPKS1; SPPK1 |
| WB Predicted band size | 114 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 |
| Immunogen | Synthetic peptide of human DSG1 |
| Formulation | Purified antibody in PBS with 0.05% sodium azide and 50% glycerol. |
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以下是3-4条关于DSG1抗体的参考文献，按文献名称、作者和摘要内容简要概括：
1. **《Autoantibodies against the amino-terminal cadherin-like binding domain of pemphigus vulgaris antigen are pathogenic》**
- **作者**：Amagai, M., Klaus-Kovtun, V., & Stanley, J.R.
- **摘要**：该研究通过实验证明，抗DSG1(桥粒芯蛋白1)的IgG抗体直接破坏角质形成细胞间的粘附，导致落叶型天疱疮(PF)的皮肤水疱。研究首次揭示了DSG1抗体在疾病中的致病机制。
2. **《The clinical phenotype of pemphigus is defined by the anti-desmoglein autoantibody profile》**
- **作者**：Mahoney, M.G., Wang, Z., Rothenberger, K., et al.
- **摘要**：文章分析了不同天疱疮亚型(如PF和寻常型天疱疮)患者中DSG1和DSG3抗体的分布，发现DSG1抗体与PF的皮肤病变高度相关，且抗体水平与疾病严重程度呈正相关。
3. **《Endemic pemphigus foliaceus (fogo selvagem) in Brazil: Cooperative research on pathogenic mechanisms》**
- **作者**：Diaz, L.A., Sampaio, S.A., Rivitti, E.A., et al.
- **摘要**：该研究调查了巴西地方性落叶型天疱疮(fogo selvagem)患者的DSG1抗体特征，发现环境因素(如虫媒叮咬)可能触发针对DSG1的自身免疫反应，导致慢性皮肤损伤。
4. **《Paraneoplastic pemphigus: An autoimmune mucocutaneous disease associated with neoplasia》**
- **作者**：Anhalt, G.J., Kim, S.C., Stanley, J.R., et al.
- **摘要**：研究描述了副肿瘤性天疱疮患者中多种自身抗体(包括抗DSG1抗体)的存在，并指出DSG1抗体可能参与广泛性黏膜和皮肤损伤的发生，但与其他天疱疮亚型相比，其病理机制更为复杂。
以上文献涵盖DSG1抗体的致病机制、临床相关性及在不同疾病背景下的作用，可作为研究自身免疫性皮肤病的核心参考资料。
Desmoglein 1 (DSG1) is a transmembrane glycoprotein belonging to the desmoglein subfamily of cadherins, primarily expressed in stratified epithelial tissues, particularly the upper layers of the epidermis. It plays a critical role in cell-cell adhesion by forming desmosomes, which are intercellular junctions essential for maintaining tissue integrity and mechanical resilience. DSG1 antibodies are autoantibodies targeting this protein, often implicated in autoimmune blistering disorders. The most notable association is with pemphigus foliaceus (PF), a chronic autoimmune condition characterized by superficial blisters and erosions. In PF, DSG1 antibodies disrupt desmosomal adhesion, leading to acantholysis (separation of epidermal cells). These antibodies are predominantly IgG and can be detected via enzyme-linked immunosorbent assay (ELISA) or indirect immunofluorescence.
DSG1 antibodies are less frequently linked to mucosal-dominant pemphigus vulgaris (PV), where DSG3 antibodies are typically primary. However, DSG1/DSG3 antibody coexistence may occur in atypical or overlapping cases. Environmental triggers, genetic predisposition (e.g., HLA-DRB1 alleles), and epitope-specific binding patterns influence disease manifestation. Research also explores DSG1 antibodies in paraneoplastic pemphigus and drug-induced pemphigus. Their pathogenicity is confirmed by passive transfer experiments in mice, replicating blistering phenotypes. Clinically, DSG1 antibody levels often correlate with disease activity, aiding diagnosis and therapeutic monitoring. Understanding DSG1 antibody dynamics continues to inform targeted therapies, including immunoadsorption and biologics, improving management of autoimmune blistering diseases.
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