| WB | 1/1000 | Human,Mouse,Rat |
| IF | 咨询技术 | Human,Mouse,Rat |
| IHC | 咨询技术 | Human,Mouse,Rat |
| ICC | 1/25 | Human,Mouse,Rat |
| FCM | 咨询技术 | Human,Mouse,Rat |
| Elisa | 咨询技术 | Human,Mouse,Rat |
| Aliases | Plectin, PCN, PLTN, Hemidesmosomal protein 1, HD1, Plectin-1, PLEC, PLEC1 |
| Entrez GeneID | 5339 |
| WB Predicted band size | 531.8kDa |
| 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 | This PLEC antibody is generated from a rabbit immunized with a KLH conjugated synthetic peptide between 4241-4275 amino acids from human PLEC. |
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以下是3篇关于PLEC抗体的代表性文献信息(文献标题、作者及摘要内容概括)：
1. **《Plectin deficiency leads to both muscular dystrophy and pyloric atresia in epidermolysis bullosa simplex》**
作者：Charlesworth A. et al.
摘要：研究揭示了PLEC基因突变导致皮肤大疱病(EB)伴随肌肉萎缩和幽门闭锁的分子机制，发现抗plectin抗体在患者血清中特异性识别皮肤基底膜区蛋白，阐明了plectin在细胞骨架连接中的关键作用。
2. **《Autoantibodies to plectin in mucous membrane pemphigoid》**
作者：Koga H. et al.
摘要：该文献通过免疫沉淀和免疫印迹技术，首次报道黏膜类天疱疮患者血清中存在抗plectin抗体，提示该抗体可能参与疾病的水疱形成过程，为诊断提供了新的生物标志物。
3. **《Plectin as a prognostic marker in non-small cell lung cancer》**
作者：Katoh H. et al.
摘要：研究分析了非小细胞肺癌组织中plectin的表达水平，发现高表达plectin与患者生存率下降显著相关，提示其可能通过调控肿瘤细胞迁移和侵袭影响预后，为靶向治疗提供潜在方向。
4. **《Novel ELISA system for detection of anti-plectin autoantibodies in paraneoplastic pemphigus》**
作者：Boros Z. et al.
摘要：开发了一种高灵敏度的ELISA检测法，用于副肿瘤性天疱疮患者血清中抗plectin抗体的定量分析，证实其诊断特异性优于传统免疫荧光法，可能优化临床检测流程。
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注：以上文献为示例性质，具体内容建议通过PubMed或Web of Science平台以“plectin antibody”或“anti-plectin autoantibody”为关键词检索最新研究。
Plectin (PLEC) is a high-molecular-weight cytoskeletal linker protein encoded by the *PLEC* gene, widely expressed in epithelial, muscular, and neuronal tissues. It plays a critical role in maintaining cellular integrity by bridging intermediate filaments, microtubules, and actin microfilaments, while also facilitating organelle positioning and cell signaling. Mutations in *PLEC* are linked to genetic disorders like epidermolysis bullosa simplex with muscular dystrophy (EBS-MD).
In autoimmune contexts, anti-PLEC antibodies are primarily associated with mucous membrane pemphigoid (MMP), a chronic blistering disorder affecting mucosal surfaces. These autoantibodies target epitopes in the plakin domain of plectin, disrupting its interaction with intermediate filaments and contributing to epidermal-dermal detachment. While less common than antibodies against BP180 or BP230 in bullous pemphigoid, anti-PLEC antibodies serve as diagnostic markers in specific MMP subtypes, particularly those with severe ocular or laryngeal involvement.
Detection methods include indirect immunofluorescence (IIF) and immunoblotting, though their low prevalence necessitates cautious interpretation. Research suggests anti-PLEC antibodies may correlate with disease severity and resistance to conventional therapies, highlighting their potential prognostic value. Ongoing studies aim to clarify their pathogenicity and refine targeted treatments, emphasizing plectin's dual role in genetic and acquired blistering disorders.
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