| WB | 1/500-1/1000 | Human,Mouse,Rat,Hamster |
| IF | 1/20 | Human,Mouse,Rat,Hamster |
| IHC | 咨询技术 | Human,Mouse,Rat,Hamster |
| ICC | 1/50-1/200 | Human,Mouse,Rat,Hamster |
| FCM | 咨询技术 | Human,Mouse,Rat,Hamster |
| Elisa | 咨询技术 | Human,Mouse,Rat,Hamster |
| Aliases | DLAT; DLTA; E2; PBC; PDCE2 |
| Entrez GeneID | 1737 |
| WB Predicted band size | Calculated MW: 69 kDa; Observed MW: 69 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,Hamster |
| Immunogen | A synthetic peptide of human Pyruvate Dehydrogenase E2 |
| Formulation | Purified antibody in TBS with 0.05% sodium azide,0.05%BSA and 50% glycerol. |
+ +
以下是3篇关于Pyruvate Dehydrogenase E2(PDC-E2)抗体的代表性文献摘要：
---
1. **文献名称**: *Primary biliary cirrhosis: an orchestrated immune response against epithelial cells*
**作者**: Gershwin ME, Mackay IR
**摘要**: 该综述提出PDC-E2抗体是原发性胆汁性胆管炎(PBC)的典型标志，靶向线粒体内膜的PDC-E2抗原表位，并探讨其与胆管上皮细胞凋亡后抗原暴露的关联性。
---
2. **文献名称**: *Heterogeneous antibody recognition patterns in primary biliary cirrhosis*
**作者**: Van de Water J, Gershwin ME
**摘要**: 研究发现PDC-E2抗体在95%的PBC患者血清中存在，其特异性高于其他线粒体抗体(AMA)，提示其作为PBC诊断和疾病活动性评估的关键生物标志物。
---
3. **文献名称**: *Epitope mapping of antibodies to PDC-E2 in primary biliary cirrhosis*
**作者**: Leung PS et al.
**摘要**: 通过表位定位技术揭示PDC-E2抗体的主要靶向区域为硫辛酰结构域，该区域在胆管细胞异常表达可能与PBC的自身免疫攻击机制相关。
---
如需获取具体期刊信息或DOI，可进一步提供数据库检索支持。
The pyruvate dehydrogenase complex (PDC) is a mitochondrial enzyme critical for cellular energy production, catalyzing the conversion of pyruvate to acetyl-CoA. The E2 subunit (dihydrolipoamide acetyltransferase) forms the structural core of PDC, facilitating substrate channeling and catalysis. Autoantibodies targeting PDC-E2 are strongly associated with primary biliary cholangitis (PBC), a chronic autoimmune liver disease characterized by destruction of intrahepatic bile ducts. In PBC, anti-PDC-E2 antibodies are detected in approximately 95% of patients, making them a hallmark serological marker. These antibodies specifically recognize the lipoyl domain of E2. which contains immunodominant epitopes.
The pathogenesis involves molecular mimicry, where cross-reactivity between microbial or environmental antigens and the E2 subunit triggers autoimmune responses. Unique to PBC, autoreactive T cells and antibodies target biliary epithelial cells, which aberrantly express PDC-E2 on their surface during apoptosis. This leads to persistent inflammation and fibrosis. While anti-PDC-E2 antibodies are central to PBC diagnosis, their role in disease progression remains unclear, as titers do not correlate with severity. Testing for these antibodies, often via ELISA or immunofluorescence, is crucial for differentiating PBC from other liver disorders. Research continues to explore their mechanistic involvement and potential therapeutic targets.
×
