| WB | 1/500-1/1000 | Human,Mouse,Rat |
| IF | 1/20 | Human,Mouse,Rat |
| IHC | 1/50-1/100 | Human,Mouse,Rat |
| ICC | 1/50-1/200 | Human,Mouse,Rat |
| FCM | 1/50-1/100 | Human,Mouse,Rat |
| Elisa | 咨询技术 | Human,Mouse,Rat |
| Aliases | mitochondrial; ODPA_HUMAN; PDH; PDHA; PDHA1; PDHCE1A; PDHE1 A type I; PDHE1-A type I; PHE1A; Pyruvate Dehydrogenase (lipoamide) alpha 1; Pyruvate dehydrogenase complex; E1 alpha polypeptide 1; Pyruvate Dehydrogenase E1 alpha; Pyruvate dehydrogenase E1 component subunit alpha; Pyruvate dehydrogenase E1 component subunit alpha; somatic form; mitochondrial; somatic form. |
| Entrez GeneID | 5160 |
| WB Predicted band size | Calculated MW: 43 kDa; Observed MW: 43 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 PDHA1 |
| Formulation | Purified antibody in PBS with 0.05% sodium azide. |
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以下是关于Pyruvate Dehydrogenase E1 Alpha(PDHA1)抗体的3篇模拟参考文献示例(实际文献需通过学术数据库验证)：
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1. **文献名称**：*Autoantibodies against pyruvate dehydrogenase E1α in primary biliary cirrhosis: Diagnostic utility and epitope specificity*
**作者**：Van de Water, J., Gershwin, M.E., et al.
**摘要**：该研究探讨了抗PDHA1抗体在原发性胆汁性胆管炎(PBC)患者血清中的诊断价值，通过ELISA和免疫印迹法验证其特异性，发现约30%的PBC患者存在该抗体，提示其可能作为辅助诊断标志物。
2. **文献名称**：*Structural characterization of pyruvate dehydrogenase E1α subunit and its autoimmune epitopes*
**作者**：Yang, X., Klyuyeva, A., et al.
**摘要**：利用X射线晶体学解析PDHA1蛋白结构，并定位其与自身抗体结合的线性表位(如氨基酸残基100-120区域)，为研究丙酮酸脱氢酶复合物相关自身免疫疾病机制提供依据。
3. **文献名称**：*PDHA1 mutations and antibody cross-reactivity in mitochondrial encephalopathies*
**作者**：Brown, R.M., Dahl, H.H., et al.
**摘要**：分析PDHA1基因突变导致的丙酮酸脱氢酶缺乏症患者样本，发现部分患者血清中存在抗PDHA1抗体，可能与线粒体功能障碍引发的自身免疫反应相关。
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**注意**：以上文献为模拟示例，实际引用时请通过PubMed/Google Scholar等平台检索真实文献(关键词：PDHA1 antibody, pyruvate dehydrogenase autoantibody)。
The pyruvate dehydrogenase E1 alpha (PDH-E1α) antibody is a crucial tool in studying the mitochondrial multienzyme complex responsible for cellular energy metabolism. PDH-E1α is a subunit of the pyruvate dehydrogenase complex (PDC), which catalyzes the oxidative decarboxylation of pyruvate to acetyl-CoA, linking glycolysis to the tricarboxylic acid (TCA) cycle. Dysregulation of PDC activity is implicated in metabolic disorders, neurodegenerative diseases, and cancer.
PDH-E1α antibodies specifically target the α-subunit of the E1 component, a heterotetramer (α2β2) that requires thiamine pyrophosphate as a cofactor. These antibodies are widely used in research to investigate PDC expression, activity, and post-translational modifications (e.g., phosphorylation-mediated inactivation). They aid in diagnosing genetic deficiencies in PDHA1 (the gene encoding PDH-E1α), which cause severe metabolic disorders like Leigh syndrome. Additionally, they are employed in studying metabolic reprogramming in cancers, where altered PDC function supports tumor growth via the Warburg effect.
Available as monoclonal or polyclonal forms, these antibodies are validated for techniques including Western blotting, immunohistochemistry, and ELISA. Researchers prioritize antibodies with high specificity, often verified using knockout controls or recombinant proteins. Their applications extend to exploring mitochondrial dysfunction in Alzheimer’s disease, diabetes, and other conditions linked to impaired glucose oxidation. Proper validation remains critical due to potential cross-reactivity with related dehydrogenases or isoforms.
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