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- bs-19695R-Bio
- 2025年09月30日
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100ul
| 产品编号 | bs-19695R-Bio |
| 英文名称 | DLAT Rabbit pAb, Biotin conjugated |
| 中文名称 | Biotin标记的丙酮酸脱氢酶E2抗体 |
| 英文别名 | ODP2_HUMAN; Dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex, mitochondrial; EC:2.3.1.12; 70 kDa mitochondrial autoantigen of primary biliary cirrhosis(PBC); Dihydrolipoamide acetyltransferase component of pyruvate dehydrogenase complex; M2 antigen complex 70 kDa subunit; Pyruvate dehydrogenase complex component E2(PDC-E2; PDCE2); dihydrolipoamide S-acetyltransferase; E2; PBC; DLTA; PDCE2; PDC-E2; |
| 产品应用 | WB=1:500-2000, IHC-P=1:100-500, IHC-F=1:100-500, IF=1:100-500 Not yet tested in other applications. |
| 抗体来源 | Rabbit |
| 免疫原 | KLH conjugated synthetic peptide derived from human PDCE2 |
| 亚型 | IgG |
| 纯化方法 | affinity purified by Protein A |
| 克隆类型 | Polyclonal |
| 浓度 | 1mg/ml |
| 储存液 | 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol. |
| 研究领域 | Cancer > Cancer Metabolism > Metabolic signaling pathway > Metabolism of carbohydrates Metabolism > Pathways and Processes > Metabolic signaling pathways > Carbohydrate metabolism Metabolism > Pathways and Processes > Metabolic signaling pathways > Energy transfer pathways > Energy Metabolism Metabolism > Pathways and Processes > Mitochondrial Metabolism > Mitochondrial markers Metabolism > Types of disease > Cancer Signal Transduction > Metabolism > Energy Metabolism Signal Transduction > Metabolism > Mitochondrial |
| 亚细胞定位 | Mitochondrion matrix. |
| 相似性 | Belongs to the 2-oxoacid dehydrogenase family. _x000D_ Contains 2 lipoyl-binding domains. |
| 功能 | The pyruvate dehydrogenase complex catalyzes the overall conversion of pyruvate to acetyl-CoA and CO(2). It contains multiple copies of three enzymatic components: pyruvate dehydrogenase (E1), dihydrolipoamide acetyltransferase (E2) and lipoamide dehydrogenase (E3). |
| 保存条件 | Shipped at 4℃. Store at -20°C for one year. Avoid repeated freeze/thaw cycles. |
| 注意事项 | This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
| 背景资料 | This gene encodes component E2 of the multi-enzyme pyruvate dehydrogenase complex (PDC). PDC resides in the inner mitochondrial membrane and catalyzes the conversion of pyruvate to acetyl coenzyme A. The protein product of this gene, dihydrolipoamide acetyltransferase, accepts acetyl groups formed by the oxidative decarboxylation of pyruvate and transfers them to coenzyme A. Dihydrolipoamide acetyltransferase is the antigen for antimitochondrial antibodies. These autoantibodies are present in nearly 95% of patients with the autoimmune liver disease primary biliary cirrhosis (PBC). In PBC, activated T lymphocytes attack and destroy epithelial cells in the bile duct where this protein is abnormally distributed and overexpressed. PBC enventually leads to cirrhosis and liver failure. Mutations in this gene are also a cause of pyruvate dehydrogenase E2 deficiency which causes primary lactic acidosis in infancy and early childhood.[provided by RefSeq, Oct 2009] |
| 应用 | 推荐稀释比例 |
| {WB} | {1:500-2000} |
| {IHC-P} | {1:100-500} |
| {IHC-F} | {1:100-500} |
| {IF} | {1:100-500} |
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