| 产品编号 | bs-7610R |
| 英文名称 | ETHE1 |
| 中文名称 | 乙基丙二酸脑病蛋白抗体 |
| 别 名 | mitochondrial; Ethe1; ETHE1 protein, mitochondrial precursor; ETHE1_HUMAN; ethylmalonic encephalopathy 1; Ethylmalonic encephalopathy protein 1; hepatoma subtracted clone one; Hepatoma subtracted clone one protein; HSCO; Protein ETHE1; YF13H12. |
| 研究领域 | 细胞生物 信号转导 细胞凋亡 |
| 抗体来源 | Rabbit |
| 克隆类型 | Polyclonal |
| 交叉反应 | (predicted: Human,Mouse,Dog,Pig,Cow,Rabbit,Sheep) |
| 产品应用 | ELISA=1:5000-10000
not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 理论分子量 | 26kDa |
| 细胞定位 | 细胞核 细胞浆 |
| 性 状 | Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human ETHE1: 191-254/254 |
| 亚 型 | IgG |
| 纯化方法 | affinity purified by Protein A |
| 缓 冲 液 | 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol. |
| 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. |
| PubMed | PubMed |
| 产品介绍 |
Probably plays an important role in metabolic homeostasis in mitochondria. May function as a nuclear-cytoplasmic shuttling protein that binds transcription factor RELA/NFKB3 in the nucleus and exports it to the cytoplasm. Suppresses p53-induced apoptosis by preventing nuclear localization of RELA. Involvement in disease:Defects in ETHE1 are a cause of ethylmalonic encephalopathy (EE) . EE is an autosomal recessive disorder characterized by neurodevelopmental delay and regression, recurrent petechiae, acrocyanosis, diarrhea, leading to death in the first decade of life. It is also associated with persistent lactic acidemia and ethylmalonic and methylsuccinic aciduria. Function: Probably plays an important role in metabolic homeostasis in mitochondria. May function as a nuclear-cytoplasmic shuttling protein that binds transcription factor RELA/NFKB3 in the nucleus and exports it to the cytoplasm. Suppresses p53-induced apoptosis by preventing nuclear localization of RELA. Subunit: Interacts with RELA. Subcellular Location: Cytoplasm. Nucleus. Mitochondrion matrix. Note=According to PubMed:12398897, it is cytoplasmic and nuclear. According to PubMed:14732903, it is found in the mitochondrial matrix. Tissue Specificity: Ubiquitously expressed. DISEASE: Defects in ETHE1 are a cause of ethylmalonic encephalopathy (EE) [MIM:602473]. EE is an autosomal recessive disorder characterized by neurodevelopmental delay and regression, recurrent petechiae, acrocyanosis, diarrhea, leading to death in the first decade of life. It is also associated with persistent lactic acidemia and ethylmalonic and methylsuccinic aciduria. Similarity: Belongs to the metallo-beta-lactamase superfamily. Glyoxalase II family. SWISS: O95571 Gene ID: 23474 Database links: Entrez Gene: 23474 Human Entrez Gene: 66071 Mouse Omim: 608451 Human SwissProt: O95571 Human SwissProt: Q9DCM0 Mouse Unigene: 7486 Human Unigene: 29553 Mouse Unigene: 14691 Rat |
| 1、抗体溶解方法 | |
| 2、抗体修复方式 | |
| 3、常用试剂的配制 | |
| 4、免疫组化操作步骤 | |
| 5、免疫组化问题解答 | |
| 6、Western Blotting 操作步骤 | |
| 7、Western Blotting 问题解答 | |
| 8、关于肽链的设计 | |
| 9、多肽的溶解与保存 | |
| 10、酶标抗体效价测定程序 | |
