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BN41037R-100ul
100ul
¥2360.00
交叉反应：Human,Mouse(predicted:Rat,Dog,Pig,Cow,Rabbit,Sheep) 推荐应用：WB,IHC-P,IHC-F,IF,ELISA
BN41037R-200ul
200ul
¥3490.00
交叉反应：Human,Mouse(predicted:Rat,Dog,Pig,Cow,Rabbit,Sheep) 推荐应用：WB,IHC-P,IHC-F,IF,ELISA
产品描述
| 英文名称 | HADHB |
| 中文名称 | 羟辅酶A脱氢酶β抗体 |
| 别 名 | 2 enoyl Coenzyme A (CoA) hydratase beta subunit; 3 ketoacyl Coenzyme A (CoA) thiolase of mitochondrial trifunctional protein beta subunit; 3 ketoacyl Coenzyme A thiolase; 3 ketoacyl Coenzyme A thiolase of mitochondrial trifunctional protein beta subunit; Acetyl CoA acyltransferase; Beta ketothiolase; ECHB; HADH; Hydroxyacyl Coenzyme A (CoA) dehydrogenase beta subunit; Hydroxyacyl Coenzyme A dehydrogenase; Hydroxyacyl Coenzyme A dehydrogenase beta subunit; Hydroxyacyl Coenzyme A dehydrogenase/3 ketoacyl Coenzyme A thiolase/enoyl Coenzyme A hydratase (trifunctional protein) beta subunit; Mitochondrial trifunctional enzyme beta subunit; Mitochondrial trifunctional protein beta subunit; MTPB; TP beta. TPbeta; Trifunctional enzyme subunit beta; Trifunctional enzyme subunit beta mitochondrial; Trifunctional protein; ECHB_HUMAN. |
| 研究领域 | 肿瘤 细胞生物 免疫学 转录调节因子 激酶和磷酸酶 |
| 抗体来源 | Rabbit |
| 克隆类型 | Polyclonal |
| 交叉反应 | Human, Mouse, (predicted: Rat, Dog, Pig, Cow, Rabbit, Sheep, ) |
| 产品应用 | WB=1:500-2000 ELISA=1:5000-10000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user. |
| 分 子 量 | 47kDa |
| 细胞定位 | 细胞浆 细胞膜 |
| 性 状 | Liquid |
| 浓 度 | 1mg/ml |
| 免 疫 原 | KLH conjugated synthetic peptide derived from human HADHB:231-330/474 |
| 亚 型 | IgG |
| 纯化方法 | affinity purified by Protein A |
| 储 存 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. |
| PubMed | PubMed |
| 产品介绍 | The HADHB gene encodes the beta subunit of the mitochondrial trifunctional protein, which catalyzes the last three steps of mitochondrial beta-oxidation of long chain fatty acids. The mitochondrial membrane-bound heterocomplex is composed of four alpha and four beta subunits, with the beta subunit catalyzing the 3-ketoacyl-CoA thiolase activity. Mutations in this gene result in trifunctional protein deficiency. The encoded protein can also bind RNA and decreases the stability of some mRNAs. The genes of the alpha and beta subunits of the mitochondrial trifunctional protein are located adjacent to each other in the human genome in a head-to-head orientation. Alternatively spliced transcript variants have been found; however, their full-length nature is not known. Subunit: Octamer of 4 alpha (HADHA) and 4 beta (HADHB) subunits. Interacts with RSAD2/viperin. Subcellular Location: Mitochondrion. Mitochondrion inner membrane. Mitochondrion outer membrane. Endoplasmic reticulum. DISEASE: Defects in HADHB are a cause of trifunctional protein deficiency (TFP deficiency) [MIM:609015]. The clinical manifestations are very variable and include hypoglycemia, cardiomyopathy and sudden death. Phenotypes with mainly hepatic and neuromyopathic involvement can also be distinguished. Biochemically, TFP deficiency is defined by the loss of all three enzyme activities of the TFP complex. Similarity: Belongs to the thiolase family. SWISS: P55084 Gene ID: 3032 Database links: Entrez Gene: 3032 Human Omim: 143450 Human SwissProt: P55084 Human Unigene: 515848 Human Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. |
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