Overview
| 别名 | Trifunctional enzyme subunit beta; mitochondrial; TP-beta) [Includes: 3-ketoacyl-CoA thiolase; Acetyl-CoA acyltransferase; Beta-ketothiolase] |
| 基因名 | HADHB |
| UniProt ID | P55084 |
| 反应种属 | Human |
| 应用 | WB,IHC-P |
| 宿主 | Mouse |
| 偶联物 | Unconjugated |
| 修饰 | Unmodified |
| 亚型 | IgG2a |
| 克隆号 | 8M8-Y7-I2 |
| 克隆性 | Monoclonal Antibody |
| 分子量 | Calculated MW: 51 kDa |
| 纯化方式 | Affinity Purified |
| 产品形式 | Liquid |
| 推荐稀释比 | WB-1:1000; IHC-1:100-1:200 |
| 存储缓冲液 | Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.09% sodium azide |
| 保存温度 | Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles. |
| 背景信息 | Hydroxyacyl-CoA dehydrogenase trifunctional multienzyme complex subunit beta (HADHB) is a subunit of the mitochondrial trifunctional enzyme (MTP) located in the inner mitochondrial membrane. It forms a heterooctamer with HADHA to catalyze the final three steps of long-chain fatty acid beta-oxidation, specifically providing long-chain 3-ketoacyl-CoA thiolase activity. This process is vital for energy production in the heart, liver, and skeletal muscle. Clinically, biallelic mutations in HADHB result in mitochondrial trifunctional protein deficiency, characterized by hypoketotic hypoglycemia, cardiomyopathy, and rhabdomyolysis. Furthermore, heterozygous maternal carriers of a fetus with HADHB mutations are at increased risk for developing acute fatty liver of pregnancy (AFLP) and HELLP syndrome. |
检测原理