Overview
| 别名 | Peroxisomal multifunctional enzyme type 2; MFE-2; 17-beta-hydroxysteroid dehydrogenase 4; 17-beta-HSD 4; D-bifunctional protein; DBP; Multifunctional protein 2; MFP-2; Short chain dehydrogenase/reductase family 8C member 1) [Cleaved into:; 3R)-hydroxyacyl-CoA dehydrogenase; Enoyl-CoA hydratase 2; 3-alpha; 7-alpha; 12-alpha-trihydroxy-5-beta-cholest-24-enoyl-CoA hydratase] |
| 基因名 | HSD17B4 |
| UniProt ID | P51659 |
| 反应种属 | Human |
| 应用 | WB |
| 宿主 | Mouse |
| 偶联物 | Unconjugated |
| 修饰 | Unmodified |
| 亚型 | IgG2b |
| 克隆号 | 7A6-C9-B1 |
| 克隆性 | Monoclonal Antibody |
| 分子量 | Calculated MW: 79 kDa |
| 纯化方式 | Affinity Purified |
| 产品形式 | Liquid |
| 推荐稀释比 | WB-1:1000 |
| 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 | Hydroxysteroid 17-beta dehydrogenase 4 (HSD17B4), also known as D-bifunctional protein, is a peroxisomal multifunctional enzyme essential for the beta-oxidation of fatty acids. It contains distinct hydratase and dehydrogenase domains that catalyze two consecutive steps in the breakdown of very long-chain fatty acids, branched-chain fatty acids, and bile acid intermediates. Specifically, it hydrates trans-2-enoyl-CoA to (3R)-3-hydroxyacyl-CoA and subsequently dehydrogenates it to 3-ketoacyl-CoA. Pathogenic variants in HSD17B4 cause autosomal recessive D-bifunctional protein deficiency, a severe peroxisomal disorder. This condition is characterized by neonatal-onset hypotonia, seizures, and progressive leukodystrophy resulting from the toxic accumulation of very long-chain fatty acids, underscoring the enzyme's critical role in lipid metabolism and neurological health. |
检测原理