Overview
| 别名 | Hydroxyacyl-thioester dehydratase type 2; mitochondrial; HsHTD2; 3-hydroxyacyl-[acyl-carrier-protein] dehydratase |
| 基因名 | HTD2 |
| UniProt ID | P86397 |
| 反应种属 | Human |
| 应用 | IHC-P |
| 宿主 | Mouse |
| 偶联物 | Unconjugated |
| 修饰 | Unmodified |
| 亚型 | IgG1 |
| 克隆号 | 8H4-Y7-C4 |
| 克隆性 | Monoclonal Antibody |
| 分子量 | Calculated MW: 18 kDa |
| 纯化方式 | Affinity Purified |
| 产品形式 | Liquid |
| 推荐稀释比 | 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-thioester dehydratase type 2 (HTD2) is a mitochondrial protein that plays an essential role in fatty acid biosynthesis and lipid metabolism. It functions as a 3-hydroxyacyl-thioester dehydratase, catalyzing the dehydration of (3R)-hydroxyacyl-[acyl-carrier-protein] intermediates within the mitochondrial fatty acid synthesis pathway. This enzymatic activity is critical for maintaining mitochondrial function and overall cellular energy balance. Mutations in the HTD2 gene are associated with severe clinical conditions, including anauxetic dysplasia 2, a skeletal disorder characterized by extreme growth restriction. Additionally, HTD2 dysfunction is linked to childhood-onset dystonia accompanied by optic atrophy and basal ganglia abnormalities, highlighting the protein's vital importance in neurological health and skeletal development. |
检测原理