Overview
| 别名 | Hydroxyacyl-coenzyme A dehydrogenase; mitochondrial; HCDH; Medium and short-chain L-3-hydroxyacyl-coenzyme A dehydrogenase; Short-chain 3-hydroxyacyl-CoA dehydrogenase |
| 基因名 | HADH |
| UniProt ID | Q16836 |
| 反应种属 | Human |
| 应用 | WB,IHC-P |
| 宿主 | Mouse |
| 偶联物 | Unconjugated |
| 修饰 | Unmodified |
| 亚型 | IgG2b |
| 克隆号 | 6I8-G2-D5 |
| 克隆性 | Monoclonal Antibody |
| 分子量 | Calculated MW: 34 kDa |
| 纯化方式 | Affinity Purified |
| 产品形式 | Liquid |
| 推荐稀释比 | WB-1:1000; IHC-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 (HADH), also known as 3‑hydroxyacyl‑CoA dehydrogenase or short‑chain L‑3‑hydroxyacyl‑CoA dehydrogenase (SCHAD), is a mitochondrial matrix enzyme encoded by the HADH gene on chromosome 4q22‑q26, producing a homodimeric 34.3 kDa protein of 314 amino acids. It catalyzes the third step of fatty acid β‑oxidation, oxidizing straight‑chain 3‑hydroxyacyl‑CoAs to 3‑ketoacyl‑CoAs with NAD⁺ as cofactor, with highest activity toward medium‑chain substrates. HADH shows oxidoreductase and NAD‑binding activity and contributes to energy production in high‑demand tissues such as heart, liver, kidney, skeletal muscle, and pancreas. Beyond fatty acid oxidation, it modulates insulin secretion by inhibiting glutamate dehydrogenase (GLUD1) and participates in spermatogenesis and tumor‑suppressive pathways in several cancers. Biallelic HADH mutations cause a rare diazoxide‑sensitive congenital hyperinsulinemic hypoglycemia (HADH deficiency), typically presenting in infancy with recurrent hypoglycemia and seizures, and showing marked protein sensitivity. |
检测原理