Overview
| 别名 | Superoxide dismutase [Cu-Zn]; Superoxide dismutase 1; hSod1 |
| 基因名 | SOD1 |
| UniProt ID | P00441 |
| 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 | 5W9-N1-U9 |
| 克隆性 | Monoclonal Antibody |
| 分子量 | Calculated MW: 15 kDa |
| 纯化方式 | Affinity Purified |
| 产品形式 | Liquid |
| 推荐稀释比 | 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 | Superoxide dismutase 1 (SOD1) is a critical intracellular antioxidant enzyme that exists as a 32 kDa homodimer stabilized by an intramolecular disulfide bond and a binuclear metal-binding site coordinating copper and zinc ions. Its primary function is to catalyze the dismutation of toxic superoxide radicals into hydrogen peroxide and molecular oxygen, thereby regulating basal oxidative stress. Beyond its enzymatic role, wild-type SOD1 acts as a nuclear transcription factor for oxidative resistance genes. Clinically, over 180 mutations in the SOD1 gene are linked to familial amyotrophic lateral sclerosis (fALS), accounting for approximately 20% of cases. Disease-associated mutants undergo structural destabilization and misfolding, leading to the formation of toxic aggregates. These misfolded species accumulate on mitochondrial membranes and interact with VDAC1 and Bcl-2, ultimately triggering mitochondrial dysfunction and motor neuron death. |
检测原理