Overview
| 别名 | Iron-sulfur cluster assembly enzyme ISCU; NifU-like N-terminal domain-containing protein; NifU-like protein |
| 基因名 | ISCU |
| UniProt ID | Q9H1K1 |
| 反应种属 | Human |
| 应用 | IHC-P |
| 宿主 | Mouse |
| 偶联物 | Unconjugated |
| 修饰 | Unmodified |
| 亚型 | IgG2b |
| 克隆号 | 7S7-L8-C8 |
| 克隆性 | Monoclonal Antibody |
| 分子量 | Calculated MW: 17 kDa |
| 纯化方式 | Affinity Purified |
| 产品形式 | Liquid |
| 推荐稀释比 | IHC-1:200-1:250 |
| 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 | Iron-sulfur cluster assembly enzyme (ISCU) is a mitochondrial scaffold protein belonging to the NifU family, serving as the central platform for the synthesis and maturation of iron-sulfur (Fe-S) clusters. These clusters are essential cofactors for enzymes involved in energy metabolism, iron homeostasis, and oxidative stress responses. ISCU facilitates the transient assembly of [2Fe-2S] and [4Fe-4S] clusters through interactions with the NFS1-LYRM4/ISD11 complex and molecular chaperones HscA and HscB, eventually transferring them to recipient proteins such as GLRX5. The protein exists in mitochondrial and nuclear/cytoplasmic isoforms, exhibiting dynamic conformational changes stabilized by zinc ions. Clinically, mutations in the ISCU gene are well-established causes of hereditary myopathy with lactic acidosis, characterized by deficiencies in Fe-S-dependent enzymes like succinate dehydrogenase and aconitase, leading to exercise intolerance and muscle weakness. |
检测原理