| 别名 | NADH-ubiquinone oxidoreductase 75 kDa subunit mitochondrial |
| 基因名称 | Endogenous |
| 蛋白名称 | NDUFS1 |
| 推荐应用 | |
| 反应物种 | NDUFS1 |
| 存储缓冲液 | IHC 1:200-1:1000;WB 1:2000-1:10000;IF 1:200-1:1000;ELISA 1:5000-1:20000;IP 1:50-1:200; |
| Human Gene Link | NDUFS1;NADH-ubiquinone oxidoreductase 75 kDa subunit;mitochondrial;Complex I-75kD;CI-75kD |
| Human Swissprot No. | 4719 |
| Mouse Gene ID | P28331 |
| Mouse Swissprot No. | 227197 |
| Rat Gene ID | 301458 |
| Rat Swissprot No. | Q66HF1 |
| 特异性 | Monoclonal |
| 稀释度 | Unmodified |
| 参考分子量 | 80kD |
| 预测分子量 | WB,IHC,IF,IP,ELISA |
| 运输及保存条件 | Protein A |
| 宿主 | 77kD |
| 同种型 | Rabbit |
| 背景介绍 | The protein encoded by this gene belongs to the complex I 75 kDa subunit family. Mammalian complex I is composed of 45 different subunits. It locates at the mitochondrial inner membrane. This protein has NADH dehydrogenase activity and oxidoreductase activity. It transfers electrons from NADH to the respiratory chain. The immediate electron acceptor for the enzyme is believed to be ubiquinone. This protein is the largest subunit of complex I and it is a component of the iron-sulfur (IP) fragment of the enzyme. It may form part of the active site crevice where NADH is oxidized. Mutations in this gene are associated with complex I deficiency. Several transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Jan 2011], |
| 细胞定位 | Mitochondrion inner membrane ; Peripheral membrane protein ; Matrix side . |
| 功能 | Catalytic activity:NADH + acceptor = NAD(+) + reduced acceptor.,Catalytic activity:NADH + ubiquinone = NAD(+) + ubiquinol.,cofactor:Binds 1 2Fe-2S cluster per subunit.,cofactor:Binds 2 4Fe-4S clusters per subunit.,Disease:Defects in NDUFS1 are a cause of complex I mitochondrial respiratory chain deficiency [MIM:252010]. Complex I (NADH-ubiquinone oxidoreductase), the largest complex of the mitochondrial respiratory chain, contains more than 40 subunits. It is embedded in the inner mitochondrial membrane and is partly protruding in the matrix. Complex I deficiency is the most common cause of mitochondrial disorders. It represents largely one-third of all cases of respiratory chain deficiency and is responsible for a variety of clinical symptoms, ranging from neurological disorders to cardiomyopathy, liver failure, and myopathy.,Function:Core subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (Complex I) that is believed to belong to the minimal assembly required for catalysis. Complex I functions in the transfer of electrons from NADH to the respiratory chain. The immediate electron acceptor for the enzyme is believed to be ubiquinone (By similarity). This is the largest subunit of complex I and it is a component of the iron-sulfur (IP) fragment of the enzyme. It may form part of the active site crevice where NADH is oxidized.,similarity:Belongs to the complex I 75 kDa subunit family.,similarity:Contains 1 2Fe-2S ferredoxin-type domain.,subunit:Complex I is composed of 45 different subunits., |
| 纯化 | PBS, 50% glycerol, 0.05% Proclin 300, 0.05%BSA |
| Clonality | -15°C to -25°C/1 year(Do not lower than -25°C) |
| 修饰类型 | IgG, Kappa |
| 细胞定位 | Mitochondrion inner membrane ; Peripheral membrane protein ; Matrix side . |
| 组织表达 | Brain,Cajal-Retzius cell,Colon,Fetal brain cortex,Liver, |
| 研究领域 | >>Oxidative phosphorylation;>>Metabolic pathways;>>Thermogenesis;>>Retrograde endocannabinoid signaling;>>Non-alcoholic fatty liver disease;>>Alzheimer disease;>>Parkinson disease;>>Amyotrophic lateral sclerosis;>>Huntington disease;>>Prion disease;>>Pathways of neurodegeneration - multiple diseases;>>Chemical carcinogenesis - reactive oxygen species;>>Diabetic cardiomyopathy |
周一至周日 8:00-18:00
