| 基因名称 | NDUFS2 |
| 蛋白名称 | NADH dehydrogenase [ubiquinone] iron-sulfur protein 2, mitochondrial (Complex I-49kD) (CI-49kD) (NADH-ubiquinone oxidoreductase 49 kDa subunit) |
| 推荐应用 | |
| 反应物种 | Human,Mouse,Rat |
| 存储缓冲液 | PBS, 50% glycerol, 0.05% Proclin 300, 0.05%BSA |
| Human Swissprot No. | O75306 |
| Mouse Swissprot No. | Q91WD5 |
| Rat Swissprot No. | Q641Y2 |
| 特异性 | Endogenous |
| 稀释度 | IHC-P 1:200-1:1000;WB 1:2000-1:10000;IF-P/IF-F/IF-ICC 1:200-1:1000;ELISA 1:5000-1:20000;IP 1:50-1:200; |
| 参考分子量 | 49kDa |
| 预测分子量 | 53kDa |
| 运输及保存条件 | Biological ice bag transportation. Store at -20°C for at least 12 months(Do not lower than -25°C). Avoid freeze/thaw cycles |
| 宿主 | Rabbit |
| 同种型 | IgG,Kappa |
| 背景介绍 | The protein encoded by this gene is a core subunit of the mitochondrial membrane respiratory chain NADH dehydrogenase (complex I). Mammalian mitochondrial complex I is composed of at least 43 different subunits, 7 of which are encoded by the mitochondrial genome, and the rest are the products of nuclear genes. The iron-sulfur protein fraction of complex I is made up of 7 subunits, including this gene product. Complex I catalyzes the NADH oxidation with concomitant ubiquinone reduction and proton ejection out of the mitochondria. Mutations in this gene are associated with mitochondrial complex I deficiency. Alternatively spliced transcript variants encoding different isoforms have been found for this gene.[provided by RefSeq, Oct 2009], |
| 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 4Fe-4S cluster.,Disease:Defects in NDUFS2 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.,similarity:Belongs to the complex I 49 kDa subunit family.,subunit:Complex I is composed of 45 different subunits. Component of the iron-sulfur (IP) fragment of the enzyme., |
| Clonality | Monoclonal |
| 细胞定位 | Mitochondrion inner membrane ; Peripheral membrane protein ; Matrix side . |
周一至周日 8:00-18:00
