Anti-NDUFS7抗體,線(xiàn)粒體復(fù)合物NDUFS7蛋白抗體-技術(shù)說(shuō)明產(chǎn)品介紹:Located in the mitochondrial inner membrane, mitochondrial complex I is the first and largest enzyme in the electron transport chain of oxidative phosphorylation. By oxidizing NADH that is produced in the Krebs cycle, this complex utilizes the two electrons to reduce ubiquinone to ubiquinol, thereby initiating the passage of electrons to successive complexes and ultimay leading to the reduction of oxygen to water. Mitochondrial complex I consists of over 40 subunits and is of considerable clinical interest since defects in any of the subunits can lead to various myopathies and neuropathies. As a subunit of mitochondrial complex I, NDUFS7 (NADH dehydrogenase [ubiquinone] iron-sulfur protein 7), also designated NADH-ubiquinone oxidoreductase 20 kDa subunit, is a 213 amino acid protein that is suggested to be required for catalytic activity. Defects in the gene encoding NDUFS7 are the cause of Leigh syndrome, a severe neurological disorder that is characterized by bilaterally symmetrical necrotic lesions in subcortical brain regions.
保存條件: Store at -20℃ for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20℃. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody, the antibody is stable for at least six weeks at 2-4 ℃