|Other Names||NADH dehydrogenase [ubiquinone] iron-sulfur protein 8, mitochondrial, Complex I-23kD, CI-23kD, NADH-ubiquinone oxidoreductase 23 kDa subunit, TYKY subunit, NDUFS8|
|Format||Synthetic peptide was lyophilized with 100% acetonitrile and is supplied as a powder. Reconstitute with 0.1 ml DI water for a final concentration of 1 mg/ml.|
|Storage||Maintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C.|
|Precautions||This product is for research use only. Not for use in diagnostic or therapeutic procedures.|
|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). May donate electrons to ubiquinone.|
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This gene encodes a subunit of mitochondrialNADH:ubiquinone oxidoreductase, or Complex I, a multimeric enzymeof the respiratory chain responsible for NADH oxidation, ubiquinonereduction, and the ejection of protons from mitochondria. Theencoded protein is involved in the binding of two of the six toeight iron-sulfur clusters of Complex I and, as such, is requiredin the electron transfer process. Mutations in this gene have beenassociated with Leigh syndrome.
Rose, J.E., et al. Mol. Med. 16 (7-8), 247-253 (2010) :Bourges, I., et al. Biochem. J. 383 (PT 3), 491-499 (2004) :Procaccio, V., et al. Neurology 62(10):1899-1901(2004)Ugalde, C., et al. Hum. Mol. Genet. 13(6):659-667(2004)Murray, J., et al. J. Biol. Chem. 278(39):37223-37230(2003)
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