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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 ultimately 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 one of the subunits can lead to various myopathies and neuropathies. As a subunit of mitochondrial complex I, NDUFV3 (NADH dehydrogenase [ubiquinone] flavoprotein 3), also designated NADH-ubiquinone oxidoreductase 9 kDa subunit or CI-9kD, is a 108 amino acid protein that is believed to not be involved in catalysis.
1500032D16Rik; CI-10k; CI-9KD; complex I 10kDa subunit; complex I, mitochondrial respiratory chain, 10-kD subunit; complex I-9kD; Mipp65; mitochondrial NADH oxidoreductase-like protein; NADH dehydrogenase (ubiquinone) flavoprotein 3; NADH dehydrogenase (ubiquinone) flavoprotein 3, 10kDa; NADH dehydrogenase (ubiquinone) flavoprotein 3-like; NADH dehydrogenase [ubiquinone] flavoprotein 3, mitochondrial; NADH:ubiquinone oxidoreductase subunit V3; NADH-ubiquinone oxidoreductase 9 kD subunit; NADH-ubiquinone oxidoreductase 9 kDa subunit; NADH-ubiquinone oxidoreductase flavoprotein 3; NADH-ubiquinone oxidoreductase flavoprotein 3, 10kD; NDUFV3; Ndufv3l; renal carcinoma antigen NY-REN-4
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