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Electron-transferring-flavoprotein dehydrogenase in the inner mitochondrial membrane accepts electrons from electron-transfer flavoprotein which is located in the mitochondrial matrix and reduces ubiquinone in the mitochondrial membrane. The protein is synthesized as a 67-kDa precursor which is targeted to mitochondria and processed in a single step to a 64-kDa mature form located in the mitochondrial membrane. Deficiency in electron-transferring-flavoprotein dehydrogenase have been demonstrated in some patients with type II glutaricacidemia.
For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.
Protein Aliases: Electron transfer flavoprotein-ubiquinone oxidoreductase, mitochondrial; Electron-transferring-flavoprotein dehydrogenase; ETF dehydrogenase; ETF-QO; ETF-ubiquinone oxidoreductase
Gene Aliases: ETFDH; ETFQO; MADD
UniProt ID: (Human) Q16134
Entrez Gene ID: (Human) 2110
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