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Electron-transferring-flavoprotein dehydrogenase
Ribbon diagram of electron-transferring-flavoprotein dehydrogenase in complex with ubiquinone (bottom).
Identifiers
Symbol ETFD
Alt. symbols ETF-QO
Entrez 2110
HUGO 3483
OMIM 231675
PDB 2GMH
RefSeq NM_004453
UniProt Q16134
Other data
EC number 1.5.5.1
Locus Chr. 4 q4q32.1

Electron-transferring-flavoprotein dehydrogenase (ETF dehydrogenase or electron transfer flavoprotein-ubiquinone oxidoreductase, EC 1.5.5.1) is an enzyme that transfers electrons from electron-transferring flavoprotein in the mitochondrial matrix, to the ubiquinone pool in the inner mitochondrial membrane.[1] It is part of the electron transport chain. The enzyme is found in both prokaryotes and eukaryotes and contains a flavin and FE-S cluster.[2] Deficiency in EFT dehydrogenase causes the human genetic disease multiple acyl-CoA dehydrogenase deficiency.[3]

Reduced electron-transferring flavoprotein + ubiquinone <=> electron-transferring flavoprotein + ubiquinol

[edit] See also

[edit] References

  1. ^ Ramsay RR, Steenkamp DJ, Husain M (1987). "Reactions of electron-transfer flavoprotein and electron-transfer flavoprotein: ubiquinone oxidoreductase". Biochem. J. 241 (3): 883–92. PMID 3593226. 
  2. ^ Zhang J, Frerman FE, Kim JJ (2006). "Structure of electron transfer flavoprotein-ubiquinone oxidoreductase and electron transfer to the mitochondrial ubiquinone pool". Proc. Natl. Acad. Sci. U.S.A. 103 (44): 16212–7. doi:10.1073/pnas.0604567103. PMID 17050691. 
  3. ^ Vianey-Liaud C, Divry P, Gregersen N, Mathieu M (1987). "The inborn errors of mitochondrial fatty acid oxidation". J. Inherit. Metab. Dis. 10 Suppl 1: 159–200. doi:10.1007/BF01812855. PMID 3119938. 

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