Electron-transferring-flavoprotein dehydrogenase
| 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 |
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Overview
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
See also
References
- ↑ 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.
- ↑ 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.
- ↑ 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. PMID 3119938.