Electron-transferring-flavoprotein dehydrogenase

Protein
Name=Electron-transferring-flavoprotein dehydrogenase


caption=Ribbon diagram of electron-transferring-flavoprotein dehydrogenase in complex with ubiquinone (bottom).
Symbol=ETFD
AltSymbols=ETF-QO
HGNCid=3483
Chromosome=4
Arm=q
Band=
LocusSupplementaryData=4q32.1
ECnumber=1.5.5.1
OMIM=231675
EntrezGene=2110
RefSeq=NM_004453
UniProt=Q16134
PDB=2GMH

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. [cite journal |author=Ramsay RR, Steenkamp DJ, Husain M |title=Reactions of electron-transfer flavoprotein and electron-transfer flavoprotein: ubiquinone oxidoreductase |journal=Biochem. J. |volume=241 |issue=3 |pages=883–92 |year=1987 |pmid=3593226 |doi=] 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. [cite journal |author=Zhang J, Frerman FE, Kim JJ |title=Structure of electron transfer flavoprotein-ubiquinone oxidoreductase and electron transfer to the mitochondrial ubiquinone pool |journal=Proc. Natl. Acad. Sci. U.S.A. |volume=103 |issue=44 |pages=16212–7 |year=2006 |pmid=17050691 |doi=10.1073/pnas.0604567103] Deficiency in EFT dehydrogenase causes the human genetic disease multiple acyl-CoA dehydrogenase deficiency. [cite journal |author=Vianey-Liaud C, Divry P, Gregersen N, Mathieu M |title=The inborn errors of mitochondrial fatty acid oxidation |journal=J. Inherit. Metab. Dis. |volume=10 Suppl 1 |issue= |pages=159–200 |year=1987 |pmid=3119938 |doi=10.1007/BF01812855]

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

ee also

*Oxidative phosphorylation
*Electron transport chain
*Microbial metabolism
*Metabolism

References

External links

* [http://us.expasy.org/enzyme/1.5.5.1 ENZYME entry on EC 1.5.5.1]
* [http://www.brenda-enzymes.org/php/result_flat.php4?ecno=1.5.5.1 BRENDA entry on EC 1.5.5.1]
* [http://www.ncbi.nlm.nih.gov/entrez/dispomim.cgi?id=231680 OMIM Multiple acyl-CoA dehydrogenase deficiency]