electron transfer flavoprotein-ubiquinone oxidoreductase, mitochondrial; ETF-ubiquinone oxidoreductase; ETF-QO; electron-transferring-flavoprotein dehydrogenase; ETF dehydrogenase (ETFDH)

Function

• accepts electrons from ETF & reduces ubiquinone

reduced electron-transferring flavoprotein + ubiquinone

<-->

electron-transferring flavoprotein + ubiquinol

Cofactor:

• binds 1 4Fe-4S cluster
• FAD

Structure

• belongs to the ETF-QO/fixC family
• contains 1 4Fe-4S ferredoxin-type domain

Compartment

mitochondrial inner membrane

Pathology

• defects in ETFDH are the cause of glutaric aciduria type 2C

More general terms

• mitochondrial membrane protein
• flavoprotein
• metalloprotein
• dehydrogenase

Database

• Entrez gene: http://www.ncbi.nlm.nih.gov/sites/entrez?db=gene&cmd=Retrieve&dopt=Graphics&list_uids=2110
• Kegg: http://www.genome.jp/dbget-bin/www_bget?hsa:2110
• OMIM: https://mirror.omim.org/entry/231675
• OMIM: https://mirror.omim.org/entry/231680
• UniProt: http://www.uniprot.org/uniprot/Q16134.html