Electron-transferring-flavoprotein dehydrogenase

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.

Multiple hydrogen bonds and a positive helix dipole modulate the redox potential of FAD and can possibly stabilize the anionic semiquinone intermediate.

[9] Complications can involve acidosis or hypoglycemia, with other symptoms such as general weakness, liver enlargement, increased heart failure, and carnitine deficiency.

More severe cases involve congenital defects and full metabolic crisis.

Most affected patients are the result of single point mutations around the FAD ubiquinone interface.

ETF-QO functional domains
ETF-QO Functional Domains