Electron-transferring-flavoprotein dehydrogenase

Etf oxidoreductase functional domains

Electron-transferring-flavoprotein dehydrogenase (ETF dehydrogenase) is an enzyme that plays a crucial role in the electron transport chain, which is a series of reactions that generate energy in the form of adenosine triphosphate (ATP) in cells. This enzyme is involved in the oxidation of electron transfer flavoprotein (ETF), transferring electrons from ETF to the electron transport chain, specifically to coenzyme Q10 (CoQ10), which is a component of the mitochondria's inner membrane.

Function

Electron-transferring-flavoprotein dehydrogenase is part of the mitochondrial oxidative phosphorylation system. It catalyzes the transfer of electrons from reduced ETF to CoQ10, linking the oxidation of fatty acids and some amino acids to the mitochondrial respiratory chain. This process is essential for the production of ATP, which is the primary energy currency of the cell. The enzyme is encoded by the ETFDH gene in humans.

Structure

The enzyme is a flavoprotein, containing a flavin adenine dinucleotide (FAD) cofactor, and is located in the mitochondrial inner membrane. It operates in a complex with electron-transferring-flavoprotein, which is a soluble protein that shuttles electrons from various dehydrogenases to the ETF dehydrogenase.

Clinical Significance

Mutations in the ETFDH gene can lead to metabolic disorders, such as Multiple acyl-CoA dehydrogenase deficiency (MADD), also known as Glutaric aciduria type II. This condition is characterized by an inability to metabolize certain fats and proteins properly, leading to a buildup of toxic substances in the body. Symptoms can range from mild to severe and may include hypoglycemia, metabolic acidosis, and muscle weakness.

See Also

• Electron transport chain
• Mitochondrion
• Oxidative phosphorylation
• Flavoprotein
• Coenzyme Q10

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