Electron-transferring flavoprotein

Error creating thumbnail:

PDB 1efp EBI

Electron-transferring flavoprotein

Electron-transferring flavoprotein (ETF) is a key component in the process of electron transfer within cells. It plays a crucial role in various metabolic pathways, particularly in the oxidation of fatty acids and amino acids. ETF is a heterodimeric protein complex consisting of two subunits, α and β, each containing a flavin adenine dinucleotide (FAD) cofactor.

Structure

The α subunit of ETF contains the FAD cofactor, which is involved in accepting electrons from various dehydrogenase enzymes. The β subunit, on the other hand, serves as a bridge between the α subunit and other enzymes involved in the electron transfer process.

Function

ETF functions as an electron carrier, shuttling electrons between different enzymes in metabolic pathways. It plays a critical role in the electron transport chain, facilitating the transfer of electrons from fatty acid oxidation and amino acid metabolism to the respiratory chain for ATP production.

Clinical Significance

Mutations in the genes encoding ETF subunits can lead to metabolic disorders such as glutaric acidemia type II, a rare inherited disorder characterized by the accumulation of toxic metabolites. Understanding the structure and function of ETF is essential for diagnosing and treating these metabolic disorders.

See also

• Electron transport chain
• Flavin adenine dinucleotide
• Metabolic pathway