Target intelligence / Profile preview

Electron transfer flavoprotein subunit alpha (ETFA)

Target
ETFA
Molecular classification
Enzyme (component of a multi-subunit electron transfer enzyme), Mitochondrial protein, Flavoprotein
01

Overview

Electron transfer flavoprotein subunit alpha is one subunit of the heterodimeric electron transfer flavoprotein (ETF) complex, a mitochondrial matrix enzyme that functions as a central electron acceptor for multiple flavoprotein dehydrogenases involved in fatty acid and amino acid metabolism[1][2][3][4][5]. The ETF complex consists of an alpha subunit (ETFA) and a beta subunit (ETFB), together binding the cofactor flavin adenine dinucleotide (FAD)[4][5][6]. ETF collects electrons from at least 14 mitochondrial dehydrogenases and relays these electrons to the respiratory chain via electron transfer flavoprotein-ubiquinone oxidoreductase, contributing to cellular ATP production[5]. Mutations in the ETFA gene can cause multiple acyl-CoA dehydrogenase deficiency (glutaric acidemia type II), a severe inherited disorder of metabolism that affects the breakdown of fatty acids and amino acids[6]. This subunit does *not* function as a classical therapeutic target such as a receptor or transporter, but is an important enzyme for mitochondrial metabolism and energy production[1][2][3][4][5][6].

Other names
Electron transfer flavoprotein alphaETFαETFA
02

Biological functions

Electron transportFatty acid oxidationAmino acid catabolismMitochondrial metabolism
03

Disease associations

Glutaric acidemia type II (multiple acyl-CoA dehydrogenase deficiency)Mitochondrial metabolic disorders
04

Safety considerations

Deficiency causes severe metabolic disorders (e.g., glutaric acidemia type II), often presenting with life-threatening metabolic crises in infants and children.
05

Biomarkers

Mutations in ETFA gene (for diagnosis of glutaric acidemia type II, also known as multiple acyl-CoA dehydrogenase deficiency)

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