The electron transfer flavoprotein serves as a specific electron acceptor for several dehydrogenases, including five acyl- CoA dehydrogenases, glutaryl-CoA and sarcosine dehydrogenase. It transfers the electrons to the main mitochondrial respiratory chain via ETF-ubiquinone oxidoreductase (ETF dehydrogenase). Defects in ETFB are the cause of glutaric aciduria type 2B (GA2B). GA2B is an autosomal recessively inherited disorder of fatty acid, amino acid, and choline metabolism. It is characterized by multiple acyl-CoA dehydrogenase deficiencies resulting in large excretion not only of glutaric acid, but also of lactic, ethylmalonic, butyric, isobutyric, 2-methyl-butyric, and isovaleric acids. Belongs to the ETF beta-subunit/FixA family. 2 isoforms of the human protein are produced by alternative splicing. Note: This description may include information from UniProtKB.
Alt. Names/Synonyms: Beta-ETF; electron transfer flavoprotein beta subunit; electron transfer flavoprotein beta-subunit; Electron transfer flavoprotein subunit beta; electron transfer flavoprotein, beta polypeptide; electron-transfer-flavoprotein, beta polypeptide; electron-transferring-flavoprotein, beta polypeptide; ETFB; FP585; MADD
SS: The number of records in which this modification site was determined using site-specific methods. SS methods include amino acid sequencing, site-directed mutagenesis, modification site-specific antibodies, specific MS strategies, etc.