Electron transfer flavoprotein subunit alpha, mitochondrial

Definition:

Heterodimeric electron transfer flavoprotein that accepts electrons from several mitochondrial dehydrogenases, including acyl-CoA dehydrogenases, glutaryl-CoA and sarcosine dehydrogenase. It transfers the electrons to the main mitochondrial respiratory chain via ETF-ubiquinone oxidoreductase (ETF dehydrogenase). Required for normal mitochondrial fatty acid oxidation and normal amino acid metabolism.

References:

[1]. P Bross, et al. A polymorphic variant in the human electron transfer flavoprotein alpha-chain (alpha-T171) displays decreased thermal stability and is overrepresented in very-long-chain acyl-CoA dehydrogenase-deficient patients with mild childhood presentation. Mol Genet Metab. 1999 Jun;67(2):138-47. [Content Brief]

[2]. Y Indo, et al. Molecular characterization of variant alpha-subunit of electron transfer flavoprotein in three patients with glutaric acidemia type II--and identification of glycine substitution for valine-157 in the sequence of the precursor, producing an unstable mature protein in a patient. Am J Hum Genet. 1991 Sep;49(3):575-80. [Content Brief]

[3]. Brendan J Floyd, et al. Mitochondrial Protein Interaction Mapping Identifies Regulators of Respiratory Chain Function. Mol Cell. 2016 Aug 18;63(4):621-632. [Content Brief]

[4]. D Salazar, et al. Expression and characterization of two pathogenic mutations in human electron transfer flavoprotein. J Biol Chem. 1997 Oct 17;272(42):26425-33. [Content Brief]

[5]. Helen S Toogood, et al. Dynamics driving function: new insights from electron transferring flavoproteins and partner complexes. FEBS J. 2007 Nov;274(21):5481-504. [Content Brief]

[6]. Rikke K J Olsen, et al. Clear relationship between ETF/ETFDH genotype and phenotype in patients with multiple acyl-CoA dehydrogenation deficiency. Hum Mutat. 2003 Jul;22(1):12-23. [Content Brief]

[7]. Helen S Toogood, et al. Stabilization of non-productive conformations underpins rapid electron transfer to electron-transferring flavoprotein. J Biol Chem. 2005 Aug 26;280(34):30361-6. [Content Brief]

[8]. Helen S Toogood, et al. Extensive domain motion and electron transfer in the human electron transferring flavoprotein.medium chain Acyl-CoA dehydrogenase complex. J Biol Chem. 2004 Jul 30;279(31):32904-12. [Content Brief]

[9]. E Freneaux, et al. Glutaric acidemia type II. Heterogeneity in beta-oxidation flux, polypeptide synthesis, and complementary DNA mutations in the alpha subunit of electron transfer flavoprotein in eight patients. J Clin Invest. 1992 Nov;90(5):1679-86. [Content Brief]

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