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Plays an essential role in the assembly of succinate dehydrogenase (SDH), an enzyme complex (also referred to as respiratory complex II) that is a component of both the tricarboxylic acid (TCA) cycle and the mitochondrial electron transport chain, and which couples the oxidation of succinate to fumarate with the reduction of ubiquinone (coenzyme Q) to ubiquinol (PubMed:24954416). Binds to the flavoprotein subunit Sdha in its FAD-bound form, blocking the generation of excess reactive oxigen species (ROS) and facilitating its assembly with the iron-sulfur protein subunit Sdhb into the SDH catalytic dimer.
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Protein Aliases: hypothetical protein LOC685888; SDH assembly factor 4; Succinate dehydrogenase assembly factor 4, mitochondrial; UPF0369 protein C6orf57; UPF0369 protein C6orf57 homolog
Gene Aliases: 1110058L19Rik; 1700001E18Rik; C6orf57; Sdh8; SDHAF4
UniProt ID: (Human) Q5VUM1, (Mouse) Q8BTE0
Entrez Gene ID: (Rat) 685888, (Human) 135154, (Mouse) 68002
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