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MitoProteome Human Mitochondrial Protein Database
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Related Proteins
MT001205
UniProt Annotations
Entry Information
Gene Name
ACN9 homolog (S. cerevisiae)
Protein Entry
SDHF3_HUMAN
UniProt ID
Q9NRP4
Species
Human
Comments
Comment type
Description
Function
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. Promotes maturation of the iron-sulfur protein subunit SDHB of the SDH catalytic dimer, protecting it from the deleterious effects of oxidants. May act together with SDHAF1. {ECO:0000250|UniProtKB:Q04401, ECO:0000250|UniProtKB:Q8SZ16}.
Similarity
Belongs to the complex I LYR family. SDHAF3 subfamily. {ECO:0000305}.
Subcellular Location
Mitochondrion matrix {ECO:0000250|UniProtKB:Q04401}.
Subunit
Interacts with SDHB within an SDHA-SDHB subcomplex. {ECO:0000250|UniProtKB:Q04401}.
MitoProteome Human Mitochondrial Protein Database
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