succinate dehydrogenase flavoprotein subunit (SDHA, SDH2, SDHF)

Function

• component of succinate dehydrogenase (complex 2)
• nuclear-encoded mitochondrial flavoprotein
• carbohydrate metabolism; tricarboxylic acid cycle

succinate + ubiquinone <--> fumarate + ubiquinol

Cofactor: FAD

Structure

• belongs to the FAD-dependent oxidoreductase 2 family FRD/SDH subfamily

Compartment

mitochondrial inner membrane

Pathology

• defects in SDHA are a cause of succinate CoQ reductase deficiency
• defects in SDHA are a cause of Leigh syndrome in this form of Leigh syndrome complex 2 no longer reduces ubiquinone

More general terms

• flavoprotein
• protein subunit
• mitochondrial membrane protein
• phosphoprotein

Component of

• succinate dehydrogenase (mitochondrial complex 2)

Database

• UniProt: http://www.uniprot.org/uniprot/P31040.html
• Kegg: http://www.genome.jp/dbget-bin/www_bget?hsa:6389
• OMIM: https://mirror.omim.org/entry/252011
• OMIM: https://mirror.omim.org/entry/256000
• OMIM: https://mirror.omim.org/entry/600857