Literature summary extracted from

MacKenzie, E.D.; Selak, M.A.; Tennant, D.A.; Payne, L.J.; Crosby, S.; Frederiksen, C.M.; Watson, D.G.; Gottlieb, E.
Cell-permeating alpha-ketoglutarate derivatives alleviate pseudohypoxia in succinate dehydrogenase-deficient cells (2007), Mol. Cell. Biol., 27, 3282-3289.

Protein Variants

EC Number	Protein Variants	Comment	Organism
1.3.5.1	additional information	inactivation of SDHD by scrambled and SDHD-targeting small interfering RNA short hairpins, Sc and Di3, expression, succinate dehydrogenase-deficient cells show cell-permeating alpha-ketoglutarate derivatives alleviate pseudohypoxia, overview	Homo sapiens

Natural Substrates/ Products (Substrates)

EC Number	Natural Substrates	Organism	Comment (Nat. Sub.)	Natural Products	Comment (Nat. Pro.)	Rev.	Reac.
1.3.5.1	succinate + ubiquinone	Homo sapiens	the enzyme is part of the tricarboxylic acid cycle, it is also a tumor suppressor. Succinate stabilizes and activates hypoxia-inducible factor HIFalpha and reversibly and competitively inhibits HIF-prolyl hydroxylase leading to induction of hypoxia in SDH-deficient cells, 2-oxoglutarate overcomes succinate-mediated inhibition of PHD in vitro, overview	fumarate + ubiquinol	-	?

Organism

EC Number	Organism	UniProt	Comment	Textmining
1.3.5.1	Homo sapiens	-	-	-

Substrates and Products (Substrate)

EC Number	Substrates	Comment Substrates	Organism	Products	Comment (Products)	Rev.	Reac.
1.3.5.1	succinate + ubiquinone	-	Homo sapiens	fumarate + ubiquinol	-	?
1.3.5.1	succinate + ubiquinone	the enzyme is part of the tricarboxylic acid cycle, it is also a tumor suppressor. Succinate stabilizes and activates hypoxia-inducible factor HIFalpha and reversibly and competitively inhibits HIF-prolyl hydroxylase leading to induction of hypoxia in SDH-deficient cells, 2-oxoglutarate overcomes succinate-mediated inhibition of PHD in vitro, overview	Homo sapiens	fumarate + ubiquinol	-	?

Synonyms

EC Number	Synonyms	Comment	Organism
1.3.5.1	SDH	-	Homo sapiens

Cofactor

EC Number	Cofactor	Comment	Organism	Structure
1.3.5.1	ubiquinone	-	Homo sapiens

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Release 2023.1 (January 2023)