Literature summary extracted from

Gibson, G.E.; Blass, J.P.; Beal, M.F.; Bunik, V.
The alpha-ketoglutarate-dehydrogenase complex: a mediator between mitochondria and oxidative stress in neurodegeneration (2005), Mol. Neurobiol., 31, 43-63.

Activating Compound

EC Number	Activating Compound	Comment	Organism	Structure
1.2.1.105	thiamine	can reverse the loss in KGDHC in Alzheimer's disease patients	Homo sapiens
1.2.1.105	Trolox	can dramatically increase KGDHC in cultured cells	Homo sapiens

EC Number	Application	Comment	Organism
1.2.1.105	medicine	KGDHC is diminished in brains from patients with Alzheimers's disease, Parkinson's disease, Huntington's disease, Wernicke-Korskoff disease, and progressive supranuclear palsy, not diminished in brains of patients that died with schizophrenia, reduced KDGDHC may be a better indicator of the primary reactive oxygen species action than commonly used measures of oxidative stress	Homo sapiens
1.2.1.105	medicine	loss of KGDHC activity resulting from the lack of thiamine diphosphate coenzyme may lead to oxidative stress and to neuronal death both directly and by predisposing to other insults	Rattus norvegicus
1.2.1.105	additional information	alpha-ketoglutarate-involving reactions belong to the backbone of high-flux reactions, which is rather conserved in evolution	Escherichia coli

EC Number	Protein Variants	Comment	Organism
1.2.1.105	additional information	patients bearing the APP670/671 mutation show declined KGDHC activity	Homo sapiens
1.2.1.105	additional information	thiamine diphosphate deficiency diminishes KGDHC activity, knockouts of eNOS or ICAM-1 diminish oxidative stress and reduce neuronal death in thiamine-deficient mice, knockouts of E3 have diminished KGDHC in brain and liver	Mus musculus

EC Number	Inhibitors	Comment	Organism
1.2.1.105	(R)-2-amino-3-((1,1,2,2-tetrafluoroethyl)thio)propanoic acid	inactivates	Homo sapiens
1.2.1.105	1-methyl-4-phenylpyridine	i.e. MPP+	Homo sapiens
1.2.1.105	3-nitropropionic acid	-	Homo sapiens
1.2.1.105	3-NP	-	Mus musculus
1.2.1.105	amyloid-beta peptide	-	Homo sapiens
1.2.1.105	H2O2	addition outside of cells reduces KGDHC activity in proportion to the increase in reactive oxygen species	Homo sapiens
1.2.1.105	H2O2	oxidative damage to KGDHC	Saccharomyces cerevisiae
1.2.1.105	HNE	-	Homo sapiens
1.2.1.105	isoquinoline derivative	-	Homo sapiens
1.2.1.105	KMV	severely inhibits KGDHC activity	Mus musculus
1.2.1.105	KMV	inhibits KGDHC in PC-12 cells and does not alter mitochondrial membrane potential, but is associated with the release of cytochrome-c from mitochondria into the cytosol, reduction in basal cytosolic Ca2+, and diminishing endoplasmic reticulum calcium stores	Rattus norvegicus
1.2.1.105	NADH	allosteric inhibitor, inhibition relieved by micromolar Ca2+ and ADP	Bos taurus
1.2.1.105	reactive oxygen species	-	Mesocricetus auratus
1.2.1.105	reactive oxygen species	-	Mus musculus
1.2.1.105	tryptamine-4,5-dione	strongly inhibits	Rattus norvegicus

EC Number	Localization	Comment	Organism	GeneOntology No.	Textmining
1.2.1.105	mitochondrion	-	Homo sapiens	5739	-
1.2.1.105	mitochondrion	-	Rattus norvegicus	5739	-
1.2.1.105	mitochondrion	-	Sus scrofa	5739	-

EC Number	Metals/Ions	Comment	Organism
1.2.1.105	ADP	activates through allosteric binding	Bos taurus
1.2.1.105	Ca2+	dependent on	Sus scrofa
1.2.1.105	Ca2+	dependent on, activates through allosteric binding	Bos taurus

EC Number	Organism	UniProt	Comment	Textmining
1.2.1.105	Bos taurus	-	-	-
1.2.1.105	Escherichia coli	-	-	-
1.2.1.105	Homo sapiens	-	-	-
1.2.1.105	Mesocricetus auratus	-	-	-
1.2.1.105	Mus musculus	-	-	-
1.2.1.105	Rattus norvegicus	-	-	-
1.2.1.105	Saccharomyces cerevisiae	-	-	-
1.2.1.105	Sus scrofa	-	-	-

EC Number	Source Tissue	Comment	Organism	Textmining
1.2.1.105	brain	-	Mus musculus	-
1.2.1.105	brain	-	Rattus norvegicus	-
1.2.1.105	brain	diminished activities in regions with severe pathology, as well as in areas of minimal pathology, activity declines with the severity of disease	Homo sapiens	-
1.2.1.105	cerebellum	-	Homo sapiens	-
1.2.1.105	heart	-	Sus scrofa	-
1.2.1.105	kidney	-	Bos taurus	-
1.2.1.105	liver	-	Mus musculus	-
1.2.1.105	additional information	N2a cells	Mus musculus	-
1.2.1.105	ovary	-	Mesocricetus auratus	-
1.2.1.105	PC-12 cell	-	Rattus norvegicus	-

EC Number	Substrates	Comment Substrates	Organism	Products	Comment (Products)	Rev.	Reac.
1.2.1.105	2-oxoglutarate + [dihydrolipoyllysine-residue succinyltransferase] lipoyllysine	-	Bos taurus	[dihydrolipoyllysine-residue succinyltransferase] S-succinyldihydrolipoyllysine + CO2	-	?
1.2.1.105	2-oxoglutarate + [dihydrolipoyllysine-residue succinyltransferase] lipoyllysine	reaction significant for energy production, neurotransmitter metabolism, and metabolic interaction between mitochondria and cytoplasm	Escherichia coli	[dihydrolipoyllysine-residue succinyltransferase] S-succinyldihydrolipoyllysine + CO2	-	?

EC Number	Synonyms	Comment	Organism
1.2.1.105	alpha-ketoglutarate dehydrogenase	-	Mus musculus
1.2.1.105	alpha-ketoglutarate dehydrogenase	-	Homo sapiens
1.2.1.105	alpha-ketoglutarate dehydrogenase	-	Rattus norvegicus
1.2.1.105	alpha-ketoglutarate dehydrogenase	-	Saccharomyces cerevisiae
1.2.1.105	alpha-ketoglutarate dehydrogenase	-	Bos taurus
1.2.1.105	alpha-ketoglutarate dehydrogenase	-	Mesocricetus auratus
1.2.1.105	alpha-ketoglutarate dehydrogenase complex	-	Mus musculus
1.2.1.105	alpha-ketoglutarate dehydrogenase complex	-	Escherichia coli
1.2.1.105	alpha-ketoglutarate dehydrogenase complex	-	Homo sapiens
1.2.1.105	alpha-ketoglutarate dehydrogenase complex	-	Rattus norvegicus
1.2.1.105	alpha-ketoglutarate dehydrogenase complex	-	Sus scrofa
1.2.1.105	alpha-ketoglutarate dehydrogenase complex	-	Saccharomyces cerevisiae
1.2.1.105	alpha-ketoglutarate dehydrogenase complex	-	Bos taurus
1.2.1.105	alpha-ketoglutarate dehydrogenase complex	-	Mesocricetus auratus
1.2.1.105	E1k	-	Mus musculus
1.2.1.105	E1k	-	Homo sapiens
1.2.1.105	E1k	-	Rattus norvegicus
1.2.1.105	E1k	-	Saccharomyces cerevisiae
1.2.1.105	E1k	-	Bos taurus
1.2.1.105	E1k	-	Mesocricetus auratus
1.2.1.105	KGDHC	-	Mus musculus
1.2.1.105	KGDHC	-	Escherichia coli
1.2.1.105	KGDHC	-	Homo sapiens
1.2.1.105	KGDHC	-	Rattus norvegicus
1.2.1.105	KGDHC	-	Sus scrofa
1.2.1.105	KGDHC	-	Saccharomyces cerevisiae
1.2.1.105	KGDHC	-	Bos taurus
1.2.1.105	KGDHC	-	Mesocricetus auratus

EC Number	Cofactor	Comment	Organism
1.2.1.105	lipoic acid	increases KGDHC activity in aged rates	Rattus norvegicus
1.2.1.105	lipoic acid	leads to a stabilization of cognitive functions in Alzheimer's disease patients	Homo sapiens
1.2.1.105	thiamine diphosphate	-	Rattus norvegicus