Names & Taxonomy

Uniprot ID:
Q02218
Entry Name:
ODO1_HUMAN
Status:
reviewed
Protein Names:
2-oxoglutarate dehydrogenase, mitochondrial (EC 1.2.4.2) (2-oxoglutarate dehydrogenase complex component E1) (OGDC-E1) (Alpha-ketoglutarate dehydrogenase)
Gene Names:
OGDH
Gene Names Primary:
OGDH
Organism:
Homo sapiens (Human)

Structure

Length:
1023
Sequence:
MFHLRTCAAKLRPLTASQTVKTFSQNRPAAARTFQQIRCYSAPVAAEPFLSGTSSNYVEEMYCAWLENPKSVHKSWDIFFRNTNAGAPPGTAYQSPLPLSRGSLAAVAHAQSLVEAQPNVDKLVEDHLAVQSLIRAYQIRGHHVAQLDPLGILDADLDSSVPADIISSTDKLGFYGLDESDLDKVFHLPTTTFIGGQESALPLREIIRRLEMAYCQHIGVEFMFINDLEQCQWIRQKFETPGIMQFTNEEKRTLLARLVRSTRFEEFLQRKWSSEKRFGLEGCEVLIPALKTIIDKSSENGVDYVIMGMPHRGRLNVLANVIRKELEQIFCQFDSKLEAADEGSGDVKYHLGMYHRRINRVTDRNITLSLVANPSHLEAADPVVMGKTKAEQFYCGDTEGKKVMSILLHGDAAFAGQGIVYETFHLSDLPSYTTHGTVHVVVNNQIGFTTDPRMARSSPYPTDVARVVNAPIFHVNSDDPEAVMYVCKVAAEWRSTFHKDVVVDLVCYRRNGHNEMDEPMFTQPLMYKQIRKQKPVLQKYAELLVSQGVVNQPEYEEEISKYDKICEEAFARSKDEKILHIKHWLDSPWPGFFTLDGQPRSMSCPSTGLTEDILTHIGNVASSVPVENFTIHGGLSRILKTRGEMVKNRTVDWALAEYMAFGSLLKEGIHIRLSGQDVERGTFSHRHHVLHDQNVDKRTCIPMNHLWPNQAPYTVCNSSLSEYGVLGFELGFAMASPNALVLWEAQFGDFHNTAQCIIDQFICPGQAKWVRQNGIVLLLPHGMEGMGPEHSSARPERFLQMCNDDPDVLPDLKEANFDINQLYDCNWVVVNCSTPGNFFHVLRRQILLPFRKPLIIFTPKSLLRHPEARSSFDEMLPGTHFQRVIPEDGPAAQNPENVKRLLFCTGKVYYDLTRERKARDMVGQVAITRIEQLSPFPFDLLLKEVQKYPNAELAWCQEEHKNQGYYDYVKPRLRTTISRAKPVWYAGRDPAAAPATGNKKTHLTELQRLLDTAFDLDVFKNFS
Proteomes:
UP000005640

Subcellular location

Subcellular Location:
Mitochondrion matrix.

Function

Function:
The 2-oxoglutarate dehydrogenase complex catalyzes the overall conversion of 2-oxoglutarate to succinyl-CoA and CO(2). It contains multiple copies of three enzymatic components: 2-oxoglutarate dehydrogenase (E1), dihydrolipoamide succinyltransferase (E2) and lipoamide dehydrogenase (E3).
Catalytic Activity:
2-oxoglutarate + lipoyllysine = S-succinyldihydrolipoyllysine + CO(2).
Cofactor:
COFACTOR: Name=thiamine diphosphate; Xref=ChEBI:CHEBI:58937;
Enzyme Regulation:
ENZYME REGULATION: Calcium ions and ADP stimulate, whereas ATP and NADH reduce catalytic activity.
Cross Reference Drug Bank:
DB00313
Gene Ontology Go:
mitochondrial matrix
mitochondrial membrane
mitochondrion
oxoglutarate dehydrogenase complex
metal ion binding
oxoglutarate dehydrogenase (NAD+) activity
oxoglutarate dehydrogenase (succinyl-transferring) activity
thiamine pyrophosphate binding
2-oxoglutarate metabolic process
cellular metabolic process
cellular nitrogen compound metabolic process
cerebellar cortex development
generation of precursor metabolites and energy
glycolytic process
glyoxylate metabolic process
hippocampus development
lysine catabolic process
NADH metabolic process
olfactory bulb mitral cell layer development
pyramidal neuron development
small molecule metabolic process
striatum development
succinyl-CoA metabolic process
tangential migration from the subventricular zone to the olfactory bulb
thalamus development
tricarboxylic acid cycle
Gene Ontology Biological Process:
2-oxoglutarate metabolic process
cellular metabolic process
cellular nitrogen compound metabolic process
cerebellar cortex development
generation of precursor metabolites and energy
glycolytic process
glyoxylate metabolic process
hippocampus development
lysine catabolic process
NADH metabolic process
olfactory bulb mitral cell layer development
pyramidal neuron development
small molecule metabolic process
striatum development
succinyl-CoA metabolic process
tangential migration from the subventricular zone to the olfactory bulb
thalamus development
tricarboxylic acid cycle
Gene Ontology Molecular Function:
metal ion binding
oxoglutarate dehydrogenase (NAD+) activity
oxoglutarate dehydrogenase (succinyl-transferring) activity
thiamine pyrophosphate binding
Gene Ontology Cellular Component:
mitochondrial matrix
mitochondrial membrane
mitochondrion
oxoglutarate dehydrogenase complex
Keywords:
3D-structure
Acetylation
Alternative splicing
Calcium
Complete proteome
Glycolysis
Isopeptide bond
Metal-binding
Mitochondrion
Oxidoreductase
Phosphoprotein
Polymorphism
Reference proteome
Thiamine pyrophosphate
Transit peptide
Ubl conjugation

Publication

PubMed ID:
1542694 7622061 14702039 12853948 15489334 17370265 19608861 21269460 24495017 24275569 25944712 18973345