OGDH

Protein identityUniProt · HPA

UniProt accession

Q02218

Gene

OGDH

Ensembl

ENSG00000105953

Chromosome

7

Canonical length

1023 aa

Protein class

Citric acid cycle related proteins, Enzymes, FDA approved drug targets, Human disease related genes, Metabolic proteins, Plasma proteins, Predicted intracellular proteins

Subcellular location

Mitochondria

Quaternary structure

Homodimer

OverviewNCBI Gene

This gene encodes one subunit of the 2-oxoglutarate dehydrogenase complex. This complex catalyzes the overall conversion of 2-oxoglutarate (alpha-ketoglutarate) to succinyl-CoA and CO(2) during the Krebs cycle. The protein is located in the mitochondrial matrix and uses thiamine pyrophosphate as a cofactor. A congenital deficiency in 2-oxoglutarate dehydrogenase activity is believed to lead to hypotonia, metabolic acidosis, and hyperlactatemia. Alternative splicing results in multiple transcript variants encoding distinct isoforms.[provided by RefSeq, Sep 2009]

Canonical amino-acid sequenceUniProt

1023 residues, UniProt reviewed canonical sequence.

>Q02218|OGDH
     1  MFHLRTCAAK LRPLTASQTV KTFSQNRPAA ARTFQQIRCY SAPVAAEPFL SGTSSNYVEE
    61  MYCAWLENPK SVHKSWDIFF RNTNAGAPPG TAYQSPLPLS RGSLAAVAHA QSLVEAQPNV
   121  DKLVEDHLAV QSLIRAYQIR GHHVAQLDPL GILDADLDSS VPADIISSTD KLGFYGLDES
   181  DLDKVFHLPT TTFIGGQESA LPLREIIRRL EMAYCQHIGV EFMFINDLEQ CQWIRQKFET
   241  PGIMQFTNEE KRTLLARLVR STRFEEFLQR KWSSEKRFGL EGCEVLIPAL KTIIDKSSEN
   301  GVDYVIMGMP HRGRLNVLAN VIRKELEQIF CQFDSKLEAA DEGSGDVKYH LGMYHRRINR
   361  VTDRNITLSL VANPSHLEAA DPVVMGKTKA EQFYCGDTEG KKVMSILLHG DAAFAGQGIV
   421  YETFHLSDLP SYTTHGTVHV VVNNQIGFTT DPRMARSSPY PTDVARVVNA PIFHVNSDDP
   481  EAVMYVCKVA AEWRSTFHKD VVVDLVCYRR NGHNEMDEPM FTQPLMYKQI RKQKPVLQKY
   541  AELLVSQGVV NQPEYEEEIS KYDKICEEAF ARSKDEKILH IKHWLDSPWP GFFTLDGQPR
   601  SMSCPSTGLT EDILTHIGNV ASSVPVENFT IHGGLSRILK TRGEMVKNRT VDWALAEYMA
   661  FGSLLKEGIH IRLSGQDVER GTFSHRHHVL HDQNVDKRTC IPMNHLWPNQ APYTVCNSSL
   721  SEYGVLGFEL GFAMASPNAL VLWEAQFGDF HNTAQCIIDQ FICPGQAKWV RQNGIVLLLP
   781  HGMEGMGPEH SSARPERFLQ MCNDDPDVLP DLKEANFDIN QLYDCNWVVV NCSTPGNFFH
   841  VLRRQILLPF RKPLIIFTPK SLLRHPEARS SFDEMLPGTH FQRVIPEDGP AAQNPENVKR
   901  LLFCTGKVYY DLTRERKARD MVGQVAITRI EQLSPFPFDL LLKEVQKYPN AELAWCQEEH
   961  KNQGYYDYVK PRLRTTISRA KPVWYAGRDP AAAPATGNKK THLTELQRLL DTAFDLDVFK
  1021  NFS

LocalizationUniProt · AlphaFold · HPA

Whether an antibody against OGDH can act on the native protein depends on physical access: surface and secreted proteins are reachable by circulating antibodies, intracellular proteins usually are not.

Antibody reachability

Intracellular

Secreted

No

Transmembrane segments

0

Mean surface accessibility (rSASA)

0.24

Highest tissue expression

350 nTPM

Expression across tissuesHPA

DiseaseUniProt · ClinVar · IEDB · PubMed

Four sources answering four different questions about OGDH.

Disease | GeneticClinVar

5 pathogenic / likely-pathogenic of 278 ClinVar records.

Conditions with pathogenic or likely-pathogenic variants.

• Oxoglutaricaciduria

Disease | ImmuneIEDB

Conditions an epitope on OGDH was assayed in.

• Parkinson's disease T cell

Genetic constraint and essentialitygnomAD · DepMap

Does the body need this protein intact? Low LOEUF or a strong DepMap dependency means loss or blockade of the protein is likely to be felt.

gnomAD LOEUF (loss-of-function intolerance)

0.27

gnomAD pLI

1

gnomAD missense Z

4.76

DepMap mean gene effect

-0.59

DepMap dependency class

common

Cancer expressionTCGA

Across TCGA tumor cohorts, this protein is over-expressed in roughly 9% of surveyed tumor types (aggregate summary; per-cohort expression, alteration, and survival load in the interactive view).

OntologyGO

Biological processes

• 2-oxoglutarate decarboxylation to succinyl-CoA
• 2-oxoglutarate metabolic process
• cerebellar cortex development
• generation of precursor metabolites and energy
• glycolytic process
• hippocampus development
• olfactory bulb mitral cell layer development
• pyramidal neuron development
• striatum development
• succinyl-CoA metabolic process
• tangential migration from the subventricular zone to the olfactory bulb
• thalamus development
• tricarboxylic acid cycle

Molecular functions

• heat shock protein binding
• metal ion binding
• oxoglutarate dehydrogenase (succinyl-transferring) activity
• protein-folding chaperone binding
• thiamine pyrophosphate binding

Cellular components

• mitochondrial matrix
• mitochondrial membrane
• mitochondrion
• nucleus
• oxoglutarate dehydrogenase complex

Protein domainsUniProt · Pfam · InterPro

• Dehydrogenase, E1 component
• Transketolase-like, pyrimidine-binding domain
• 2-oxoglutarate dehydrogenase E1 component
• Thiamin diphosphate-binding fold
• 2-oxoglutarate dehydrogenase E1 component/KDG, C-terminal
• 2-oxoglutarate dehydrogenase E1 component, N-terminal domain
• Multifunctional 2-oxoglutarate metabolism enzyme, C-terminal domain superfamily
• Dehydrogenase E1 component
• Transketolase, pyrimidine binding domain
• 2-oxoglutarate dehydrogenase N-terminus
• 2-oxoglutarate dehydrogenase C-terminal

KeywordsUniProt

• Acetylation
• Calcium
• Glycolysis
• Isopeptide bond
• Magnesium
• Metal-binding
• Mitochondrion
• Nucleus
• Oxidoreductase
• Phosphoprotein
• Thiamine pyrophosphate
• Transit peptide
• Ubl conjugation

InteractionsUniProt · HPA

Protein binding partners of OGDH in the human serome: UniProt-annotated complex subunits plus reported interactors. Each links to its own Seroatlas record.

• ABHD11
• OGDHL