• Gene id: 4967

Gene Summary

• Gene Symbol: OGDH   Gene   UCSC   Ensembl
• Aliases: AKGDH, E1k, KGD1, OGDC, OGDH2
• Gene name: oxoglutarate dehydrogenase
• Alternate names: 2-oxoglutarate dehydrogenase, mitochondrial, 2-oxoglutarate dehydrogenase complex component E1, OGDC-E1, oxoglutarate (alpha-ketoglutarate) dehydrogenase (lipoamide), oxoglutarate decarboxylase, oxoglutarate dehydrogenase (succinyl-transferring), testicular tis,
• Gene location: 7p13 (44606571: 44709065)     Exons: 26     NC_000007.14
• Gene summary(Entrez): 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
• OMIM: 613022

Protein Summary

• Protein general information: Q02218  
  • Name: 2 oxoglutarate dehydrogenase, mitochondrial (EC 1.2.4.2) (2 oxoglutarate dehydrogenase complex component E1) (OGDC E1) (Alpha ketoglutarate dehydrogenase)
  • Length: 1023  
  • Mass: 115935
• Sequence:

  MFHLRTCAAKLRPLTASQTVKTFSQNRPAAARTFQQIRCYSAPVAAEPFLSGTSSNYVEEMYCAWLENPKSVHKS
  WDIFFRNTNAGAPPGTAYQSPLPLSRGSLAAVAHAQSLVEAQPNVDKLVEDHLAVQSLIRAYQIRGHHVAQLDPL
  GILDADLDSSVPADIISSTDKLGFYGLDESDLDKVFHLPTTTFIGGQESALPLREIIRRLEMAYCQHIGVEFMFI
  NDLEQCQWIRQKFETPGIMQFTNEEKRTLLARLVRSTRFEEFLQRKWSSEKRFGLEGCEVLIPALKTIIDKSSEN
  GVDYVIMGMPHRGRLNVLANVIRKELEQIFCQFDSKLEAADEGSGDVKYHLGMYHRRINRVTDRNITLSLVANPS
  HLEAADPVVMGKTKAEQFYCGDTEGKKVMSILLHGDAAFAGQGIVYETFHLSDLPSYTTHGTVHVVVNNQIGFTT
  DPRMARSSPYPTDVARVVNAPIFHVNSDDPEAVMYVCKVAAEWRSTFHKDVVVDLVCYRRNGHNEMDEPMFTQPL
  MYKQIRKQKPVLQKYAELLVSQGVVNQPEYEEEISKYDKICEEAFARSKDEKILHIKHWLDSPWPGFFTLDGQPR
  SMSCPSTGLTEDILTHIGNVASSVPVENFTIHGGLSRILKTRGEMVKNRTVDWALAEYMAFGSLLKEGIHIRLSG
  QDVERGTFSHRHHVLHDQNVDKRTCIPMNHLWPNQAPYTVCNSSLSEYGVLGFELGFAMASPNALVLWEAQFGDF
  HNTAQCIIDQFICPGQAKWVRQNGIVLLLPHGMEGMGPEHSSARPERFLQMCNDDPDVLPDLKEANFDINQLYDC
  NWVVVNCSTPGNFFHVLRRQILLPFRKPLIIFTPKSLLRHPEARSSFDEMLPGTHFQRVIPEDGPAAQNPENVKR
  LLFCTGKVYYDLTRERKARDMVGQVAITRIEQLSPFPFDLLLKEVQKYPNAELAWCQEEHKNQGYYDYVKPRLRT
  TISRAKPVWYAGRDPAAAPATGNKKTHLTELQRLLDTAFDLDVFKNFS

• Structural information:
  • Interpro: IPR032106  IPR011603  IPR001017  IPR031717  IPR042179   IPR029061  IPR005475  
  • PDB: 3ERY
  • PDBsum: 3ERY
  • DIP: 39353
  • STRING: ENSP00000222673
• Other Databases: GeneCards:  OGDH  Malacards:  OGDH

Gene ontology

GO accession	Term name	Evidence code	Go category
GO:0006099	tricarboxylic acid cycle	IBA	biological process
GO:0004591	oxoglutarate dehydrogenase (succinyl-transferring) activity	IBA	molecular function
GO:0045252	oxoglutarate dehydrogenase complex	IBA	cellular component
GO:0005739	mitochondrion	IBA	cellular component
GO:0006103	2-oxoglutarate metabolic process	IDA	biological process
GO:0006104	succinyl-CoA metabolic process	IDA	biological process
GO:0004591	oxoglutarate dehydrogenase (succinyl-transferring) activity	IDA	molecular function
GO:0030976	thiamine pyrophosphate binding	IDA	molecular function
GO:0006103	2-oxoglutarate metabolic process	IDA	biological process
GO:0006104	succinyl-CoA metabolic process	IDA	biological process
GO:0004591	oxoglutarate dehydrogenase (succinyl-transferring) activity	IDA	molecular function
GO:0106077	histone succinylation	IDA	biological process
GO:0005634	nucleus	IDA	cellular component
GO:0005739	mitochondrion	IDA	cellular component
GO:0045252	oxoglutarate dehydrogenase complex	IDA	cellular component
GO:0004591	oxoglutarate dehydrogenase (succinyl-transferring) activity	IEA	molecular function
GO:0006099	tricarboxylic acid cycle	IEA	biological process
GO:0016624	oxidoreductase activity, acting on the aldehyde or oxo group of donors, disulfide as acceptor	IEA	molecular function
GO:0030976	thiamine pyrophosphate binding	IEA	molecular function
GO:0055114	oxidation-reduction process	IEA	biological process
GO:0055114	oxidation-reduction process	IEA	biological process
GO:0046872	metal ion binding	IEA	molecular function
GO:0016491	oxidoreductase activity	IEA	molecular function
GO:0006096	glycolytic process	IEA	biological process
GO:0005739	mitochondrion	IEA	cellular component
GO:0005634	nucleus	IEA	cellular component
GO:0004591	oxoglutarate dehydrogenase (succinyl-transferring) activity	IEA	molecular function
GO:0005759	mitochondrial matrix	TAS	cellular component
GO:0005759	mitochondrial matrix	TAS	cellular component
GO:0005759	mitochondrial matrix	TAS	cellular component
GO:0006099	tricarboxylic acid cycle	TAS	biological process
GO:0006554	lysine catabolic process	TAS	biological process
GO:0005634	nucleus	IEA	cellular component
GO:0005759	mitochondrial matrix	IEA	cellular component
GO:0005739	mitochondrion	IDA	cellular component
GO:0006091	generation of precursor metabolites and energy	ISS	biological process
GO:0004591	oxoglutarate dehydrogenase (succinyl-transferring) activity	ISS	molecular function
GO:0031966	mitochondrial membrane	ISS	cellular component

KEGG pathways

Pathway id	Pathway name
hsa01100	Metabolic pathways
hsa01200	Carbon metabolism
hsa00020	Citrate cycle

Diseases

Associated diseases	References
Diseases of the tricarboxylic acid cycle	KEGG:H01022
Alpha-ketoglutarate dehydrogenase complex deficiency	KEGG:H02006
Diseases of the tricarboxylic acid cycle	KEGG:H01022
Alpha-ketoglutarate dehydrogenase complex deficiency	KEGG:H02006
Aberrant CpGs in Low Motility Sperm	MIK: 21674046
Cryptorchidism	MIK: 28606200
Spermatogenic defects	MIK: 31037746
Teratozoospermia	MIK: 17327269

PubMed references

PMID	Condition	Mutation	Ethnicity	Population details	Infertility_type	Associated_genes
17327269	Teratozoospermia			13 (5 controls, 8 cases)	Male infertility	GSE6967 analyzed by GEO2R (cutoff 1.5 fold)
21674046	Aberrant CpGs in Low Motility Sperm			18	Male infertility	GSE26881
28606200	Cryptorchidism			Monozgotic twins (1 control, I cwith cryptorchidism)	Male infertility	MeDIP-Seq
31037746	Spermatogenic defects			16 (1 control, 15 cases)	Male infertility	GSE6023 analyzed using GEO2R