OGDH

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Oxoglutarate (alpha-ketoglutarate) dehydrogenase (lipoamide)
PDB 3ery EBI.png
PDB rendering based on 3ery.
Available structures
PDB Ortholog search: PDBe, RCSB
Identifiers
Symbols OGDH ; AKGDH; E1k; OGDC
External IDs OMIM613022 MGI1098267 HomoloGene55662 ChEMBL: 2816 GeneCards: OGDH Gene
EC number 1.2.4.2
Orthologs
Species Human Mouse
Entrez 4967 18293
Ensembl ENSG00000105953 ENSMUSG00000020456
UniProt Q02218 Q60597
RefSeq (mRNA) NM_001003941 NM_001252282
RefSeq (protein) NP_001003941 NP_001239211
Location (UCSC) Chr 7:
44.65 – 44.75 Mb
Chr 11:
6.29 – 6.36 Mb
PubMed search [1] [2]

Alpha-ketoglutarate dehydrogenase also known as 2-oxoglutarate dehydrogenase E1 component, mitochondrial is an enzyme that in humans is encoded by the OGDH gene.[1][2][3]

Function[edit]

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 CO2 during the citric acid cycle. The protein is located in the mitochondrial matrix and uses thiamine pyrophosphate as a cofactor.[3]

Clinical significance[edit]

A congenital deficiency in 2-oxoglutarate dehydrogenase activity is believed to lead to hypotonia, metabolic acidosis, and hyperlactatemia.[3]

Interactive pathway map[edit]

Click on genes, proteins and metabolites below to link to respective articles. [§ 1]

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TCACycle_WP78 go to article go to article go to article go to article go to HMDB go to article go to article go to article Go to article go to article go to article go to article go to article go to article Go to article go to article go to article go to article go to article go to article go to article go to article go to article go to article go to article go to article go to article go to article go to article go to article go to article go to article go to article go to HMDB go to article go to article go to HMDB go to article go to article go to HMDB go to article go to article go to HMDB go to article go to article go to article go to article go to article go to article go to article go to article go to article go to article go to WikiPathways go to article go to article go to article go to article
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TCA Cycle edit
  1. ^ The interactive pathway map can be edited at WikiPathways: "TCACycle_WP78". 

References[edit]

  1. ^ Koike K, Urata Y, Goto S (March 1992). "Cloning and nucleotide sequence of the cDNA encoding human 2-oxoglutarate dehydrogenase (lipoamide)". Proc. Natl. Acad. Sci. U.S.A. 89 (5): 1963–7. doi:10.1073/pnas.89.5.1963. PMC 48574. PMID 1542694. 
  2. ^ Szabo P, Cai X, Ali G, Blass JP (March 1994). "Localization of the gene (OGDH) coding for the E1k component of the alpha-ketoglutarate dehydrogenase complex to chromosome 7p13-p11.2". Genomics 20 (2): 324–6. doi:10.1006/geno.1994.1178. PMID 8020988. 
  3. ^ a b c "Entrez Gene: oxoglutarate (alpha-ketoglutarate) dehydrogenase (lipoamide)". 

Further reading[edit]

This article incorporates text from the United States National Library of Medicine, which is in the public domain.