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Aliases for OGDH Gene

Aliases for OGDH Gene

  • Oxoglutarate (Alpha-Ketoglutarate) Dehydrogenase (Lipoamide) 2 3
  • 2-Oxoglutarate Dehydrogenase Complex Component E1 3 4
  • EC 1.2.4.2 4 63
  • OGDC-E1 3 4
  • Oxoglutarate Dehydrogenase (Succinyl-Transferring) 3
  • 2-Oxoglutarate Dehydrogenase, Mitochondrial 3
  • Alpha-Ketoglutarate Dehydrogenase 4
  • Oxoglutarate Decarboxylase 3
  • AKGDH 3
  • OGDC 3
  • E1k 3

External Ids for OGDH Gene

Summaries for OGDH Gene

Entrez Gene Summary for OGDH 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]

GeneCards Summary for OGDH Gene

OGDH (Oxoglutarate (Alpha-Ketoglutarate) Dehydrogenase (Lipoamide)) is a Protein Coding gene. Diseases associated with OGDH include alpha-ketoglutarate dehydrogenase deficiency and door syndrome. Among its related pathways are Metabolism and Metabolism. GO annotations related to this gene include chaperone binding and thiamine pyrophosphate binding. An important paralog of this gene is DHTKD1.

UniProtKB/Swiss-Prot for OGDH Gene

  • 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)

Gene Wiki entry for OGDH Gene

No data available for Tocris Summary , PharmGKB "VIP" Summary , fRNAdb sequence ontologies and piRNA Summary for OGDH Gene

Genomics for OGDH Gene

Genomic Location for OGDH Gene

Start:
44,606,522 bp from pter
End:
44,709,070 bp from pter
Size:
102,549 bases
Orientation:
Plus strand

Genomic View for OGDH Gene

UCSC Golden Path with GeneCards custom track
Cytogenetic band:
Genomic Location for OGDH Gene
GeneLoc Logo Genomic Neighborhood Exon StructureGene Density

RefSeq DNA sequence for OGDH Gene

Regulatory Elements for OGDH Gene

Proteins for OGDH Gene

  • Protein details for OGDH Gene (UniProtKB/Swiss-Prot)

    Protein Symbol:
    Q02218-ODO1_HUMAN
    Recommended name:
    2-oxoglutarate dehydrogenase, mitochondrial
    Protein Accession:
    Q02218
    Secondary Accessions:
    • B4E2U9
    • D3DVL0
    • E9PBM1
    • Q96DD3
    • Q9UDX0

    Protein attributes for OGDH Gene

    Size:
    1023 amino acids
    Molecular mass:
    115935 Da
    Cofactor:
    Name=thiamine diphosphate; Xref=ChEBI:CHEBI:58937;
    Quaternary structure:
    No Data Available
    SequenceCaution:
    • Sequence=BAA01393.1; Type=Frameshift; Positions=1003; Evidence={ECO:0000305}; Sequence=BAA06836.1; Type=Frameshift; Positions=1003; Evidence={ECO:0000305};

    Three dimensional structures from OCA and Proteopedia for OGDH Gene

    Alternative splice isoforms for OGDH Gene

    UniProtKB/Swiss-Prot:

neXtProt entry for OGDH Gene

Proteomics data for OGDH Gene at MOPED

Post-translational modifications for OGDH Gene

  • Modification sites at PhosphoSitePlus
  • Ubiquitination at Lys74 and Lys534

Other Protein References for OGDH Gene

Domains for OGDH Gene

Protein Domains for OGDH Gene

Graphical View of Domain Structure for InterPro Entry

Q02218

UniProtKB/Swiss-Prot:

ODO1_HUMAN
Family:
  • Belongs to the alpha-ketoglutarate dehydrogenase family.:
    • Q02218
genes like me logo Genes that share domains with OGDH: view

No data available for Gene Families for OGDH Gene

Function for OGDH Gene

Molecular function for OGDH Gene

GENATLAS Biochemistry: 2-oxoglutarate lipoamide 2 oxidoreductase (decarboxylating and acceptor-succinylating,component E1 of the 2-oxoglutarate complex,mitochondrial matrix
UniProtKB/Swiss-Prot CatalyticActivity: 2-oxoglutarate + [dihydrolipoyllysine-residue succinyltransferase] lipoyllysine = [dihydrolipoyllysine-residue succinyltransferase] S-succinyldihydrolipoyllysine + CO(2)
UniProtKB/Swiss-Prot EnzymeRegulation: Calcium ions and ADP stimulate, whereas ATP and NADH reduce catalytic activity.
UniProtKB/Swiss-Prot 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)

Enzyme Numbers (IUBMB) for OGDH Gene

Gene Ontology (GO) - Molecular Function for OGDH Gene

GO ID Qualified GO term Evidence PubMed IDs
GO:0004591 oxoglutarate dehydrogenase (succinyl-transferring) activity ISS --
GO:0016624 oxidoreductase activity, acting on the aldehyde or oxo group of donors, disulfide as acceptor --
GO:0030976 thiamine pyrophosphate binding IEA --
GO:0031072 heat shock protein binding IEA --
GO:0034602 oxoglutarate dehydrogenase (NAD+) activity IEA --
genes like me logo Genes that share ontologies with OGDH: view

Phenotypes for OGDH Gene

genes like me logo Genes that share phenotypes with OGDH: view

miRNA for OGDH Gene

No data available for Animal Models , Transcription Factor Targeting and HOMER Transcription for OGDH Gene

Localization for OGDH Gene

Subcellular locations from UniProtKB/Swiss-Prot for OGDH Gene

Mitochondrion matrix.

Subcellular locations from

COMPARTMENTS
Jensen Localization Image for OGDH Gene COMPARTMENTS Subcellular localization image for OGDH gene
Compartment Confidence
mitochondrion 5
cytosol 2
nucleus 1
plasma membrane 1

Gene Ontology (GO) - Cellular Components for OGDH Gene

GO ID Qualified GO term Evidence PubMed IDs
GO:0005739 mitochondrion IDA --
GO:0005759 mitochondrial matrix TAS --
GO:0005829 NOT cytosol IBA --
GO:0031966 mitochondrial membrane ISS --
GO:0045252 oxoglutarate dehydrogenase complex IBA --
genes like me logo Genes that share ontologies with OGDH: view

Pathways for OGDH Gene

genes like me logo Genes that share pathways with OGDH: view

Gene Ontology (GO) - Biological Process for OGDH Gene

GO ID Qualified GO term Evidence PubMed IDs
GO:0006091 generation of precursor metabolites and energy ISS --
GO:0006096 glycolytic process IEA --
GO:0006099 tricarboxylic acid cycle TAS --
GO:0006103 2-oxoglutarate metabolic process IEA --
GO:0006104 succinyl-CoA metabolic process IEA --
genes like me logo Genes that share ontologies with OGDH: view

Compounds for OGDH Gene

(16) HMDB Compounds for OGDH Gene

Compound Synonyms Cas Number PubMed IDs
(S)-Succinyldihydrolipoamide
  • S-Succinyldihydrolipoamide
Not Available
2-(a-Hydroxyethyl)thiamine diphosphate
  • 2-Hydroxyethyl-ThPP
10055-47-7
2-Ketobutyric acid
  • 2-Ketobutanoate
600-18-0
3-carboxy-1-hydroxypropylthiamine diphosphate
  • 3-Carboxy-1-hydroxypropyl-ThPP
Not Available
Carbon dioxide
  • Carbon oxide
124-38-9

(2) Drugbank Compounds for OGDH Gene

Compound Synonyms Cas Number Type Actions PubMed IDs
NADH
  • beta-DPNH
606-68-8 target
Valproic Acid
  • 2-Propylpentanoic Acid
99-66-1 target inhibitor

(55) Novoseek inferred chemical compound relationships for OGDH Gene

Compound -log(P) Hits PubMed IDs
alpha-ketoglutarate 97.3 150
lspfpfdl 86.3 1
dihydrolipoamide 85.1 13
pyruvate 84.9 73
2-oxo acid 83.2 5
genes like me logo Genes that share compounds with OGDH: view

Transcripts for OGDH Gene

Unigene Clusters for OGDH Gene

Oxoglutarate (alpha-ketoglutarate) dehydrogenase (lipoamide):
Representative Sequences:

Alternative Splicing Database (ASD) splice patterns (SP) for OGDH Gene

ExUns: 1a · 1b · 1c · 1d · 1e · 1f ^ 2a · 2b · 2c ^ 3 ^ 4a · 4b · 4c · 4d ^ 5a · 5b · 5c · 5d · 5e ^ 6 ^ 7 ^ 8 ^ 9a · 9b · 9c ^ 10a ·
SP1: - -
SP2: - - - - - - -
SP3: - - - - - - - -
SP4: - - - - - - -
SP5:
SP6: - - - - - - - - - - - - - - -
SP7: - - - - - - - - - - - - - - - - - - - -
SP8: - - - - - - - - - - - - - - -
SP9: - - - - -
SP10: - - - - - - - - - - - - - - - - - - -
SP11: - - -
SP12: -
SP13:
SP14:
SP15: - - -
SP16: -
SP17:

ExUns: 10b · 10c · 10d ^ 11a · 11b · 11c · 11d ^ 12 ^ 13a · 13b · 13c ^ 14 ^ 15 ^ 16a · 16b ^ 17 ^ 18a · 18b · 18c ^ 19 ^ 20a · 20b ^ 21 ^ 22 ^ 23 ^ 24 ^
SP1: - - -
SP2:
SP3:
SP4:
SP5: -
SP6:
SP7: - - - - - - - - - - - - - - - - -
SP8: - - -
SP9:
SP10: - - - - - - - - - -
SP11:
SP12:
SP13:
SP14:
SP15:
SP16:
SP17:

ExUns: 25 ^ 26 ^ 27
SP1:
SP2:
SP3:
SP4:
SP5:
SP6:
SP7:
SP8:
SP9:
SP10:
SP11:
SP12:
SP13:
SP14:
SP15:
SP16:
SP17:

Relevant External Links for OGDH Gene

GeneLoc Exon Structure for
OGDH
ECgene alternative splicing isoforms for
OGDH

Expression for OGDH Gene

mRNA expression in normal human tissues for OGDH Gene

mRNA expression in embryonic tissues and stem cells from LifeMap Discovery

mRNA differential expression in normal tissues according to GTEx for OGDH Gene

This gene is overexpressed in Heart - Left Ventricle (6.9) and Muscle - Skeletal (5.6).

Integrated Proteomics: protein expression from ProteomicsDB, PaxDb, MOPED, and MaxQB for OGDH Gene

SOURCE GeneReport for Unigene cluster for OGDH Gene Hs.488181

genes like me logo Genes that share expressions with OGDH: view

No data available for mRNA Expression by UniProt/SwissProt for OGDH Gene

Orthologs for OGDH Gene

This gene was present in the common ancestor of eukaryotes.

Orthologs for OGDH Gene

Organism Taxonomy Gene Similarity Type Details
chimpanzee
(Pan troglodytes)
Mammalia OGDH 35
  • 99.64 (n)
  • 100 (a)
OGDH 36
  • 99 (a)
OneToOne
cow
(Bos Taurus)
Mammalia OGDH 35
  • 90.16 (n)
  • 98.24 (a)
OGDH 36
  • 98 (a)
OneToOne
dog
(Canis familiaris)
Mammalia -- 36
  • 97 (a)
OneToMany
-- 36
  • 92 (a)
OneToMany
OGDH 35
  • 89.46 (n)
  • 98.58 (a)
mouse
(Mus musculus)
Mammalia Ogdh 35
  • 89.21 (n)
  • 95.89 (a)
Ogdh 16
Ogdh 36
  • 93 (a)
OneToOne
oppossum
(Monodelphis domestica)
Mammalia OGDH 36
  • 93 (a)
OneToOne
platypus
(Ornithorhynchus anatinus)
Mammalia OGDH 36
  • 87 (a)
OneToOne
rat
(Rattus norvegicus)
Mammalia Ogdh 35
  • 89.44 (n)
  • 96.29 (a)
chicken
(Gallus gallus)
Aves OGDH 35
  • 82.78 (n)
  • 91.83 (a)
OGDH 36
  • 92 (a)
OneToOne
lizard
(Anolis carolinensis)
Reptilia OGDH 36
  • 91 (a)
OneToOne
African clawed frog
(Xenopus laevis)
Amphibia MGC68800 35
tropical clawed frog
(Silurana tropicalis)
Amphibia ogdh 35
  • 78.39 (n)
  • 89.49 (a)
Str.8708 35
rainbow trout
(Oncorhynchus mykiss)
Actinopterygii Omy.12508 35
zebrafish
(Danio rerio)
Actinopterygii -- 35
ogdha 35
  • 77.1 (n)
  • 86.85 (a)
ogdha 36
  • 87 (a)
OneToMany
ogdhb 36
  • 84 (a)
OneToMany
African malaria mosquito
(Anopheles gambiae)
Insecta AgaP_AGAP006366 35
  • 64.78 (n)
  • 65.57 (a)
fruit fly
(Drosophila melanogaster)
Insecta CG33791 36
  • 42 (a)
ManyToMany
Nc73EF 35
  • 62.41 (n)
  • 62.98 (a)
Nc73EF 36
  • 56 (a)
ManyToMany
CG18170 37
  • 51 (a)
CG11661 37
  • 61 (a)
CG32316-ORFB 37
  • 53 (a)
worm
(Caenorhabditis elegans)
Secernentea ogdh-1 35
  • 57.97 (n)
  • 59.03 (a)
ogdh-1 36
  • 56 (a)
OneToMany
T22B11.5 37
  • 57 (a)
A. gosspyii yeast
(Ashbya gossypii)
Saccharomycetes AGOS_AER374C 35
  • 53.96 (n)
  • 48.21 (a)
baker's yeast
(Saccharomyces cerevisiae)
Saccharomycetes KGD1 35
  • 51.38 (n)
  • 47.91 (a)
KGD1 36
  • 45 (a)
OneToMany
KGD1 38
K. lactis yeast
(Kluyveromyces lactis)
Saccharomycetes KLLA0F04477g 35
  • 50.83 (n)
  • 47.93 (a)
Alicante grape
(Vitis vinifera)
eudicotyledons Vvi.10739 35
thale cress
(Arabidopsis thaliana)
eudicotyledons AT3G55410 35
  • 54.56 (n)
  • 52.03 (a)
corn
(Zea mays)
Liliopsida Zm.6807 35
rice
(Oryza sativa)
Liliopsida Os.11872 35
Os07g0695800 35
  • 57.81 (n)
  • 52.22 (a)
bread mold
(Neurospora crassa)
Ascomycetes NCU05425 35
  • 55.61 (n)
  • 47.38 (a)
fission yeast
(Schizosaccharomyces pombe)
Schizosaccharomycetes SPBC3H7.03c 35
  • 52.36 (n)
  • 47.74 (a)
sea squirt
(Ciona savignyi)
Ascidiacea CSA.7934 36
  • 64 (a)
OneToMany
Species with no ortholog for OGDH:
  • Actinobacteria (Mycobacterium tuberculosis)
  • alpha proteobacteria (Wolbachia pipientis)
  • amoeba (Dictyostelium discoideum)
  • Archea (Pyrococcus horikoshii)
  • barley (Hordeum vulgare)
  • beta proteobacteria (Neisseria meningitidis)
  • Chromalveolata (Phytophthora infestans)
  • common water flea (Daphnia pulex)
  • E. coli (Escherichia coli)
  • filamentous fungi (Aspergillus nidulans)
  • Firmicute bacteria (Streptococcus pneumoniae)
  • green algae (Chlamydomonas reinhardtii)
  • honey bee (Apis mellifera)
  • loblloly pine (Pinus taeda)
  • malaria parasite (Plasmodium falciparum)
  • medicago trunc (Medicago Truncatula)
  • moss (Physcomitrella patens)
  • orangutan (Pongo pygmaeus)
  • pig (Sus scrofa)
  • rice blast fungus (Magnaporthe grisea)
  • schistosome parasite (Schistosoma mansoni)
  • sea anemone (Nematostella vectensis)
  • sea urchin (Strongylocentrotus purpuratus)
  • sorghum (Sorghum bicolor)
  • soybean (Glycine max)
  • stem rust fungus (Puccinia graminis)
  • sugarcane (Saccharum officinarum)
  • tomato (Lycopersicon esculentum)
  • toxoplasmosis (Toxoplasma gondii)
  • Trichoplax (Trichoplax adhaerens)
  • wheat (Triticum aestivum)

Evolution for OGDH Gene

ENSEMBL:
Gene Tree for OGDH (if available)
TreeFam:
Gene Tree for OGDH (if available)

Paralogs for OGDH Gene

Paralogs for OGDH Gene

Selected SIMAP similar genes for OGDH Gene using alignment to 10 proteins:

genes like me logo Genes that share paralogs with OGDH: view

Variants for OGDH Gene

Sequence variations from dbSNP and Humsavar for OGDH Gene

SNP ID Clin Chr 07 pos Sequence Context AA Info Type MAF
rs12868 -- 44,708,731(+) CCACC(A/G)CCGGG utr-variant-3-prime
rs14239 -- 44,708,304(+) CACAA(C/T)CCTCC utr-variant-3-prime
rs710887 -- 44,669,773(-) AGCGC(A/G)TACAG intron-variant
rs718336 -- 44,659,373(+) ACTAT(A/C)TTTGC intron-variant
rs732797 -- 44,607,164(-) AAAGC(C/T)AGATG intron-variant

Structural Variations from Database of Genomic Variants (DGV) for OGDH Gene

Variant ID Type Subtype PubMed ID
esv259584 OTHER Complex 20981092
esv259884 OTHER Complex 20981092
nsv820209 CNV Loss 19587683

Relevant External Links for OGDH Gene

HapMap Linkage Disequilibrium report
OGDH

No data available for Polymorphic Variants from UniProtKB/Swiss-Prot for OGDH Gene

Disorders for OGDH Gene

(1) OMIM Diseases for OGDH Gene (613022)

(16) Novoseek inferred disease relationships for OGDH Gene

Disease -log(P) Hits PubMed IDs
door syndrome 74.1 1
biliary cirrhosis primary 71.8 12
thiamine deficiency 70.2 2
korsakoff syndrome 53.9 1
alzheimers disease 36.2 3

Genatlas disease for OGDH Gene

congenital lactic acidosis with hypotonia and progressive neurologic deterioration leading to early death

Relevant External Links for OGDH

Genetic Association Database (GAD)
OGDH
Human Genome Epidemiology (HuGE) Navigator
OGDH
genes like me logo Genes that share disorders with OGDH: view

No data available for UniProtKB/Swiss-Prot for OGDH Gene

Publications for OGDH Gene

  1. Cloning and nucleotide sequence of the cDNA encoding human 2- oxoglutarate dehydrogenase (lipoamide). (PMID: 1542694) Koike K. … Goto S. (Proc. Natl. Acad. Sci. U.S.A. 1992) 2 3 4 23
  2. Localization of the gene (OGDH) coding for the E1k component of the alpha-ketoglutarate dehydrogenase complex to chromosome 7p13-p11.2. (PMID: 8020988) Szabo P. … Blass J.P. (Genomics 1994) 2 3 23
  3. The gene encoding human 2-oxoglutarate dehydrogenase: structural organization and mapping to chromosome 7p13-p14. (PMID: 7622061) Koike K. (Gene 1995) 3 4 23
  4. Regulation of 2-oxoglutarate (alpha-ketoglutarate) dehydrogenase stability by the RING finger ubiquitin ligase Siah. (PMID: 15466852) Habelhah H. … Ronai Z. (J. Biol. Chem. 2004) 3 23
  5. Tryptic digestion of ubiquitin standards reveals an improved strategy for identifying ubiquitinated proteins by mass spectrometry. (PMID: 17370265) Denis N.J. … Figeys D. (Proteomics 2007) 3 4

Products for OGDH Gene

Sources for OGDH Gene

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