TCA Cycle and Deficiency of Pyruvate Dehydrogenase complex (PDHc) (Homo sapiens)

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Pyruvate Dehydrogenase ComplexIDH3AOxaloacetate(S)-MalateSUCLG2Succinoyl-CoACitrateS-Succinyl-dihydrolipoamide-ESuccinateDLATMDH1Lipoamide-EGlycolysisPDHA1FH2-Oxo-glutaratePhosphoenol-pyruvateOGDHOxalosuccinateDihydro-lipoamide-EPCIDH1Acetyl-CoAACLYIsocitrateCS2-Hydroxy-ethyl-ThPPPCK1FumarateIDH1DLSTOGDHPyruvateACO1DLD3-carboxy-1-hydroxypropyl-ThPPSDHAThPPS-Acetyldihydro-lipoamide-EPDHA1ThPPDihydro-lipoamide-ELipoamide-EDLD


When transported into the inner mitochondrial matrix, pyruvate encounters two principal metabolizing enzymes: pyruvate carboxylase, PC (a gluconeogenic enzyme) and pyruvate dehydrogenase (PDH), the first enzyme of the PDH complex (PDHc). With a high cell-energy charge, co-enzyme A (CoA) is highly acylated, principally as acetyl-CoA, and able to obligately activate pyruvate carboxylase, directing pyruvate toward gluconeogenesis. When the energy charge is low, CoA is not acylated, therefore, pyruvate carboxylase is inactive, and pyruvate is preferentially metabolized via the PDHc and the TCA cycle to CO2 and H2O. The acetyl-CoA produced by the PDHc enters the TCA cycle and the reduced electron carriers (NADH and FADH2) that are generated during the oxidative reactions can then be used to drive ATP synthesis via oxidative phosphorylation.

Description source: The Medical Biochemistryp Page

Proteins on this pathway have targeted assays available via the CPTAC Assay Portal.

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107130view14:21, 17 September 2019MaintBotChEBI identifier normalization
105833view23:08, 15 August 2019KhanspersModified description
96056view16:30, 15 February 2018Mkutmonremoved old pathway attributes
96055view16:28, 15 February 2018MkutmonModified description
96054view16:28, 15 February 2018MkutmonModified description
94220view22:19, 25 August 2017DeSlAdded IDs for 3 missing metabolites
87731view09:25, 25 July 2016MirellaKalafatiOntology Term : 'pyruvate dehydrogenase complex deficiency pathway' added !
87724view09:20, 25 July 2016MirellaKalafatiOntology Term : 'PW:0000002' removed !
87723view09:19, 25 July 2016MirellaKalafatiOntology Term : 'disease pathway' added !
87722view09:19, 25 July 2016MirellaKalafatiOntology Term : 'citric acid cycle pathway' added !
87721view09:19, 25 July 2016MirellaKalafatiOntology Term : 'classic metabolic pathway' added !
87720view09:18, 25 July 2016MirellaKalafatiModified title
87376view09:56, 22 July 2016MaintBotadded missing graphids
80143view17:16, 14 May 2015Khanspersupdated xrefs
80142view17:11, 14 May 2015Khanspersfixed connections, updated annotations
78495view10:28, 7 January 2015MaintBotadded missing graphIds
72092view21:32, 24 October 2013Mkutmonadded datasource for "Lipoamide-E"
71271view22:16, 16 October 2013MaintBotAutomated update of data sources
69020view17:49, 8 July 2013MaintBotUpdated to 2013 gpml schema
58689view18:30, 19 February 2013MaintBotModified categories
58688view18:29, 19 February 2013MaintBotModified title
58472view20:36, 13 February 2013Maguirre1FINAL v1.0
58471view20:31, 13 February 2013Maguirre1Periodical save, work in progress
58470view20:20, 13 February 2013Maguirre1Periodical save, work in progress
58469view20:10, 13 February 2013Maguirre1Periodical save, work in progress
58468view20:00, 13 February 2013Maguirre1Periodical save, work in progress
58467view19:50, 13 February 2013Maguirre1Periodical save, work in progress
58466view19:40, 13 February 2013Maguirre1Periodical save, work in progress
58465view19:30, 13 February 2013Maguirre1Periodical save, work in progress
58464view19:20, 13 February 2013Maguirre1Periodical save, work in progress
58463view19:10, 13 February 2013Maguirre1Periodical save, work in progress
58461view19:00, 13 February 2013Maguirre1Periodical save, work in progress
58460view18:49, 13 February 2013Maguirre1New pathway

External references


View all...
NameTypeDatabase referenceComment
(S)-MalateMetaboliteC00149 (KEGG Compound)
2-Hydroxy-ethyl- ThPPMetaboliteC05125 (KEGG Compound)
2-Oxo-glutarateMetaboliteC00026 (KEGG Compound)
3-carboxy-1- hydroxypropyl-ThPPMetabolite440649 (PubChem-compound)
ACLYGeneProductENSG00000131473 (Ensembl)
ACO1GeneProductENSG00000122729 (Ensembl)
Acetyl-CoAMetaboliteC00024 (KEGG Compound)
CSGeneProductENSG00000062485 (Ensembl)
CitrateMetaboliteC00158 (KEGG Compound)
DLATGeneProductENSG00000150768 (Ensembl)
DLDGeneProductENSG00000091140 (Ensembl)
DLSTGeneProductENSG00000119689 (Ensembl)
Dihydro- lipoamide-EMetaboliteCHEBI:80219 (ChEBI)
FHGeneProductENSG00000091483 (Ensembl)
FumarateMetaboliteC00122 (KEGG Compound)
GlycolysisPathwayWP534 (WikiPathways)
IDH1GeneProductENSG00000138413 (Ensembl)
IDH3AGeneProductENSG00000166411 (Ensembl)
IsocitrateMetaboliteC00311 (KEGG Compound)
Lipoamide-EMetaboliteC15972 (KEGG Compound)
MDH1GeneProductENSG00000014641 (Ensembl)
OGDHGeneProductENSG00000105953 (Ensembl)
OxaloacetateMetaboliteC00036 (KEGG Compound)
OxalosuccinateMetaboliteC05379 (KEGG Compound)
PCGeneProduct5091 (Entrez Gene)
PCK1GeneProductENSG00000124253 (Ensembl)
PDHA1GeneProductENSG00000131828 (Ensembl)
Phosphoenol- pyruvateMetaboliteC00074 (KEGG Compound)
PyruvateMetaboliteC00022 (KEGG Compound)
S-Acetyldihydro- lipoamide-EMetaboliteC16255 (KEGG Compound)
S-Succinyl- dihydrolipoamide-EMetaboliteC16254 (KEGG Compound)
SDHAGeneProductENSG00000073578 (Ensembl)
SUCLG2GeneProductENSG00000172340 (Ensembl)
SuccinateMetaboliteC00042 (KEGG Compound)
Succinoyl-CoAMetaboliteC00091 (KEGG Compound)
ThPPMetaboliteC00068 (KEGG Compound)

Annotated Interactions

No annotated interactions

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