Ketogenesis and Ketolysis (Homo sapiens)

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3CapillaryGlial CellOuter mitochondrial membraneBlood Brain Barrier (BBB)MitochondrionInner mitochondrial membraneHepatocyteNeuronKetogenic DietFAOFatty acids (FA)BDH1OXCT1?CPT-1CATMRCATPMitochondrial LC-Fatty Acid Beta-Oxidation2BHB1acetoneacetoneACAACAACAAcetyl-CoAglucoseglucoseglucoseTCA (Krebs)cycleAcetoacetyl-CoAMCT-1UCP2pyruvatepyruvateAcetyl-CoAGLUT-14CO2OxaloacetateGluconeogenesisGlycolysisKetogenesisACAT1acetoneTCA (Krebs)cycleFABHBBHBBHBglucoseMitochondrionFA


This pathway depicts several metabolic pathways involved in ketogenic diet treatment.

In hepatocytes in the liver, fatty acids (FAs) are normally transformed into acetyl-CoA, which can then enters the TCA (Krebs) cycle for energy production. However, when FA levels are to high for the TCA cycle to be utilized completely, acetyl-CoA is used in ketogenesis and ketolysis.

These processes are a complex process of transporter proteins and several other (mitochondrial) pathways [1].

Quality Tags

Ontology Terms



  1. Newman JC, Verdin E; ''β-Hydroxybutyrate: A Signaling Metabolite.''; Annu Rev Nutr, 2017 PubMed Europe PMC
  2. Sharpe AJ, McKenzie M; ''Mitochondrial Fatty Acid Oxidation Disorders Associated with Short-Chain Enoyl-CoA Hydratase (ECHS1) Deficiency.''; Cells, 2018 PubMed Europe PMC
  3. Noebels JL, Avoli M, Rogawski MA, et al., editors. Bethesda (MD); ''Jasper's Basic Mechanisms of the Epilepsies [Internet]. 4th edition.''; National Center for Biotechnology Information (US);, 2012
  4. Olson AL, Pessin JE; ''Structure, function, and regulation of the mammalian facilitative glucose transporter gene family.''; Annu Rev Nutr, 1996 PubMed Europe PMC


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108452view13:56, 20 December 2019DeSlOntology Term : 'glial cell' added !
108451view13:55, 20 December 2019DeSlOntology Term : 'PW:0000002' removed !
108450view13:55, 20 December 2019DeSlOntology Term : 'ketone bodies degradation pathway' added !
108449view13:54, 20 December 2019DeSlOntology Term : 'ketone bodies metabolic pathway' added !
108448view13:54, 20 December 2019DeSlModified description
108447view13:49, 20 December 2019DeSlUpdated right side of PW.
108446view13:25, 20 December 2019DeSlUpdated middle part
108445view13:03, 20 December 2019DeSlStarted updating lefthand side of PW
107929view21:23, 11 November 2019Khanspersconnected interaction
107914view10:03, 10 November 2019EgonwMissed one sec ChEBI ID :(
107911view09:14, 10 November 2019EgonwBetter representation for mediated translocations.
107910view09:07, 10 November 2019EgonwFixed two interactions, which are not conversions.
107909view09:03, 10 November 2019EgonwReplaced secondary ChEBI identifiers with a primary identifiers.
107901view19:32, 9 November 2019RGrosmanCOntology Term : 'neuron' added !
107900view19:32, 9 November 2019RGrosmanCOntology Term : 'hepatocyte' added !
107899view19:31, 9 November 2019RGrosmanCOntology Term : 'classic metabolic pathway' added !
107898view19:30, 9 November 2019RGrosmanCNew pathway

External references


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NameTypeDatabase referenceComment
ACAMetaboliteCHEBI:15344 (ChEBI)
ACAT1ProteinP24752 (Uniprot-TrEMBL) mitochondrial acetoacetyl-CoA thiolase
ATPMetaboliteCHEBI:15422 (ChEBI)
Acetoacetyl -CoAMetaboliteCHEBI:15345 (ChEBI)
Acetyl-CoAMetaboliteCHEBI:15351 (ChEBI)
BDH1ProteinQ02338 (Uniprot-TrEMBL) AKA 3-hydroxybutyrate dehydrogenase
BHBMetaboliteCHEBI:37054 (ChEBI) BHB aka β-hydroxybutyrate
CATProteinO43772 (Uniprot-TrEMBL) CAT aka carnitine translocase
CO2MetaboliteCHEBI:16526 (ChEBI)
CPT-1ProteinP23786 (Uniprot-TrEMBL) CPT1 (mitochondrial isoform) aka carnitine palmitoyl transferase
FAOPathwayWP143 (WikiPathways)
FAMetaboliteQ61476 (Wikidata)
Fatty acids (FA)MetaboliteQ61476 (Wikidata)
GLUT-1ProteinP11166 (Uniprot-TrEMBL) Gene: SLC2A1
GluconeogenesisPathwayWP4740 (WikiPathways)
GlycolysisPathwayWP4277 (WikiPathways)
KetogenesisPathwayWP4741 (WikiPathways)
MCT-1ProteinP53985 (Uniprot-TrEMBL) Gene: SLC16A1
MRCPathwayWP111 (WikiPathways) MRC aka mitochondrial respiratory complex
Mitochondrial LC-Fatty Acid Beta-OxidationPathwayWP368 (WikiPathways)
OXCT1ProteinP55809 (Uniprot-TrEMBL) AKA succinyl-CoA-3-oxaloacid CoA transferase (SCOT)
OxaloacetateMetaboliteCHEBI:30744 (ChEBI)
TCA (Krebs) cyclePathwayWP78 (WikiPathways)
TCA (Krebs)cyclePathwayWP78 (WikiPathways)
UCP2ProteinP55851 (Uniprot-TrEMBL) Mitochondrial transporter proteins which creates proton leaks across the inner mitochondrial membrane, allowing uncoupling oxidative phosphorylation from ATP synthesis. As a result, energy is dissipated in the form of heat. Source []
acetoneMetaboliteCHEBI:15347 (ChEBI)
glucoseMetaboliteCHEBI:17234 (ChEBI)
pyruvateMetaboliteCHEBI:15361 (ChEBI)

Annotated Interactions

No annotated interactions

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