Ketogenesis and ketolysis (WP4742)

Homo sapiens

Open in new tab Open in NDEx Open in Newt (SBGN)
Download PNG Download SVG Download JSON Download GPML Learn more about Downloads
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 [https://www.ncbi.nlm.nih.gov/books/NBK98219/figure/masino.f1/].

Authors

Raquel Grosman , Egon Willighagen , Kristina Hanspers , Denise Slenter , Eric Weitz , Finterly Hu , and Sam Drabbe

Activity

last edited

Discuss this pathway

Check for ongoing discussions or start your own.

Cited In

Are you planning to include this pathway in your next publication? See How to Cite and add a link here to your paper once it's online.

Organisms

Homo sapiens

Communities

Annotations

Cell Type Ontology

hepatocyte neuron glial cell

Pathway Ontology

ketone bodies metabolic pathway ketone bodies degradation pathway

Participants
Query Drugst.One

Label Type Compact URI Comment
ATP Metabolite chebi:15422
Fatty acids (FA) Metabolite wikidata:Q61476
BHB Metabolite chebi:37054 BHB aka β-hydroxybutyrate
acetone Metabolite chebi:15347
ACA Metabolite chebi:15344
ACA Metabolite chebi:15344 ACA aka Acetoacetate
Acetyl-CoA Metabolite chebi:15351
glucose Metabolite chebi:17234
Acetoacetyl-CoA Metabolite chebi:15345
pyruvate Metabolite chebi:15361
CO2 Metabolite chebi:16526
Oxaloacetate Metabolite chebi:30744
FA Metabolite wikidata:Q61476
CPT-1 Protein uniprot:P23786 CPT1 (mitochondrial isoform) aka carnitine palmitoyl transferase
OXCT1 Protein uniprot:P55809 AKA succinyl-CoA-3-oxaloacid CoA transferase (SCOT)
CAT Protein uniprot:O43772 CAT aka carnitine translocase
BDH1 Protein uniprot:Q02338 AKA 3-hydroxybutyrate dehydrogenase
MCT-1 Protein uniprot:P53985 Gene: SLC16A1
UCP2 Protein uniprot:P55851 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 [https://www.uniprot.org/uniprot/P55851]
GLUT-1 Protein uniprot:P11166 Gene: SLC2A1
ACAT1 Protein uniprot:P24752 mitochondrial acetoacetyl-CoA thiolase

References

  1. Jasper’s Basic Mechanisms of the Epilepsies [Internet]. Noebels JL, Avoli M. OUP USA; 2012. 1258 p. Available from: https://books.google.com/books/about/Jasper_s_Basic_Mechanisms_of_the_Epileps.html?hl=&id=T2_LVTB7ftgC OpenLibrary Worldcat
  2. Structure, function, and regulation of the mammalian facilitative glucose transporter gene family. Olson AL, Pessin JE. Annu Rev Nutr. 1996;16:235–56. PubMed Europe PMC Scholia
  3. β-Hydroxybutyrate: A Signaling Metabolite. Newman JC, Verdin E. Annu Rev Nutr. 2017 Aug 21;37:51–76. PubMed Europe PMC Scholia
  4. Mitochondrial Fatty Acid Oxidation Disorders Associated with Short-Chain Enoyl-CoA Hydratase (ECHS1) Deficiency. Sharpe AJ, McKenzie M. Cells. 2018 May 23;7(6):46. PubMed Europe PMC Scholia