Disorders in ketolysis (WP5195)

Homo sapiens

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The disorders of Ketolysis pathway described the molecular mechanism utilized under starvation or fasting conditions. This pathway was inspired by Chapter 23 of the book of Blau, edition 4 (ISBN 3642403360 (978-3642403361)), Figure 23.3. For the ketone body pathway see: WP5175

Authors

Sam Drabbe , Denise Slenter , Eric Weitz , and Egon Willighagen

Activity

last edited

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Organisms

Homo sapiens

Communities

Inherited Metabolic Disorders (IMD) Pathways Rare Diseases

Annotations

Pathway Ontology

classic metabolic pathway succinyl-CoA:3-oxoacid transferase deficiency pathway ketone bodies degradation pathway cholesterol biosynthetic pathway

Disease Ontology

beta-ketothiolase deficiency

Participants
Query Drugst.One

Label Type Compact URI Comment
HSCoA Metabolite chebi:15346
Succinyl-CoA Metabolite chebi:15380
Succinate Metabolite chebi:30031
Acetyl-CoA Metabolite hmdb:HMDB0001206
HMG-CoA Metabolite chebi:15467 referred to as β-Hydroxy-β-methylglutaryl-CoA in the book by Blau et al.
Acetoacetate Metabolite chebi:13705
3-hydroxybutyric acid Metabolite chebi:20067
Acetoacetyl-CoA Metabolite hmdb:HMDB0001484
MAT Protein uniprot:P24752 AKA Acetyl-CoA acetyltransferase
HMCS1 Protein uniprot:Q01581
BDH1 Protein uniprot:Q02338
SCOT1 Protein uniprot:P55809 aka OXCT1
CT Protein uniprot:Q9BWD1 AKA Acetyl-CoA acetyltransferase

References

  1. Enzymes of ketone body utilization in human tissues: protein and messenger RNA levels of succinyl-coenzyme A (CoA):3-ketoacid CoA transferase and mitochondrial and cytosolic acetoacetyl-CoA thiolases. Fukao T, Song XQ, Mitchell GA, Yamaguchi S, Sukegawa K, Orii T, et al. Pediatr Res. 1997 Oct;42(4):498–502. PubMed Europe PMC Scholia