Disorders in ketolysis (WP5195)

Homo sapiens

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: [https://www.wikipathways.org/index.php/Pathway:WP5175 WP5175]


Sam Drabbe , Denise Slenter , and Eric Weitz


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Homo sapiens


Inherited Metabolic Disorders (IMD) Pathways Rare Diseases


Disease Ontology

beta-ketothiolase deficiency

Pathway Ontology

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


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
Acetyl-CoA Metabolite hmdb:HMDB0001206
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


  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