Disorders in ketolysis (Homo sapiens)

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346Mitochondrial matrixCytosolMultistep processMethylacetoacetyl-CoA thiolase deficiencySuccinyl-CoA:3-oxoacid CoA transferase deficiencyCytosolic acetoacetyl-CoA thiolase deficiencyCholesterol synthesis215BDH1acetoacetateAcetyl-CoACTAcetoacetyl-CoA3-hydroxybutyric acidHMG-CoASCOT1HMCS1succinatesuccinyl-CoAMATHSCoAAcetyl-CoAAcetoacetyl-CoACholesterol synthesisdisordersDiseaseCatalysisPathwayStimulationProteinMetaboliteConversionConnection to diseaseLegend


Description

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)).

For the ketone body pathway see: WP5175

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Ontology Terms

 

Bibliography

  1. Blau, Nenad, Duran, Marinus, Gibson, K. Michael, Dionisi-Vici, Carlos; ''Physician's Guide to the Diagnosis, Treatment, and Follow-Up of Inherited Metabolic Diseases''; ISBN: 978-3-642-40337-8, 2014
  2. Fukao T, Song XQ, Mitchell GA, Yamaguchi S, Sukegawa K, Orii T, Kondo N; ''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.''; Pediatr Res, 1997 PubMed Europe PMC Scholia
  3. AMM, Morris; ''Inborn metabolic diseases''; ISBN: 978-3-642-15720-2, 2012

History

View all...
CompareRevisionActionTimeUserComment
128225view13:20, 29 January 2024EweitzSoften disease color, standardize case
122514view12:44, 13 April 2022DeSl
122434view11:36, 5 April 2022EweitzModified title
121868view21:26, 7 March 2022DeSlChanged colour in legend for disease connection
121867view21:26, 7 March 2022DeSlConverted all GeneProducts to Proteins (UniProt IDs)
121866view21:24, 7 March 2022DeSlUpdated HMDB IDs to new ID structure
121865view21:23, 7 March 2022DeSlRemoved links to OMIM in literature section
121864view21:21, 7 March 2022DeSlMoved cytosol
121863view21:18, 7 March 2022DeSlOntology Term : 'succinyl-CoA:3-oxoacid transferase deficiency pathway' added !
121862view21:17, 7 March 2022DeSlOntology Term : 'beta-ketothiolase deficiency' added !
121861view21:15, 7 March 2022DeSlOntology Term : 'cholesterol biosynthetic pathway' added !
121860view21:15, 7 March 2022DeSlOntology Term : 'classic metabolic pathway' added !
121859view21:15, 7 March 2022DeSlOntology Term : 'ketone bodies degradation pathway' added !
121858view21:13, 7 March 2022DeSlAdded legend, updated description
121857view21:06, 7 March 2022DeSlAdded reviewed UniProt ID for BDH1
121856view21:02, 7 March 2022DeSlConnected one disorder; connected formation of two Acetyl CoAs in reaction catalyzed by MAT
121855view20:57, 7 March 2022DeSllayout, added PW node IDs
121649view11:16, 23 February 2022SamDrabbeAdded diseases linked with Ketolysis
121643view10:08, 23 February 2022SamDrabbeadded literature
121641view10:04, 23 February 2022SamDrabbeadded Rhea annotation
121572view14:26, 21 February 2022SamDrabbeAdjusted some lines
121569view14:06, 21 February 2022SamDrabbeAdded extra pathway
121370view13:50, 16 February 2022SamDrabbeNew pathway

External references

DataNodes

View all...
NameTypeDatabase referenceComment
3-hydroxybutyric acidMetabolite20067 (ChEBI)
Acetoacetyl-CoAMetaboliteHMDB01484 (HMDB)
Acetyl-CoAMetaboliteHMDB01206 (HMDB)
BDH1ProteinQ02338 (Uniprot-TrEMBL)
CTGeneProductQ9BWD1 (Uniprot-TrEMBL) AKA Acetyl-CoA acetyltransferase
Cholesterol synthesis disordersPathwayWP5193 (WikiPathways)
Cholesterol synthesisPathwayWP197 (WikiPathways)
HMCS1GeneProductQ01581 (Uniprot-TrEMBL)
HMG-CoAMetaboliteCHEBI:15467 (ChEBI) referred to as β-Hydroxy-β-methylglutaryl-CoA in the book by Blau et al.
HSCoAMetaboliteCHEBI:15346 (ChEBI)
MATProteinP24752 (Uniprot-TrEMBL) AKA Acetyl-CoA acetyltransferase
SCOT1GeneProductP55809 (Uniprot-TrEMBL) aka OXCT1
acetoacetateMetaboliteCHEBI:13705 (ChEBI)
succinateMetaboliteCHEBI:30031 (ChEBI)
succinyl-CoAMetaboliteCHEBI:15380 (ChEBI)

Annotated Interactions

View all...
SourceTargetTypeDatabase referenceComment
Acetoacetyl-CoAAcetyl-CoAmim-conversion21039 (Rhea)
Acetoacetyl-CoAHMG-CoAmim-conversion24406 (Rhea)
Acetyl-CoAAcetoacetyl-CoAmim-conversion21037 (Rhea)
Acetyl-CoAmim-conversion21037 (Rhea)
acetoacetate3-hydroxybutyric acidmim-conversion20524 (Rhea)
acetoacetateAcetoacetyl-CoAmim-conversion25483 (Rhea)
mim-conversion21039 (Rhea)
Retrieved from "https://classic.wikipathways.org/index.php/Pathway:WP5195"
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