Disorders in ketolysis (Homo sapiens)
From WikiPathways
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: WP5175Quality Tags
Ontology Terms
Bibliography
- 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
- 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
- AMM, Morris; ''Inborn metabolic diseases''; ISBN: 978-3-642-15720-2, 2012
History
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External references
DataNodes
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Name | Type | Database reference | Comment |
---|---|---|---|
3-hydroxybutyric acid | Metabolite | 20067 (ChEBI) | |
Acetoacetyl-CoA | Metabolite | HMDB0001484 (HMDB) | |
Acetyl-CoA | Metabolite | HMDB0001206 (HMDB) | |
BDH1 | Protein | Q02338 (Uniprot-TrEMBL) | |
CT | GeneProduct | Q9BWD1 (Uniprot-TrEMBL) | AKA Acetyl-CoA acetyltransferase |
Cholesterol synthesis disorders | Pathway | WP5193 (WikiPathways) | |
Cholesterol synthesis | Pathway | WP197 (WikiPathways) | |
HMCS1 | GeneProduct | Q01581 (Uniprot-TrEMBL) | |
HMG-CoA | Metabolite | CHEBI:15467 (ChEBI) | referred to as β-Hydroxy-β-methylglutaryl-CoA in the book by Blau et al. |
HSCoA | Metabolite | CHEBI:15346 (ChEBI) | |
MAT | Protein | P24752 (Uniprot-TrEMBL) | AKA Acetyl-CoA acetyltransferase |
SCOT1 | GeneProduct | P55809 (Uniprot-TrEMBL) | aka OXCT1 |
acetoacetate | Metabolite | CHEBI:13705 (ChEBI) | |
succinate | Metabolite | CHEBI:30031 (ChEBI) | |
succinyl-CoA | Metabolite | CHEBI:15380 (ChEBI) |
Annotated Interactions
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Source | Target | Type | Database reference | Comment |
---|---|---|---|---|
Acetoacetyl-CoA | Acetyl-CoA | mim-conversion | 21039 (Rhea) | |
Acetoacetyl-CoA | HMG-CoA | mim-conversion | 24406 (Rhea) | |
Acetyl-CoA | Acetoacetyl-CoA | mim-conversion | 21037 (Rhea) | |
Acetyl-CoA | mim-conversion | 21037 (Rhea) | ||
acetoacetate | 3-hydroxybutyric acid | mim-conversion | 20524 (Rhea) | |
acetoacetate | Acetoacetyl-CoA | mim-conversion | 25483 (Rhea) | |
mim-conversion | 21039 (Rhea) |