Leucine, isoleucine and valine metabolism (Homo sapiens)
From WikiPathways
Description
This pathway shows disorders related to leucine, isoleusine and valine metabolism. Disorders resulting from an enzyme defect are highlighted in pink.
This pathway was inspired by Chapter 7 of the book of Blau (ISBN 3642403360 (978-3642403361)).
Quality Tags
Ontology Terms
Saving...Bibliography
- Blau N, Duran M, Gibson KM, Dionisi-Vici, C; ''Physician's Guidee to the Diagnosis, Treatment, and Follow-Up of Inherited Metabolic Diseases''; Springer ISBN 3642403360, 2014
- Chu CH, Cheng D; ''Expression, purification, characterization of human 3-methylcrotonyl-CoA carboxylase (MCCC).''; Protein Expr Purif, 2007 PubMed Europe PMC Scholia
- Agnihotri G, Liu HW; ''Enoyl-CoA hydratase. reaction, mechanism, and inhibition.''; Bioorg Med Chem, 2003 PubMed Europe PMC Scholia
History
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External references
DataNodes
| View all... |
| Name | Type | Database reference | Comment |
|---|---|---|---|
| 2-Ethylhydracrylic acid | Metabolite | ||
| 2-Hydroxyisocaproate | Metabolite | ||
| 2-Hydroxyisovalerate | Metabolite | ||
| 2-Hyroxy-methylvalerate | Metabolite | ||
| 2-Methyl-3-hydroxy-butyrylcarnitine | Metabolite | ||
| 2-Methyl-3-hydroxybutyrate | Metabolite | ||
| 2-Methyl-3-hydroxybutyryl-carnitine | Metabolite | ||
| 2-Methylaceto-acetyl-CoA | Metabolite | ||
| 2-Methylacetoacetic acid | Metabolite | ||
| 2-Methylbulyryl-CoA | Metabolite | ||
| 2-Methylbutyrylcarnitine | Metabolite | ||
| 2-Methylbutyrylglycine | Metabolite | ||
| 2-Methzl-3-hydroxy-butyryl-CoA | Metabolite | ||
| 2-Oxisocaproate | Metabolite | ||
| 2-Oxo-3-methylvalerate | Metabolite | ||
| 2-Oxo-isovalerate | Metabolite | ||
| 2-Oxoisocaproate | Metabolite | ||
| 2-Oxoisovalerate | Metabolite | ||
| 3-Aminoisobutyric acid | Metabolite | ||
| 3-Hydroxy-3-methylglutarate | Metabolite | ||
| 3-Hydroxy-3-methylglutaryl-CoA | Metabolite | ||
| 3-Hydroxybutyrate | Metabolite | ||
| 3-Hydroxyhexanoyl-carnitine | Metabolite | ||
| 3-Hydroxyhexanoylcarnitine | Metabolite | ||
| 3-Hydroxyiso-butyryl-CoA | Metabolite | ||
| 3-Hydroxyisobutyrate | Metabolite | ||
| 3-Hydroxyisobutyrylcarnitine | Metabolite | ||
| 3-Hydroxyisovalerate | Metabolite | ||
| 3-Hydroxypropionate | Metabolite | ||
| 3-Hydroxypropionic acid | Metabolite | ||
| 3-Methyl-glutaconyl-CoA | Metabolite | ||
| 3-Methylcrotonyl-CoA | Metabolite | ||
| 3-Methylcrotonylglycine | Metabolite | ||
| 3-Methylglutaconate | Metabolite | ||
| 3-Methylglutarate | Metabolite | ||
| 3-Methylglutarylcarnitine | Metabolite | ||
| 4-Hydroxyisovaleric acid | Metabolite | ||
| ACSF3 | Protein | ||
| Acetoacetate | Metabolite | ||
| Acetyl-CoA | Metabolite | ||
| Aminotransferase | Protein | ||
| BCKDH | Protein | P11182 (Uniprot-TrEMBL)  | |
| Beta-alanine | Metabolite | ||
| Beta-ketothiolase | Protein | ||
| C5-Hydroxyacylcarnitine | Metabolite | ||
| C5-Hydroxyacylcyarnitine | Metabolite | ||
| DH | Protein | Q02252 (Uniprot-TrEMBL) | |
| DH | Protein | Q6NVY1 (Uniprot-TrEMBL) | |
| Deacylase | Protein | Q6NVY1 (Uniprot-TrEMBL) | |
| Ethylmalonic acid | Metabolite | ||
| HMG-CoA lyase | Protein | Q8TB92 (Uniprot-TrEMBL) | |
| Hydratase | Protein | ||
| Hydratase | Protein | Q13825 (Uniprot-TrEMBL) | |
| IBD | Protein | Q9UKU7 (Uniprot-TrEMBL) | |
| IVD | Protein | P26440 (Uniprot-TrEMBL) | |
| Isobutyryl-CoA | Metabolite | ||
| Isobutyrylcarnitine | Metabolite | ||
| Isobutyrylglycine | Metabolite | ||
| Isoleucine | Metabolite | ||
| Isovaleric acid | Metabolite | ||
| Isovaleryglucusonide | Metabolite | ||
| Isovalerylcarnitine | Metabolite | ||
| Isovalerylglycine | Metabolite | ||
| Isovalerzl-CoA | Metabolite | ||
| Ketone Bodies | Metabolite | ||
| L-Isoleucine | Metabolite | ||
| L-Leucine | Metabolite | ||
| L-Valine | Metabolite | ||
| Leucine | Metabolite | ||
| MBD | Protein | P45954 (Uniprot-TrEMBL) | |
| MCC | Protein | ||
| MHBD | Protein | Q99714 (Uniprot-TrEMBL) | |
| Malonic acid | Metabolite | ||
| Malonyl-CoA | Metabolite | ||
| Malonyl-CoA decarboxylase | Protein | O95822 (Uniprot-TrEMBL) | |
| Malonylcarnitine | Metabolite | ||
| Melonate/Methylmalonate | Metabolite | ||
| Methacrylyl-CoA | Metabolite | ||
| Methylmalonate | Metabolite | ||
| Methylmalonate semialdehyde | Metabolite | ||
| Methylmalonic acid | Metabolite | ||
| Methylmalonyl-CoA | Metabolite | ||
| Methylmalonylcarnitine | Metabolite | ||
| Mutase | Protein | P22033 (Uniprot-TrEMBL) | |
| PCC | Protein | ||
| Propionyl-CoA | Metabolite | ||
| Propionylcarnitine | Metabolite | ||
| Propionylglycine | Metabolite | ||
| R,S-Methylcitrate | Metabolite | ||
| S-2-carboxypropylcysteamine | Metabolite | ||
| S-2-carboxypropylcysteine | Metabolite | ||
| Succinyl-CoA | Metabolite | ||
| Tiglycarnitine | Metabolite | ||
| Tiglyl-CoA | Metabolite | ||
| Tiglylcarnitine | Metabolite | ||
| Tiglylglycine | Metabolite | ||
| Tiglylgycine | Metabolite | ||
| Valine | Metabolite |
Annotated Interactions
No annotated interactions
