Leucine, isoleucine and valine metabolism (Homo sapiens)

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1BCAA aminotransferase deficencyBCKDHMethacrylic acid conjugatesMethylmalonateIsoleucineE1 (alpha)ValineMalonic acidAminotransferaseAminotransferase2-HydroxyisovalerateS-2-carboxypropylcysteineS-2-carboxypropylcysteamineMalonyl-CoAL-LeucineAcetoacetate3-HydroxyisobutyrateL-Valine2-OxoisovalerateMelonate/MethylmalonateHMG-CoA lyaseAminotransferaseACSF3E3 (aka DLD)3-HydroxybutyrateIsobutyrylcarnitine3-HydroxyisobutyrylcarnitineIsobutyrylglycineMethylmalonyl-CoAMalonyl-CoA decarboxylase2-OxisocaproateE2 (aka DBT)2-OxoisocaproateL-Isoleucine2-HydroxyisovalerateIsovalerzl-CoATPP3-Methylcrotonyl-CoA3-Methyl-glutaconyl-CoAHydrataseMCCIVD3-Hydroxy-3-methylglutaryl-CoAMethylcrotonylglycinuriaIsovaleric acidemiaMaple syrup urine diseaseMethylglutaconic aciduria type IV3-Hydroxy-3-methylglutaric aciduriaBCAA aminotransferase deficency2-Methyl-3-hydroxybutyryl-CoA dehydrogenase deficiency2-MethylbutyrylglycinuriaMaple syrup urine diseaseAlpha-methylacetoacetic aciduria2-Methylaceto-acetyl-CoA2-Methzl-3-hydroxy-butyryl-CoATiglyl-CoA2-Methylbulyryl-CoA2-Oxo-3-methylvalerateAcetyl-CoAMHBDHydrataseMBDBeta-ketothiolaseBCAA aminotransferase deficencyMaple syrup urine diseaseDeacylaseHydrataseIBDMethacrylyl-CoAIsobutyryl-CoA2-Oxo-isovalerateSuccinyl-CoAPropionyl-CoAMethylmalonate semialdehyde3-Hydroxyisobutyrate3-Hydroxyiso-butyryl-CoAPCCDHDHMutase3-Hydroxyisobutyryl-CoAdecylase deficencyIsobutyryl-CoAdehydrogensae deficency3-Hydroxyisobutyratedehydrogenase deficiencyMethylmalonate semialdehyde dehydrogenase deficiencyPropionic acidemiaMethylmalonic academiaCombined MMA and MAMalonic aciduria2-Hydroxyisocaproate3-HydroxyisovalerateLeucineIsovalerylglycineIsovaleryglucusonideIsovaleric acid4-Hydroxyisovaleric acidIsovalerylcarnitine3-Hydroxyisovalerate3-MethylcrotonylglycineC5-Hydroxyacylcyarnitine3-Methylglutaconate3-Methylglutarylcarnitine3-HydroxyisovalerateC5-Hydroxyacylcarnitine3-Methylglutarate3-Methylglutaconate3-Hydroxyisovalerate3-Hydroxyhexanoylcarnitine3-MethylglutarateC5-Hydroxyacylcarnitine3-Hydroxy-3-methylglutarate3-Methylcrotonylglycine3-Hydroxyhexanoyl-carnitineKetone BodiesMalonic acidEthylmalonic acidMethylmalonic acidMalonylcarnitine2-Oxo-3-methylvalerate2-Methylbutyrylcarnitine2-Hyroxy-methylvalerate2-Methylbutyrylglycine2-Ethylhydracrylic acidTiglylglycineTiglylgycineTiglylglycine2-Methylacetoacetic acid2-Methyl-3-hydroxybutyrate2-Methyl-3-hydroxybutyrateTiglylcarnitineTiglycarnitine2-Methyl-3-hydroxybutyryl-carnitine2-Methyl-3-hydroxy-butyrylcarnitine2-Hydroxyisovalerate3-Hydroxyisobutyrylcarnitine3-Aminoisobutyric acid3-Hydroxyisobutyrate3-Hydroxypropionic acidEthylmalonic acidBeta-alanine3-HydroxypropionatePropionylglycineTiglylglycinePropionylcarnitineR,S-MethylcitrateR,S-Methylcitrate3-HydroxypropionatePropionylcarnitineMethylmalonylcarnitineMethylmalonic acidMalonylcarnitineE1 (beta)TPPBCKDHE2 (aka DBT)E3 (aka DLD)E1 (alpha)TPPE1 (beta)TPPBCKDHE2 (aka DBT)E3 (aka DLD)E1 (alpha)TPPE1 (beta)TPP


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)).

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Quality Tags

Ontology Terms

 

Bibliography

  1. 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
  2. Chu CH, Cheng D; ''Expression, purification, characterization of human 3-methylcrotonyl-CoA carboxylase (MCCC).''; Protein Expr Purif, 2007 PubMed Europe PMC Scholia
  3. Agnihotri G, Liu HW; ''Enoyl-CoA hydratase. reaction, mechanism, and inhibition.''; Bioorg Med Chem, 2003 PubMed Europe PMC Scholia

History

View all...
CompareRevisionActionTimeUserComment
128283view01:25, 30 January 2024EweitzSoften disease color, use standard font weight
122622view08:44, 22 April 2022DeSlAnnotated most side metabolites/biomarkers
122618view08:00, 22 April 2022DeSlAnnotated righthand side fully
122617view07:42, 22 April 2022DeSlAnnotated most of righthand side main reactions
122615view15:30, 21 April 2022DeSlAnnotated all remaining proteins
122614view15:23, 21 April 2022DeSlFinished annotating middle section
122613view15:00, 21 April 2022DeSlAdded three additional subtypes for Maple syrup urine disease
122612view13:40, 21 April 2022DeSlMore layout updates
122611view13:33, 21 April 2022DeSlFixing layout, connectiond to disorders, replacing complexes with groups for Metabolites
122610view13:11, 21 April 2022DeSlStarting to annotate middle section
122605view09:28, 21 April 2022DeSlUpdate description
122059view13:37, 9 March 2022SamDrabbeketone body synthesis and ketolysis pathway added
119122view08:55, 18 June 2021FinterlyAdded ISBN for book citation
117776view13:59, 22 May 2021EweitzModified title
108034view14:58, 25 November 2019L DupuisConnected unconnected line
106457view10:09, 30 August 2019DeSlAdded last biomarker IDs on left.
106456view10:08, 30 August 2019DeSlAdded IDs for biomarkers on left hand side of PW.
106379view15:18, 23 August 2019DeSlAdded biotin (needed for MCC functioning).
106378view15:15, 23 August 2019DeSlFinished annotating main metabolites left hand side.
106377view14:31, 23 August 2019DeSlAdded more metabolite IDs for starting compounds
106376view14:27, 23 August 2019DeSlAdded subunits for BCKDH complex, started adding metabolite IDs + rhea interaction IDs.
105884view07:26, 16 August 2019AkutmonAdded Protein Identifiers
105883view07:11, 16 August 2019AkutmonAdded Protein identifiers
105739view11:13, 15 August 2019AkutmonUpdated Metabolic Groups
105738view10:07, 15 August 2019AkutmonAdded Metabolite Groups
105737view09:41, 15 August 2019AkutmonAdded Metabolite Groups
105736view09:04, 15 August 2019AkutmonAdded Metabolite Groups
105735view07:59, 15 August 2019AkutmonAdded Metabolite Groups
105734view07:49, 15 August 2019Akutmon
105733view07:29, 15 August 2019Akutmon
105732view07:28, 15 August 2019Akutmon
105726view15:33, 14 August 2019DeSlOntology Term : 'methylmalonic acidemia pathway' added !
105725view15:33, 14 August 2019DeSlOntology Term : 'methylmalonic acidemia' added !
105724view15:33, 14 August 2019DeSlOntology Term : 'propionic acidemia pathway' added !
105723view15:32, 14 August 2019DeSlOntology Term : 'propionic acidemia' added !
105722view15:32, 14 August 2019DeSlOntology Term : 'methylmalonate semialdehyde dehydrogenase deficiency pathway' added !
105721view15:30, 14 August 2019DeSlOntology Term : 'isobutyryl-CoA dehydrogenase deficiency pathway' added !
105720view15:29, 14 August 2019DeSlOntology Term : 'malonic aciduria pathway' added !
105719view15:27, 14 August 2019DeSlOntology Term : '3-hydroxy-3-methylglutaryl-CoA lyase deficiency pathway' added !
105718view15:26, 14 August 2019DeSlOntology Term : '3-methylglutaconic aciduria type 4' added !
105717view15:24, 14 August 2019DeSlOntology Term : '3-Methylcrotonyl-CoA carboxylase deficiency' added !
105716view15:24, 14 August 2019DeSlOntology Term : 'isovaleric acidemia pathway' added !
105715view15:24, 14 August 2019DeSlOntology Term : 'isovaleric acidemia' added !
105714view15:23, 14 August 2019DeSlOntology Term : 'maple syrup urine disease pathway' added !
105713view15:23, 14 August 2019DeSlOntology Term : 'maple syrup urine disease' added !
105712view14:47, 14 August 2019AkutmonOntology Term : 'valine metabolic pathway' added !
105711view14:46, 14 August 2019AkutmonOntology Term : 'isoleucine metabolic pathway' added !
105710view14:46, 14 August 2019AkutmonOntology Term : 'leucine metabolic pathway' added !
105709view14:46, 14 August 2019AkutmonOntology Term : 'inherited metabolic disorder' added !
105708view14:45, 14 August 2019AkutmonNew pathway

External references

DataNodes

View all...
NameTypeDatabase referenceComment
2-Ethylhydracrylic acidMetabolite
2-HydroxyisocaproateMetabolite
2-HydroxyisovalerateMetabolite
2-Hyroxy-methylvalerateMetabolite
2-Methyl-3-hydroxy-butyrylcarnitineMetabolite
2-Methyl-3-hydroxybutyrateMetabolite
2-Methyl-3-hydroxybutyryl-carnitineMetabolite
2-Methylaceto-acetyl-CoAMetabolite
2-Methylacetoacetic acidMetabolite
2-Methylbulyryl-CoAMetabolite
2-MethylbutyrylcarnitineMetabolite
2-MethylbutyrylglycineMetabolite
2-Methzl-3-hydroxy-butyryl-CoAMetabolite
2-OxisocaproateMetabolite
2-Oxo-3-methylvalerateMetabolite
2-Oxo-isovalerateMetaboliteCHEBI:11851 (ChEBI)
2-OxoisocaproateMetabolite
2-OxoisovalerateMetaboliteCHEBI:11851 (ChEBI)
3-Aminoisobutyric acidMetabolite
3-Hydroxy-3-methylglutarateMetabolite
3-Hydroxy-3-methylglutaryl-CoAMetabolite
3-HydroxybutyrateMetabolite
3-Hydroxyhexanoyl-carnitineMetabolite
3-HydroxyhexanoylcarnitineMetabolite
3-Hydroxyiso-butyryl-CoAMetabolite
3-HydroxyisobutyrateMetabolite
3-HydroxyisobutyrylcarnitineMetabolite
3-HydroxyisovalerateMetabolite
3-HydroxypropionateMetabolite
3-Hydroxypropionic acidMetabolite
3-Methyl-glutaconyl-CoAMetabolite
3-Methylcrotonyl-CoAMetabolite
3-MethylcrotonylglycineMetabolite
3-MethylglutaconateMetabolite
3-MethylglutarateMetabolite
3-MethylglutarylcarnitineMetabolite
4-Hydroxyisovaleric acidMetabolite
ACSF3Protein
AcetoacetateMetabolite
Acetyl-CoAMetabolite
AminotransferaseProtein
Beta-alanineMetabolite
Beta-ketothiolaseProtein
C5-HydroxyacylcarnitineMetabolite
C5-HydroxyacylcyarnitineMetabolite
DHProteinQ02252 (Uniprot-TrEMBL)
DHProteinQ6NVY1 (Uniprot-TrEMBL)
DeacylaseProteinQ6NVY1 (Uniprot-TrEMBL)
E1 (alpha)ProteinP12694 (Uniprot-TrEMBL) E1 uses thiamine pyrophosphate (TPP) as a catalytic cofactor.
E1 (beta)ProteinP21953 (Uniprot-TrEMBL) E1 uses thiamine pyrophosphate (TPP) as a catalytic cofactor.
E2 (aka DBT)ProteinP11182 (Uniprot-TrEMBL)
E3 (aka DLD)ProteinP09622 (Uniprot-TrEMBL)
Ethylmalonic acidMetabolite
HMG-CoA lyaseProteinQ8TB92 (Uniprot-TrEMBL)
HydrataseProtein
HydrataseProteinQ13825 (Uniprot-TrEMBL)
IBDProteinQ9UKU7 (Uniprot-TrEMBL)
IVDProteinP26440 (Uniprot-TrEMBL)
Isobutyryl-CoAMetaboliteCHEBI:15479 (ChEBI)
IsobutyrylcarnitineMetabolite
IsobutyrylglycineMetabolite
IsoleucineMetaboliteCHEBI:24898 (ChEBI)
Isovaleric acidMetabolite
IsovaleryglucusonideMetabolite
IsovalerylcarnitineMetabolite
IsovalerylglycineMetabolite
Isovalerzl-CoAMetabolite
Ketone BodiesMetabolite
L-IsoleucineMetaboliteCHEBI:17191 (ChEBI)
L-LeucineMetaboliteCHEBI:15603 (ChEBI)
L-ValineMetaboliteCHEBI:57762 (ChEBI)
LeucineMetaboliteCHEBI:25017 (ChEBI)
MBDProteinP45954 (Uniprot-TrEMBL)
MCCProtein
MHBDProteinQ99714 (Uniprot-TrEMBL)
Malonic acidMetabolite
Malonyl-CoAMetabolite
Malonyl-CoA decarboxylaseProteinO95822 (Uniprot-TrEMBL)
MalonylcarnitineMetabolite
Melonate/MethylmalonateMetabolite
Methacrylyl-CoAMetabolite
MethylmalonateMetabolite
Methylmalonate semialdehydeMetabolite
Methylmalonic acidMetabolite
Methylmalonyl-CoAMetabolite
MethylmalonylcarnitineMetabolite
MutaseProteinP22033 (Uniprot-TrEMBL)
PCCProtein
Propionyl-CoAMetabolite
PropionylcarnitineMetabolite
PropionylglycineMetabolite
R,S-MethylcitrateMetabolite
S-2-carboxypropylcysteamineMetabolite
S-2-carboxypropylcysteineMetabolite
Succinyl-CoAMetabolite
TiglycarnitineMetabolite
Tiglyl-CoAMetabolite
TiglylcarnitineMetabolite
TiglylglycineMetabolite
TiglylgycineMetabolite
ValineMetaboliteCHEBI:27266 (ChEBI)

Annotated Interactions

SourceTargetTypeDatabase referenceComment
2-Oxo-isovalerateIsobutyryl-CoAmim-conversion13458 (Rhea)
L-Valine2-Oxo-isovaleratemim-conversion22913 (Rhea)
Retrieved from "https://classic.wikipathways.org/index.php/Pathway:WP4686"
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