Reactome | Branched-chain amino acid catabolism

Schema > Pathway > Entries

Branched-chain amino acid catabolism Show undefined attributes

authored	[InstanceEdit:73677] D'Eustachio, P, 2003-06-24 00:00:00
dbId	70895
deleted	[Deleted:508389] Deletion of instance: 70845 [Deleted:508314] Deletion of instance: 70793 [Deleted:508478] Deletion of instance: 70894
diseasePathways	[Pathway:R-HSA-9915355] Beta-ketothiolase deficiency - Homo sapiens [Pathway:R-HSA-9865113] Loss-of-function mutations in DBT cause MSUD2 - Homo sapiens [Pathway:R-HSA-9912529] H139Hfs13* PPM1K causes a mild variant of MSUD - Homo sapiens [Pathway:R-HSA-9907570] Loss-of-function mutations in DLD cause MSUD3/DLDD - Homo sapiens [Pathway:R-HSA-9865125] Loss-of-function mutations in BCKDHA or BCKDHB cause MSUD - Homo sapiens [Pathway:R-HSA-9909438] 3-Methylcrotonyl-CoA carboxylase deficiency - Homo sapiens [Pathway:R-HSA-9916720] Mitochondrial short-chain enoyl-CoA hydratase deficiency 1 - Homo sapiens [Pathway:R-HSA-9912481] Branched-chain ketoacid dehydrogenase kinase deficiency - Homo sapiens [Pathway:R-HSA-9916722] 3-hydroxyisobutyryl-CoA hydrolase deficiency - Homo sapiens [Pathway:R-HSA-9914274] 3-methylglutaconic aciduria - Homo sapiens [Pathway:R-HSA-9914355] Isovaleric acidemia - Homo sapiens
displayName	Branched-chain amino acid catabolism
doi	10.3180/REACT_197.2
edited	[InstanceEdit:165080] D'Eustachio, P, 0000-00-00 00:00:00
eventOf	[Pathway:R-HSA-71291] Metabolism of amino acids and derivatives - Homo sapiens
goBiologicalProcess	[GO_BiologicalProcess:19354] branched-chain amino acid catabolic process
hasDiagram	true
hasEHLD	false
hasEvent	[Reaction:R-HSA-70723] leu, ile, or val + alpha-ketoglutarate <=> a-ketoisocaproate, a-keto-b-methylvalerate, or a-ketoisovalerate + glutamate [BCAT1] - Homo sapiens [Reaction:R-HSA-508189] a-ketoisocaproate, a-keto-b-methylvalerate, or a-ketoisovalerate + glutamate <=> leu, ile, or val + alpha-ketoglutarate [BCAT1] - Homo sapiens [Reaction:R-HSA-9672770] SLC25A44 transports Leu, Ile and Val from cytosol to mitochondrial matrix - Homo sapiens [Reaction:R-HSA-70724] leu, ile, or val + alpha-ketoglutarate <=> a-ketoisocaproate, a-keto-b-methylvalerate, or a-ketoisovalerate + glutamate [BCAT2] - Homo sapiens [Reaction:R-HSA-508179] a-ketoisocaproate, a-keto-b-methylvalerate, or a-ketoisovalerate + glutamate <=> leu, ile, or val + alpha-ketoglutarate [BCAT2] - Homo sapiens [Pathway:R-HSA-9859138] BCKDH synthesizes BCAA-CoA from KIC, KMVA, KIV - Homo sapiens [Reaction:R-HSA-5693148] BCKDK phosphorylates BCKDH - Homo sapiens [Reaction:R-HSA-9971123] CRAT transfers ISB to CAR - Homo sapiens [Reaction:R-HSA-9971621] GLYAT transfers ISB to glycine - Homo sapiens [Reaction:R-HSA-5693153] PPM1K dephosphorylates p-BCKDH - Homo sapiens [Reaction:R-HSA-70745] isovaleryl-CoA + FAD => beta-methylcrotonyl-CoA + FADH2 - Homo sapiens [Reaction:R-HSA-70773] beta-methylcrotonyl-CoA + ATP + HCO3- <=> beta-methylglutaconyl-CoA + ADP + orthophosphate + H2O (MCCA) - Homo sapiens [Reaction:R-HSA-508308] beta-methylglutaconyl-CoA + ADP + orthophosphate <=> beta-methylcrotonyl-CoA + ATP + HCO3- (MCCA) - Homo sapiens [Reaction:R-HSA-70785] beta-methylglutaconyl-CoA + H2O <=> beta-hydroxy-beta-methylglutaryl-CoA - Homo sapiens [Reaction:R-HSA-70800] alpha-methylbutyryl-CoA + FAD => tiglyl-CoA + FADH2 - Homo sapiens [Reaction:R-HSA-70830] tiglyl-CoA + H2O <=> alpha-methyl-beta-hydroxybutyryl-CoA - Homo sapiens [Reaction:R-HSA-70837] alpha-methyl-beta-hydroxybutyryl-CoA + NAD+ <=> alpha-methylacetoacetyl-CoA + NADH + H+ - Homo sapiens [Reaction:R-HSA-508369] alpha-methylacetoacetyl-CoA + NADH + H+ <=> alpha-methyl-beta-hydroxybutyryl-CoA + NAD+ - Homo sapiens [Reaction:R-HSA-70844] alpha-methylacetoacetyl-CoA + CoA => propionyl-CoA + acetyl-CoA - Homo sapiens [Reaction:R-HSA-70859] isobutyryl-CoA + FAD => methacrylyl-CoA + FADH2 - Homo sapiens [Reaction:R-HSA-70870] ECHS1 hydrates methacrylyl-CoA - Homo sapiens [Reaction:R-HSA-70881] beta-hydroxyisobutyryl-CoA + H2O => beta-hydroxyisobutyrate + CoA - Homo sapiens [Reaction:R-HSA-70885] beta-hydroxyisobutyrate + NAD+ <=> methylmalonyl semialdehyde + NADH + H+ - Homo sapiens [Reaction:R-HSA-508473] methylmalonyl semialdehyde + NADH + H+ <=> beta-hydroxyisobutyrate + NAD+ - Homo sapiens [Reaction:R-HSA-70893] methylmalonate semialdehyde + NAD+ + CoA + H2O => propionyl-CoA + HCO3- + NADH + H+ - Homo sapiens
isInDisease	false
isInferred	false
lastUpdatedDate	2020-03-16
literatureReference	[LiteratureReference:9672787] Branched Chain Amino Acids
maxDepth	3
modified	[InstanceEdit:11108703] Weiser, Joel, 2026-03-16
name	Branched-chain amino acid catabolism
orthologousEvent	[Pathway:R-DRE-70895] Branched-chain amino acid catabolism - Danio rerio [Pathway:R-MMU-70895] Branched-chain amino acid catabolism - Mus musculus [Pathway:R-RNO-70895] Branched-chain amino acid catabolism - Rattus norvegicus [Pathway:R-CFA-70895] Branched-chain amino acid catabolism - Canis familiaris [Pathway:R-BTA-70895] Branched-chain amino acid catabolism - Bos taurus [Pathway:R-SSC-70895] Branched-chain amino acid catabolism - Sus scrofa [Pathway:R-XTR-70895] Branched-chain amino acid catabolism - Xenopus tropicalis [Pathway:R-GGA-70895] Branched-chain amino acid catabolism - Gallus gallus [Pathway:R-DME-70895] Branched-chain amino acid catabolism - Drosophila melanogaster [Pathway:R-CEL-70895] Branched-chain amino acid catabolism - Caenorhabditis elegans [Pathway:R-DDI-70895] Branched-chain amino acid catabolism - Dictyostelium discoideum [Pathway:R-SPO-70895] Branched-chain amino acid catabolism - Schizosaccharomyces pombe [Pathway:R-SCE-70895] Branched-chain amino acid catabolism - Saccharomyces cerevisiae [Pathway:R-PFA-70895] Branched-chain amino acid catabolism - Plasmodium falciparum
previousReviewStatus	[ReviewStatus:9821384] two stars
releaseDate	2003-05-04
releaseStatus	UPDATED
reviewStatus	[ReviewStatus:9821382] five stars
reviewed	[InstanceEdit:9907758] D'Eustachio, Peter, 2024-04-17 [InstanceEdit:9983649] D'Eustachio, Peter, 2026-02-25
revised	[InstanceEdit:517475] D'Eustachio, P, 2010-02-18 [InstanceEdit:9672919] D'Eustachio, Peter, 2020-01-03
schemaClass	Pathway
species	[Species:48887] Homo sapiens
stId	R-HSA-70895
summation	[Summation:70896] The branched-chain amino acids, leucine, isoleucine, and val...
updateTrackers	[UpdateTracker:9777771] Update Tracker - [Pathway:70895] Branched-chain amino acid catabolism - v69:[removeHasEvent] [UpdateTracker:9784558] Update Tracker - [Pathway:70895] Branched-chain amino acid catabolism - v53:[updateContainedRLE] [UpdateTracker:9911052] Update Tracker - [Pathway:70895] Branched-chain amino acid catabolism - v89:[add_removeHasEvent] [UpdateTracker:9790343] Update Tracker - [Pathway:70895] Branched-chain amino acid catabolism - v33:[updateContainedRLE] [UpdateTracker:9777390] Update Tracker - [Pathway:70895] Branched-chain amino acid catabolism - v72:[addHasEvent, add_removeLiteratureReference, modifyText, updateContainedRLE] [UpdateTracker:9781772] Update Tracker - [Pathway:70895] Branched-chain amino acid catabolism - v60:[updateContainedPathway, updateIndirectRLE] [UpdateTracker:9984626] Update Tracker - [Pathway:70895] Branched-chain amino acid catabolism - v96:[removeHasEvent] [UpdateTracker:9864326] Update Tracker - [Pathway:70895] Branched-chain amino acid catabolism - v88:[updateContainedRLE] [UpdateTracker:9784102] Update Tracker - [Pathway:70895] Branched-chain amino acid catabolism - v54:[addHasEvent] [UpdateTracker:9788740] Update Tracker - [Pathway:70895] Branched-chain amino acid catabolism - v40:[updateContainedRLE] [UpdateTracker:9789921] Update Tracker - [Pathway:70895] Branched-chain amino acid catabolism - v36:[updateContainedRLE] [UpdateTracker:9791109] Update Tracker - [Pathway:70895] Branched-chain amino acid catabolism - v32:[addLiteratureReference, add_removeHasEvent, modifyText, updateContainedRLE]

Referrals

(hasEvent)	[Pathway:R-HSA-71291] Metabolism of amino acids and derivatives
(hasEncapsulatedEvent)	[Pathway:R-HSA-9857492] Protein lipoylation
(normalPathway)	[Pathway:R-HSA-9915355] Beta-ketothiolase deficiency [Pathway:R-HSA-9865113] Loss-of-function mutations in DBT cause MSUD2 [Pathway:R-HSA-9912529] H139Hfs13* PPM1K causes a mild variant of MSUD [Pathway:R-HSA-9907570] Loss-of-function mutations in DLD cause MSUD3/DLDD [Pathway:R-HSA-9865125] Loss-of-function mutations in BCKDHA or BCKDHB cause MSUD [Pathway:R-HSA-9909438] 3-Methylcrotonyl-CoA carboxylase deficiency [Pathway:R-HSA-9916720] Mitochondrial short-chain enoyl-CoA hydratase deficiency 1 [Pathway:R-HSA-9912481] Branched-chain ketoacid dehydrogenase kinase deficiency [Pathway:R-HSA-9916722] 3-hydroxyisobutyryl-CoA hydrolase deficiency [Pathway:R-HSA-9914274] 3-methylglutaconic aciduria [Pathway:R-HSA-9914355] Isovaleric acidemia
(replacementInstances)	[Deleted:508389] Deletion of instance: 70845 [Deleted:508314] Deletion of instance: 70793 [Deleted:508478] Deletion of instance: 70894
(updatedInstance)	[UpdateTracker:9777771] Update Tracker - [Pathway:70895] Branched-chain amino acid catabolism - v69:[removeHasEvent] [UpdateTracker:9784558] Update Tracker - [Pathway:70895] Branched-chain amino acid catabolism - v53:[updateContainedRLE] [UpdateTracker:9911052] Update Tracker - [Pathway:70895] Branched-chain amino acid catabolism - v89:[add_removeHasEvent] [UpdateTracker:9790343] Update Tracker - [Pathway:70895] Branched-chain amino acid catabolism - v33:[updateContainedRLE] [UpdateTracker:9777390] Update Tracker - [Pathway:70895] Branched-chain amino acid catabolism - v72:[addHasEvent, add_removeLiteratureReference, modifyText, updateContainedRLE] [UpdateTracker:9781772] Update Tracker - [Pathway:70895] Branched-chain amino acid catabolism - v60:[updateContainedPathway, updateIndirectRLE] [UpdateTracker:9984626] Update Tracker - [Pathway:70895] Branched-chain amino acid catabolism - v96:[removeHasEvent] [UpdateTracker:9864326] Update Tracker - [Pathway:70895] Branched-chain amino acid catabolism - v88:[updateContainedRLE] [UpdateTracker:9784102] Update Tracker - [Pathway:70895] Branched-chain amino acid catabolism - v54:[addHasEvent] [UpdateTracker:9788740] Update Tracker - [Pathway:70895] Branched-chain amino acid catabolism - v40:[updateContainedRLE] [UpdateTracker:9789921] Update Tracker - [Pathway:70895] Branched-chain amino acid catabolism - v36:[updateContainedRLE] [UpdateTracker:9791109] Update Tracker - [Pathway:70895] Branched-chain amino acid catabolism - v32:[addLiteratureReference, add_removeHasEvent, modifyText, updateContainedRLE]

Cite Us!