Reactome | BCKDH [mitochondrial matrix]

Schema > Complex > Entries

BCKDH [mitochondrial matrix] Show undefined attributes

catalystActivities	[CatalystActivity:9859174] dihydrolipoyl dehydrogenase (NADH) activity of BCKDH [mitochondrial matrix] [CatalystActivity:9859137] oxidoreductase activity, acting on the aldehyde or oxo group of donors, disulfide as acceptor of BCKDH [mitochondrial matrix] [CatalystActivity:9859164] acyltransferase activity, transferring groups other than amino-acyl groups of BCKDH [mitochondrial matrix]
compartment	[Compartment:5460] mitochondrial matrix
consumedByEvent	[Reaction:R-HSA-5693148] BCKDK phosphorylates BCKDH - Homo sapiens [FailedReaction:R-HSA-9912480] BCKDK loss-of-function mutations do not phosphorylate BCKDH - Homo sapiens
dbId	70019
deleted	[Deleted:508266] Deletion of instances: 70057, 70053, 70049, 70045, 70041, 70039, 70036, 70032
displayName	BCKDH [mitochondrial matrix]
hasComponent	12 × [Complex:R-HSA-70016] BCKDHA:BCKDHB tetramer [mitochondrial matrix] 6 × [Complex:R-HSA-5694020] DLD dimer:2xFAD [mitochondrial matrix] 24 × [EntityWithAccessionedSequence:R-HSA-70017] Lipo-K105-DBT [mitochondrial matrix]
inDisease	false
inferredTo	[Complex:R-MMU-70019] BCKDH [mitochondrial matrix] - Mus musculus [Complex:R-RNO-70019] BCKDH [mitochondrial matrix] - Rattus norvegicus [Complex:R-CFA-70019] BCKDH [mitochondrial matrix] - Canis familiaris [Complex:R-BTA-70019] BCKDH [mitochondrial matrix] - Bos taurus [Complex:R-SSC-70019] BCKDH [mitochondrial matrix] - Sus scrofa [Complex:R-DRE-70019] BCKDH [mitochondrial matrix] - Danio rerio [Complex:R-XTR-70019] BCKDH [mitochondrial matrix] - Xenopus tropicalis [Complex:R-GGA-70019] BCKDH [mitochondrial matrix] - Gallus gallus [Complex:R-DME-70019] BCKDH [mitochondrial matrix] - Drosophila melanogaster [Complex:R-CEL-70019] BCKDH [mitochondrial matrix] - Caenorhabditis elegans [Complex:R-DDI-70019] BCKDH [mitochondrial matrix] - Dictyostelium discoideum [Complex:R-PFA-70019] BCKDH [mitochondrial matrix] - Plasmodium falciparum
literatureReference	[LiteratureReference:70020] Crystal structure of human branched-chain alpha-ketoacid dehydrogenase and the molecular basis of multienzyme complex deficiency in maple syrup urine disease. [Book:70027] The Metabolic and Molecular Bases of Inherited Disease, 8th ed Scriver, CR Maple syrup urine disease (branched-chain ketoaciduria) 0079130356 [LiteratureReference:69983] Structure-function relationships in dihydrolipoamide acyltransferases
maxDepth	3
modified	[InstanceEdit:9859160] Stephan, Ralf, 2024-01-16
name	BCKDH lipoylated branched-chain alpha-ketoacid dehydrogenase complex
producedByEvent	[Reaction:R-HSA-5693153] PPM1K dephosphorylates p-BCKDH - Homo sapiens
schemaClass	Complex
species	[Species:48887] Homo sapiens
stId	R-HSA-70019
updateTrackers	[UpdateTracker:9869002] Update Tracker - [Complex:70019] BCKDH [mitochondrial matrix] - v32:[add_removeHasComponent] [UpdateTracker:9892997] Update Tracker - [Complex:70019] BCKDH [mitochondrial matrix] - v54:[modifyName, updateContainedComponent] [UpdateTracker:9892410] Update Tracker - [Complex:70019] BCKDH [mitochondrial matrix] - v53:[addName]

Referrals

(updatedInstance)	[UpdateTracker:9869002] Update Tracker - [Complex:70019] BCKDH [mitochondrial matrix] - v32:[add_removeHasComponent] [UpdateTracker:9892997] Update Tracker - [Complex:70019] BCKDH [mitochondrial matrix] - v54:[modifyName, updateContainedComponent] [UpdateTracker:9892410] Update Tracker - [Complex:70019] BCKDH [mitochondrial matrix] - v53:[addName]
(replacementInstances)	[Deleted:508266] Deletion of instances: 70057, 70053, 70049, 70045, 70041, 70039, 70036, 70032
(normalEntity)	[EntityFunctionalStatus:9865103] loss_of_function of DBT mutant-BCKDH [mitochondrial matrix] [EntityFunctionalStatus:9907589] loss_of_function of DLD mutant-BCKDH [mitochondrial matrix] [EntityFunctionalStatus:9865105] loss_of_function of BCKDH mutant tetramer [mitochondrial matrix]
(input)	[FailedReaction:R-HSA-9912480] BCKDK loss-of-function mutations do not phosphorylate BCKDH [Reaction:R-HSA-5693148] BCKDK phosphorylates BCKDH
(physicalEntity)	[CatalystActivity:9859174] dihydrolipoyl dehydrogenase (NADH) activity of BCKDH [mitochondrial matrix] [CatalystActivity:9859137] oxidoreductase activity, acting on the aldehyde or oxo group of donors, disulfide as acceptor of BCKDH [mitochondrial matrix] [CatalystActivity:9859164] acyltransferase activity, transferring groups other than amino-acyl groups of BCKDH [mitochondrial matrix]
(output)	[Reaction:R-HSA-5693153] PPM1K dephosphorylates p-BCKDH

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