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Details on Person BCKDH [mitochondrial matrix]

Class:IdComplex:70019
_displayNameBCKDH [mitochondrial matrix]
_timestamp2024-01-16 10:12:02
compartment[Compartment:5460] mitochondrial matrix
hasComponent
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
modified[InstanceEdit:508257] D'Eustachio, P, 2010-02-11
[InstanceEdit:5693146] Jassal, Bijay, 2015-05-14
[InstanceEdit:6792676] Jassal, Bijay, 2015-08-25
[InstanceEdit:9859160] Stephan, Ralf, 2024-01-16
nameBCKDH
lipoylated branched-chain alpha-ketoacid dehydrogenase complex
species[Species:48887] Homo sapiens
stableIdentifier[StableIdentifier:363801] R-HSA-70019.3
(input)[Reaction:5693148] BCKDK phosphorylates BCKDH [Homo sapiens]
[FailedReaction:9912480] BCKDK loss-of-function mutations do not phosphorylate BCKDH [Homo sapiens]
(normalEntity)[EntityFunctionalStatus:9865103] loss_of_function of DBT mutant-BCKDH [mitochondrial matrix]
[EntityFunctionalStatus:9865105] loss_of_function of BCKDH mutant tetramer [mitochondrial matrix]
[EntityFunctionalStatus:9907589] loss_of_function of DLD mutant-BCKDH [mitochondrial matrix]
(output)[Reaction:5693153] PPM1K dephosphorylates p-BCKDH [Homo sapiens]
(physicalEntity)[CatalystActivity:70029] CoA-transferase 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]
[CatalystActivity:9859174] dihydrolipoyl dehydrogenase (NADH) activity of BCKDH [mitochondrial matrix]
(replacementInstances)[_Deleted:508266] Deletion of instances: 70057, 70053, 70049, 70045, 70041, 70039, 70036, 70032
(updatedInstance)[_UpdateTracker:9869002] Update Tracker - [Complex:70019] BCKDH [mitochondrial matrix] - v32:[add_removeHasComponent]
[_UpdateTracker:9892410] Update Tracker - [Complex:70019] BCKDH [mitochondrial matrix] - v53:[addName]
[_UpdateTracker:9892997] Update Tracker - [Complex:70019] BCKDH [mitochondrial matrix] - v54:[modifyName, updateContainedComponent]
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No pathways have been reviewed or authored by BCKDH [mitochondrial matrix] (70019)
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