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Details on Person BCKDK phosphorylates BCKDH

Class:IdReaction:5693148
_displayNameBCKDK phosphorylates BCKDH
_doReleaseTRUE
_timestamp2024-11-08 15:22:44
authored[InstanceEdit:5693146] Jassal, Bijay, 2015-05-14
catalystActivity[CatalystActivity:5693164] protein kinase activity of BCKDK dimer [mitochondrial matrix]
compartment[Compartment:5460] mitochondrial matrix
created[InstanceEdit:5693146] Jassal, Bijay, 2015-05-14
crossReference[DatabaseIdentifier:9917120] RHEA:17302
edited[InstanceEdit:5693146] Jassal, Bijay, 2015-05-14
goBiologicalProcess[GO_BiologicalProcess:24669] negative regulation of amino acid metabolic process
input[Complex:70019] BCKDH [mitochondrial matrix] [Homo sapiens]
[SimpleEntity:113593] ATP [mitochondrial matrix]
[SimpleEntity:113593] ATP [mitochondrial matrix]
internalReviewed[InstanceEdit:9917225] D'Eustachio, Peter, 2024-08-01
isChimericFALSE
literatureReference[LiteratureReference:5693149] Cross-talk between thiamin diphosphate binding and phosphorylation loop conformation in human branched-chain alpha-keto acid decarboxylase/dehydrogenase
[LiteratureReference:5693118] Molecular mechanism for regulation of the human mitochondrial branched-chain alpha-ketoacid dehydrogenase complex by phosphorylation
[LiteratureReference:5693126] Mutations in BCKD-kinase lead to a potentially treatable form of autism with epilepsy
[LiteratureReference:5693166] Two novel mutations in the BCKDK (branched-chain keto-acid dehydrogenase kinase) gene are responsible for a neurobehavioral deficit in two pediatric unrelated patients
modified[InstanceEdit:6785238] Jassal, Bijay, 2015-06-26
[InstanceEdit:6785240] Jassal, Bijay, 2015-06-26
[InstanceEdit:6792911] Jassal, Bijay, 2015-08-28
[InstanceEdit:9672777] D'Eustachio, Peter, 2020-01-02
[InstanceEdit:9830342] Matthews, Lisa, 2023-03-08
[InstanceEdit:9908873] Stephan, Ralf, 2024-04-23
[InstanceEdit:9917131] Stephan, Ralf, 2024-07-30
[InstanceEdit:9919308] D'Eustachio, Peter, 2024-08-20
[InstanceEdit:9925948] D'Eustachio, Peter, 2024-10-28
[InstanceEdit:9927661] D'Eustachio, Peter, 2024-11-08
nameBCKDK phosphorylates BCKDH
ATP + L-seryl-(3-methyl-2-oxobutanoate dehydrogenase) => ADP + H(+) + O-phospho-L-seryl-(3-methyl-2-oxobutanoate dehydrogenase)
negativePrecedingEvent[NegativePrecedingEvent:9908874] Futile Cycle: PPM1K dephosphorylates p-BCKDH
output[Complex:5693120] p-BCKDH [mitochondrial matrix] [Homo sapiens]
[SimpleEntity:113581] ADP [mitochondrial matrix]
[SimpleEntity:113581] ADP [mitochondrial matrix]
[SimpleEntity:113529] H+ [mitochondrial matrix]
previousReviewStatus[ReviewStatus:9821385] four stars
releaseDate2015-09-22
releaseStatusNEW
reviewed[InstanceEdit:6785241] D'Eustachio, Peter, 2015-06-26
[InstanceEdit:9927660] D'Eustachio, Peter, 2024-11-08
reviewStatus[ReviewStatus:9821382] five stars
species[Species:48887] Homo sapiens
stableIdentifier[StableIdentifier:6796796] R-HSA-5693148.6
structureModified[InstanceEdit:9917131] Stephan, Ralf, 2024-07-30
summation[Summation:5693158] Mitochondrial 3-methyl-2-oxobutanoate dehydrogenase (lipoami...
(hasEvent)[Pathway:70895] Branched-chain amino acid catabolism [Homo sapiens]
(normalReaction)[FailedReaction:9912480] BCKDK loss-of-function mutations do not phosphorylate BCKDH [Homo sapiens]
(precedingEvent)[NegativePrecedingEvent:9908867] Futile Cycle: BCKDK phosphorylates BCKDH
(updatedInstance)[_UpdateTracker:9919415] Update Tracker - [Reaction:5693148] BCKDK phosphorylates BCKDH - v90:[removeOutput]
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