Reactome | BCKDK phosphorylates BCKDH

Schema > Reaction > Entries

BCKDK phosphorylates BCKDH Show undefined attributes

authored	[InstanceEdit:5693146] Jassal, Bijay, 2015-05-14
catalystActivity	[CatalystActivity:5693164] [3-methyl-2-oxobutanoate dehydrogenase (acetyl-transferring)] kinase activity of BCKDK [mitochondrial matrix]
category	transition
compartment	[Compartment:5460] mitochondrial matrix
created	[InstanceEdit:5693146] Jassal, Bijay, 2015-05-14
dbId	5693148
displayName	BCKDK phosphorylates BCKDH
edited	[InstanceEdit:5693146] Jassal, Bijay, 2015-05-14
eventOf	[Pathway:R-HSA-70895] Branched-chain amino acid catabolism - Homo sapiens
goBiologicalProcess	[GO_BiologicalProcess:24669] negative regulation of cellular amino acid metabolic process
input	2 × [SimpleEntity:R-ALL-113593] ATP [mitochondrial matrix] [Complex:R-HSA-70019] BCKDH [mitochondrial matrix]
isChimeric	false
isInDisease	false
isInferred	false
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:10486564] Wright, Adam, 2024-03-07
name	BCKDK phosphorylates BCKDH
orthologousEvent	[Reaction:R-BTA-5693148] BCKDK phosphorylates BCKDH - Bos taurus [Reaction:R-XTR-5693148] BCKDK phosphorylates BCKDH - Xenopus tropicalis [Reaction:R-SSC-5693148] BCKDK phosphorylates BCKDH - Sus scrofa [Reaction:R-CFA-5693148] BCKDK phosphorylates BCKDH - Canis familiaris [Reaction:R-RNO-5693148] BCKDK phosphorylates BCKDH - Rattus norvegicus [Reaction:R-MMU-5693148] BCKDK phosphorylates BCKDH - Mus musculus
output	2 × [SimpleEntity:R-ALL-113581] ADP [mitochondrial matrix] [Complex:R-HSA-5693120] p-BCKDH [mitochondrial matrix]
releaseDate	2015-09-22
reviewStatus	[ReviewStatus:9821382] five stars
reviewed	[InstanceEdit:6785241] D'Eustachio, Peter, 2015-06-26
schemaClass	Reaction
species	[Species:48887] Homo sapiens
stId	R-HSA-5693148
summation	[Summation:5693158] Mitochondrial 3-methyl-2-oxobutanoate dehydrogenase (lipoami...

Referrals

(hasEvent)

[Pathway:R-HSA-70895] Branched-chain amino acid catabolism