Reactome | DBT transfers BCAA to CoA

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DBT transfers BCAA to CoA Show undefined attributes

authored	[InstanceEdit:9859140] Stephan, Ralf, 2024-01-16
catalystActivity	[CatalystActivity:9859164] acyltransferase activity, transferring groups other than amino-acyl groups of BCKDH [mitochondrial matrix]
category	transition
compartment	[Compartment:5460] mitochondrial matrix
created	[InstanceEdit:9859165] Stephan, Ralf, 2024-01-16
crossReference	[DatabaseIdentifier:9859169] RHEA:18867
dbId	9859163
diseaseReaction	[FailedReaction:R-HSA-9865115] DBT loss-of-function mutants don't synthesize BCAA-CoA - Homo sapiens
displayName	DBT transfers BCAA to CoA
edited	[InstanceEdit:9910822] Stephan, Ralf, 2024-05-21
eventOf	[Pathway:R-HSA-9859138] BCKDH synthesizes BCAA-CoA from KIC, KMVA, KIV - Homo sapiens
followingEvent	[Reaction:R-HSA-9971621] GLYAT transfers ISB to glycine - Homo sapiens [Reaction:R-HSA-9971123] CRAT transfers ISB to CAR - Homo sapiens [Reaction:R-HSA-70745] isovaleryl-CoA + FAD => beta-methylcrotonyl-CoA + FADH2 - Homo sapiens [Reaction:R-HSA-70800] alpha-methylbutyryl-CoA + FAD => tiglyl-CoA + FADH2 - Homo sapiens [Reaction:R-HSA-70859] isobutyryl-CoA + FAD => methacrylyl-CoA + FADH2 - Homo sapiens [Reaction:R-HSA-9859172] DLD dimer dehydrogenates dihydrolipoyl - Homo sapiens
input	[DefinedSet:R-HSA-9859149] BCAA-lipoH2-K105-DBT [mitochondrial matrix] [SimpleEntity:R-ALL-29374] CoA-SH [mitochondrial matrix]
isChimeric	false
isInDisease	false
isInferred	false
literatureReference	[LiteratureReference:9859240] Genomic and biochemical analysis of repeatedly observed variants in DBT in individuals with maple syrup urine disease of Central American ancestry
maxDepth	1
modified	[InstanceEdit:11108703] Weiser, Joel, 2026-03-16
name	DBT transfers BCAA to CoA
negativePrecedingEvent	[NegativePrecedingEvent:9930372] beta-hydroxyisobutyryl-CoA + H2O => beta-hydroxyisobutyrate + CoA
orthologousEvent	[Reaction:R-PFA-9859163] DBT transfers BCAA to CoA - Plasmodium falciparum [Reaction:R-DDI-9859163] DBT transfers BCAA to CoA - Dictyostelium discoideum [Reaction:R-CEL-9859163] DBT transfers BCAA to CoA - Caenorhabditis elegans [Reaction:R-DME-9859163] DBT transfers BCAA to CoA - Drosophila melanogaster [Reaction:R-GGA-9859163] DBT transfers BCAA to CoA - Gallus gallus [Reaction:R-XTR-9859163] DBT transfers BCAA to CoA - Xenopus tropicalis [Reaction:R-DRE-9859163] DBT transfers BCAA to CoA - Danio rerio [Reaction:R-SSC-9859163] DBT transfers BCAA to CoA - Sus scrofa [Reaction:R-BTA-9859163] DBT transfers BCAA to CoA - Bos taurus [Reaction:R-CFA-9859163] DBT transfers BCAA to CoA - Canis familiaris [Reaction:R-RNO-9859163] DBT transfers BCAA to CoA - Rattus norvegicus [Reaction:R-MMU-9859163] DBT transfers BCAA to CoA - Mus musculus
output	[DefinedSet:R-ALL-508261] BCAA-CoAs [mitochondrial matrix] [EntityWithAccessionedSequence:R-HSA-9859167] LipoH2-K105-DBT [mitochondrial matrix]
precedingEvent	[Reaction:R-HSA-9859148] BCKDHA:BCKDHB tetramer decarboxylates KIC, KMVA, KIV - Homo sapiens [Reaction:R-HSA-5693153] PPM1K dephosphorylates p-BCKDH - Homo sapiens
releaseDate	2024-06-15
reviewStatus	[ReviewStatus:9821382] five stars
reviewed	[InstanceEdit:9907758] D'Eustachio, Peter, 2024-04-17
schemaClass	Reaction
species	[Species:48887] Homo sapiens
stId	R-HSA-9859163
summation	[Summation:9859239] Lipoamide acyltransferase E2 component (DBT) of branched-cha...

Referrals

(normalReaction)	[FailedReaction:R-HSA-9865115] DBT loss-of-function mutants don't synthesize BCAA-CoA
(precedingEvent)	[Reaction:R-HSA-9971621] GLYAT transfers ISB to glycine [Reaction:R-HSA-9971123] CRAT transfers ISB to CAR [Reaction:R-HSA-70745] isovaleryl-CoA + FAD => beta-methylcrotonyl-CoA + FADH2 [Reaction:R-HSA-70800] alpha-methylbutyryl-CoA + FAD => tiglyl-CoA + FADH2 [Reaction:R-HSA-70859] isobutyryl-CoA + FAD => methacrylyl-CoA + FADH2 [Reaction:R-HSA-9859172] DLD dimer dehydrogenates dihydrolipoyl
(hasEvent)	[Pathway:R-HSA-9859138] BCKDH synthesizes BCAA-CoA from KIC, KMVA, KIV

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