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Schema
>
Reaction
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Entries
DLD dimer dehydrogenates dihydrolipoyl
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authored
[InstanceEdit:9859140] Stephan, Ralf, 2024-01-16
catalystActivity
[CatalystActivity:9859174] dihydrolipoyl dehydrogenase (NADH) activity of BCKDH [mitochondrial matrix]
category
transition
compartment
[Compartment:5460] mitochondrial matrix
created
[InstanceEdit:9859173] Stephan, Ralf, 2024-01-16
crossReference
[DatabaseIdentifier:9853985] RHEA:15046
dbId
9859172
diseaseReaction
[FailedReaction:R-HSA-9907572] Loss-of-function DLD mutants don't dehydrogenate dihydrolipoyl DBT - Homo sapiens
displayName
DLD dimer dehydrogenates dihydrolipoyl
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-9859148] BCKDHA:BCKDHB tetramer decarboxylates KIC, KMVA, KIV - Homo sapiens
input
[EntityWithAccessionedSequence:R-HSA-9859167] LipoH2-K105-DBT [mitochondrial matrix]
[SimpleEntity:R-ALL-113526] NAD+ [mitochondrial matrix]
isChimeric
false
isInDisease
false
isInferred
false
literatureReference
[LiteratureReference:450848] Crystal structure of human dihydrolipoamide dehydrogenase: NAD+/NADH binding and the structural basis of disease-causing mutations
[LiteratureReference:9853970] Human dihydrolipoamide dehydrogenase (E3) deficiency: Novel insights into the structural basis and molecular pathomechanism
[LiteratureReference:9853977] Formation of reactive oxygen species by human and bacterial pyruvate and 2-oxoglutarate dehydrogenase multienzyme complexes reconstituted from recombinant components
[LiteratureReference:9853975] Biochemical characterization of patients with dihydrolipoamide dehydrogenase deficiency
maxDepth
1
modified
[InstanceEdit:11108703] Weiser, Joel, 2026-03-16
name
DLD dimer dehydrogenates dihydrolipoyl
orthologousEvent
[Reaction:R-PFA-9859172] DLD dimer dehydrogenates dihydrolipoyl - Plasmodium falciparum
[Reaction:R-DDI-9859172] DLD dimer dehydrogenates dihydrolipoyl - Dictyostelium discoideum
[Reaction:R-CEL-9859172] DLD dimer dehydrogenates dihydrolipoyl - Caenorhabditis elegans
[Reaction:R-DME-9859172] DLD dimer dehydrogenates dihydrolipoyl - Drosophila melanogaster
[Reaction:R-GGA-9859172] DLD dimer dehydrogenates dihydrolipoyl - Gallus gallus
[Reaction:R-XTR-9859172] DLD dimer dehydrogenates dihydrolipoyl - Xenopus tropicalis
[Reaction:R-DRE-9859172] DLD dimer dehydrogenates dihydrolipoyl - Danio rerio
[Reaction:R-SSC-9859172] DLD dimer dehydrogenates dihydrolipoyl - Sus scrofa
[Reaction:R-BTA-9859172] DLD dimer dehydrogenates dihydrolipoyl - Bos taurus
[Reaction:R-CFA-9859172] DLD dimer dehydrogenates dihydrolipoyl - Canis familiaris
[Reaction:R-RNO-9859172] DLD dimer dehydrogenates dihydrolipoyl - Rattus norvegicus
[Reaction:R-MMU-9859172] DLD dimer dehydrogenates dihydrolipoyl - Mus musculus
output
[SimpleEntity:R-ALL-113529] H+ [mitochondrial matrix]
[EntityWithAccessionedSequence:R-HSA-70017] Lipo-K105-DBT [mitochondrial matrix]
[SimpleEntity:R-ALL-29362] NADH [mitochondrial matrix]
precedingEvent
[Reaction:R-HSA-9859163] DBT transfers BCAA to CoA - 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-9859172
summation
[Summation:9859254] Dihydrolipoyl dehydrogenase (DLD), the E3 component of the b...
Referrals
(normalReaction)
[FailedReaction:R-HSA-9907572] Loss-of-function DLD mutants don't dehydrogenate dihydrolipoyl DBT
(hasEvent)
[Pathway:R-HSA-9859138] BCKDH synthesizes BCAA-CoA from KIC, KMVA, KIV
(precedingEvent)
[Reaction:R-HSA-9859148] BCKDHA:BCKDHB tetramer decarboxylates KIC, KMVA, KIV
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