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Details on Person DHTKD1 dimer decarboxylates 2-OA

Class:Id	Reaction:9858321
_displayName	DHTKD1 dimer decarboxylates 2-OA
_doRelease	TRUE
_timestamp	2024-11-22 22:10:58
authored	[InstanceEdit:9858341] Stephan, Ralf, 2024-01-12
catalystActivity	[CatalystActivity:9853486] oxidoreductase activity, acting on the aldehyde or oxo group of donors, disulfide as acceptor of OADH complex [mitochondrial matrix]
compartment	[Compartment:5460] mitochondrial matrix
created	[InstanceEdit:9858341] Stephan, Ralf, 2024-01-12
crossReference	[DatabaseIdentifier:9858325] RHEA:69577
edited	[InstanceEdit:9910822] Stephan, Ralf, 2024-05-21
input	[SimpleEntity:113529] H+ [mitochondrial matrix] [EntityWithAccessionedSequence:69993] Lipo-K110-DLST [mitochondrial matrix] [Homo sapiens] [SimpleEntity:70951] 2-OA [mitochondrial matrix]
literatureReference	[LiteratureReference:9858330] The mitochondrial 2-oxoadipate and 2-oxoglutarate dehydrogenase complexes share their E2 and E3 components for their function and both generate reactive oxygen species [LiteratureReference:9858322] Crystal structure and interaction studies of human DHTKD1 provide insight into a mitochondrial megacomplex in lysine catabolism [LiteratureReference:9859035] Inhibition and Crystal Structure of the Human DHTKD1-Thiamin Diphosphate Complex [LiteratureReference:9859016] A nonsense mutation in DHTKD1 causes Charcot-Marie-Tooth disease type 2 in a large Chinese pedigree [LiteratureReference:9859042] Genetic basis of alpha-aminoadipic and alpha-ketoadipic aciduria [LiteratureReference:9859026] Heterozygous DHTKD1 Variants in Two European Cohorts of Amyotrophic Lateral Sclerosis Patients [LiteratureReference:9859047] A novel DHTKD1 gene mutation with ALS like presentation: a case report
modified	[InstanceEdit:9858583] Stephan, Ralf, 2024-01-13 [InstanceEdit:9858593] Stephan, Ralf, 2024-01-13 [InstanceEdit:9858756] Stephan, Ralf, 2024-01-14 [InstanceEdit:9859030] Stephan, Ralf, 2024-01-15 [InstanceEdit:9859601] Stephan, Ralf, 2024-01-22 [InstanceEdit:9907757] Stephan, Ralf, 2024-04-17 [InstanceEdit:9909098] Stephan, Ralf, 2024-04-26 [InstanceEdit:9910823] Stephan, Ralf, 2024-05-21 [InstanceEdit:9929230] D'Eustachio, Peter, 2024-11-22
name	DHTKD1 dimer decarboxylates 2-OA 2-oxoadipate + H(+) + N(6)-((R)-lipoyl)-L-lysyl-(dihydrolipoyllysine-residue succinyltransferase) => CO2 + N(6)-((R)-S(8)-glutaryldihydrolipoyl)-L-lysyl-(dihydrolipoyllysine-residue succinyltransferase)
output	[SimpleEntity:29376] CO2 [mitochondrial matrix] [EntityWithAccessionedSequence:9858581] GlutaryllipoH2-K110-DLST [mitochondrial matrix] [Homo sapiens]
precedingEvent	[Reaction:372480] 2-oxoglutarate [mitochondrial matrix] + 2-oxoadipate [cytosol] <=> 2-oxoglutarate [cytosol] + 2-oxoadipate [mitochondrial matrix] [Homo sapiens] [Reaction:70613] GOT2 transaminates OA and L-Glu [Homo sapiens] [Reaction:508561] alpha-ketoadipate + glutamate <=> alpha-aminoadipate + alpha-ketoglutarate [Homo sapiens] [Reaction:70952] alpha-aminoadipate + alpha-ketoglutarate <=> alpha-ketoadipate + glutamate [Homo sapiens] [Reaction:70941] alpha-aminoadipoate semialdehyde + NAD+ => alpha-aminoadipate + NADH + H+ [Homo sapiens] [Reaction:9858589] DLD dimer dehydrogenates dihydrolipoyl [Homo sapiens] [Reaction:9858590] DLST transfers glutaryl to CoA [Homo sapiens]
releaseDate	2024-06-15
reviewed	[InstanceEdit:9907756] D'Eustachio, Peter, 2024-04-17
reviewStatus	[ReviewStatus:9821382] five stars
species	[Species:48887] Homo sapiens
stableIdentifier	[StableIdentifier:9858343] R-HSA-9858321.2
structureModified	[InstanceEdit:9858583] Stephan, Ralf, 2024-01-13
summation	[Summation:9859068] 2-Oxoadipate dehydrogenase complex (OADC) component E1 (DHTK...
(hasEvent)	[Pathway:9858328] OADH complex synthesizes glutaryl-CoA from 2-OA [Homo sapiens]
(precedingEvent)	[Reaction:9858590] DLST transfers glutaryl to CoA [Homo sapiens]
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No pathways have been reviewed or authored by DHTKD1 dimer decarboxylates 2-OA (9858321)