Loading the page.
Proteins/UNIPROT:Q96HY7
2-oxoadipate dehydrogenase (E1a) component of the 2-oxoadipate dehydrogenase complex (OADHC). Participates in the first step, rate limiting for the overall conversion of 2-oxoadipate (alpha-ketoadipate) to glutaryl-CoA and CO(2) catalyzed by the whole OADHC. Catalyzes the irreversible decarboxylation of 2-oxoadipate via the thiamine diphosphate (ThDP) cofactor and subsequent transfer of the decarboxylated acyl intermediate on an oxidized dihydrolipoyl group that is covalently amidated to the E2 enzyme (dihydrolipoyllysine-residue succinyltransferase or DLST) (Probable). Can catalyze the decarboxylation of 2-oxoglutarate in vitro, but at a much lower rate than 2-oxoadipate. Responsible for the last step of L-lysine, L-hydroxylysine and L-tryptophan catabolism with the common product being 2-oxoadipate (Probable)..
Function from UniProtKB (Q96HY7), read 2026-09-23. CC BY 4.0.
Curated compounds routed: 0
2 rows
109 statements
109 statements · 7 reference databases · Subject: DHTKD1 · Sources: Hetionet, Human Protein Atlas, Human-GEM, Reactome, Rhea, UniProt, WikiPathways
5 reactions
2-oxoglutarate(2-)+proton+thiamine(1+) diphosphateMAM00765m+carbon dioxide
Reaction pageLeft to rightBalancedEC 1.2.4.2mitochondrial matrix
Human-GEM MAR04209CC BY 4.0Reference data
MAM02393m+MAM20077mMAM02899m+thiamine(1+) diphosphate
9 names
Resolves to: DHTKD1
Reaction pageLeft to rightBalance undeterminableEC 1.2.4.2mitochondrial matrix
Human-GEM MAR04599CC BY 4.0Reference data
MAM00765m+MAM02393mMAM02934m+thiamine(1+) diphosphate
Reaction pageLeft to rightBalancedEC 1.2.4.2mitochondrial matrix
Human-GEM MAR06413CC BY 4.0Reference data
2-oxoadipate+proton+thiamine(1+) diphosphatecarbon dioxide+MAM20077m
Reaction pageLeft to rightBalance undeterminableEC 1.2.4.-mitochondrial matrix
Human-GEM MAR20173CC BY 4.0Reference data
N(6)-[(R)-lipoyl]-L-lysyl-[protein]+2-oxoadipate+hydronN(6)-[(R)-S(8)-glutaryldihydrolipoyl]-L-lysyl-[protein]+carbon dioxide
Catalysed by DHTKD1
Reaction pageLeft to rightBalance undeterminable
Rhea RHEA:69576CC BY 4.0Reference data