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Proteins/UNIPROT:Q08426
Peroxisomal trifunctional enzyme possessing 2-enoyl-CoA hydratase, 3-hydroxyacyl-CoA dehydrogenase, and delta 3, delta 2-enoyl-CoA isomerase activities. Catalyzes two of the four reactions of the long chain fatty acids peroxisomal beta-oxidation pathway (By similarity). Can also use branched-chain fatty acids such as 2-methyl-2E-butenoyl-CoA as a substrate, which is hydrated into (2S,3S)-3-hydroxy-2-methylbutanoyl-CoA (By similarity). Optimal isomerase for 2,5 double bonds into 3,5 form isomerization in a range of enoyl-CoA species (Probable). Also able to isomerize both 3-cis and 3-trans double bonds into the 2-trans form in a range of enoyl-CoA species (By similarity). With HSD17B4, catalyzes the hydration of trans-2-enoyl-CoA and the dehydrogenation of 3-hydroxyacyl-CoA, but with opposite chiral specificity. Regulates the amount of medium-chain dicarboxylic fatty acids which are essential regulators of all fatty acid oxidation pathways (By similarity). Also involved in the degradation of long-chain dicarboxylic acids through peroxisomal beta-oxidation..
Function from UniProtKB (Q08426), read 2026-09-23. CC BY 4.0.
Curated compounds routed: 0
2 rows
214 statements
214 statements · 7 reference databases · Subject: EHHADH · Sources: Bgee, Hetionet, Human Protein Atlas, Human-GEM, Reactome, Rhea, WikiPathways
185 reactions
4CHEBI:1146900+4FAD+4water+4NAD(+)+palmitoyl-CoA4CHEBI:15351+4FADH2+4proton+4NADH(2-)+octanoyl-CoA
Catalysed by ACAA1 & HSD17B4 & ACOX1 or ACAA1 & EHHADH & ACOX1
8 names
Resolves to: EHHADH
Human-GEM MAR00759CC BY 4.0Reference data
CHEBI:1146900+FAD+water+NAD(+)+octadecanoyl-CoACHEBI:15351+FADH2+proton+NADH(2-)+palmitoyl-CoA
Catalysed by ACAA1 & HSD17B4 & ACOX1 or ACAA1 & EHHADH & ACOX1
Reaction pageLeft to rightBalancedEC 2.3.1.16peroxisome
Human-GEM MAR00777CC BY 4.0Reference data
5CHEBI:1146900+2FAD+(6Z,9Z,12Z)-octadecatrienoyl-CoA+5water+5NAD(+)5CHEBI:15351+2FADH2+5proton+5NADH(2-)+octanoyl-CoA
Catalysed by ACAA1 & HSD17B4 & ACOX1 or ACAA1 & EHHADH & ACOX1
Reaction pageLeft to rightBalancedEC 4.2.1.74peroxisome
Human-GEM MAR00800CC BY 4.0Reference data
(6Z,9Z,12Z,15Z)-octadecatetraenoyl-CoA+5CHEBI:1146900+FAD+5water+5NAD(+)5CHEBI:15351+FADH2+5proton+5NADH(2-)+octanoyl-CoA
Catalysed by ACAA1 & HSD17B4 & ACOX1 or ACAA1 & EHHADH & ACOX1
Reaction pageLeft to rightBalancedEC 4.2.1.74peroxisome
Human-GEM MAR00833CC BY 4.0Reference data
CHEBI:1146900+eicosanoyl-CoA+FAD+water+NAD(+)CHEBI:15351+FADH2+proton+NADH(2-)+octadecanoyl-CoA
Catalysed by ACAA1 & HSD17B4 & ACOX1 or ACAA1 & EHHADH & ACOX1
Reaction pageLeft to rightBalancedEC 4.2.1.74peroxisome
Human-GEM MAR00872CC BY 4.0Reference data
(5Z,8Z,11Z,14Z,17Z)-eicosapentaenoyl-CoA+CHEBI:1146900+water+NAD(+)(6Z,9Z,12Z,15Z)-octadecatetraenoyl-CoA+CHEBI:15351+proton+NADH(2-)
Catalysed by ACAA1 & EHHADH & ACOX1 or ACAA1 & HSD17B4 & ACOX1
Reaction pageLeft to rightBalancedEC 4.2.1.74peroxisome
Human-GEM MAR00927CC BY 4.0Reference data
CHEBI:63544+CHEBI:1146900+FAD+water+NAD(+)CHEBI:15351+arachidonoyl-CoA+FADH2+proton+NADH(2-)
Catalysed by ACAA1 & HSD17B4 & ACOX1 or ACAA1 & EHHADH & ACOX1
Reaction pageLeft to rightBalancedEC 4.2.1.74peroxisome
Human-GEM MAR00952CC BY 4.0Reference data
(4Z,7Z,10Z,13Z,16Z)-docosapentaenoyl-CoA+CHEBI:1146900+FAD+water+NAD(+)(5Z,8Z,11Z,14Z,17Z)-eicosapentaenoyl-CoA+CHEBI:15351+FADH2+proton+NADH(2-)
Catalysed by ACAA1 & HSD17B4 & ACOX1 or ACAA1 & EHHADH & ACOX1
Reaction pageLeft to rightBalancedEC 4.2.1.74peroxisome
Human-GEM MAR00968CC BY 4.0Reference data
(4Z,7Z,10Z,13Z,16Z,19Z)-docosahexaenoyl-CoA+CHEBI:1146900+water+NAD(+)(5Z,8Z,11Z,14Z,17Z)-eicosapentaenoyl-CoA+CHEBI:15351+proton+NADH(2-)
Catalysed by ACAA1 & HSD17B4 & ACOX1 or ACAA1 & EHHADH & ACOX1
Reaction pageLeft to rightBalancedEC 4.2.1.74peroxisome
Human-GEM MAR00972CC BY 4.0Reference data
2CHEBI:1146900+2FAD+2water+2NAD(+)+tetracosanoyl-CoA2CHEBI:15351+eicosanoyl-CoA+2FADH2+2proton+2NADH(2-)
Catalysed by ACAA1 & HSD17B4 & ACOX1 or ACAA1 & EHHADH & ACOX1
Reaction pageLeft to rightBalancedEC 4.2.1.74peroxisome
Human-GEM MAR00974CC BY 4.0Reference data
(15Z)-tetracosenoyl-CoA+3CHEBI:1146900+3FAD+3water+3NAD(+)3CHEBI:15351+3FADH2+3proton+3NADH(2-)+oleoyl-CoA
Catalysed by ACAA1 & HSD17B4 & ACOX1 or ACAA1 & EHHADH & ACOX1
Reaction pageLeft to rightBalancedEC 4.2.1.74peroxisome
Human-GEM MAR00975CC BY 4.0Reference data
MAM00131x+CHEBI:1146900+FAD+water+NAD(+)CHEBI:63544+CHEBI:15351+FADH2+proton+NADH(2-)
Catalysed by ACAA1 & HSD17B4 & ACOX1 or ACAA1 & EHHADH & ACOX1
Reaction pageLeft to rightBalancedEC 4.2.1.74peroxisome
Human-GEM MAR00977CC BY 4.0Reference data