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Proteins/UNIPROT:Q13011
Auxiliary enzyme in the beta-oxidation of mono- and polyunsaturated fatty acids. Together with the 2,4-dienoyl-CoA reductase (DECR) and the Enoyl-CoA delta isomerase 1 (ECI1) they allow reentrance of the enoyl-CoA into the beta-oxidation cycle. Able to isomerize 3-trans,5-cis-dienoyl-CoA (3E,5Z-dienoyl-CoA) to 2-trans,4-trans-dienoyl-CoA (2E,4E-dienoyl-CoA), a crucial step in the beta-oxidation of unsaturated fatty acids with double bonds at odd position (By similarity). Based on its established catalytic mechanism, it is predicted to act also on other fatty acids besides those tested experimentally (Probable)..
Function from UniProtKB (Q13011), read 2026-09-23. CC BY 4.0.
Curated compounds routed: 0
264 statements
264 statements · 4 reference databases · Subject: ECH1 · Sources: Hetionet, Human-GEM, Reactome, WikiPathways
16 reactions
(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 ACADL & HADHA & ECI1 & HADHB & ECH1 & DECR1 or ECI2 & ACADL & HADHA & HADHB &
3 names
Resolves to: ECH1
Reaction pageLeft to rightBalancedEC 4.2.1.74mitochondrial matrix
Human-GEM MAR00970CC BY 4.0Reference data
(2E)-dodecenoyl-CoA(3Z)-dodecenoyl-CoA
Catalysed by ECH1 or ECI1 or ECI2
Reaction pageReversibleBalancedEC 5.3.3.8, 5.3.3.-mitochondrial matrix
Human-GEM MAR03239CC BY 4.0Reference data
(2E)-dodecenoyl-CoA(5Z)-dodecenoyl-CoA
Catalysed by ECH1 or ECI1 or ECI2
Reaction pageReversibleBalancedEC 5.3.3.8, 5.3.3.-mitochondrial matrix
Human-GEM MAR03272CC BY 4.0Reference data
MAM01575mMAM03021m
Catalysed by ECH1 or ECI1 or ECI2
Reaction pageLeft to rightBalancedEC 5.3.3.8, 5.3.3.-mitochondrial matrix
Human-GEM MAR03288CC BY 4.0Reference data
MAM03358mMAM03359m
Catalysed by ECH1
Reaction pageReversibleBalancedEC 2.3.1.16mitochondrial matrix
Human-GEM MAR03376CC BY 4.0Reference data
MAM03034m(2E,8Z,11Z,14Z)-eicosatetraenoyl-CoA
Catalysed by ECH1 or ECI1 or ECI2
Reaction pageLeft to rightBalancedEC 5.3.3.8, 5.3.3.-mitochondrial matrix
Human-GEM MAR03447CC BY 4.0Reference data
water+MAM03031xMAM00773x
Reaction pageLeft to rightBalancedEC 5.3.3.-, 5.3.3.8peroxisome
Human-GEM MAR09566CC BY 4.0Reference data
MAM01579x+waterMAM00773x
Reaction pageLeft to rightBalancedEC 4.2.1.17peroxisome
Human-GEM MAR09568CC BY 4.0Reference data
FAD+water+hexanedioyl-CoAFADH2+MAM03213x
Catalysed by EHHADH & ACOX1 or ECH1 & ACOX1
Reaction pageLeft to rightBalancedEC 1.3.3.6, 4.2.1.17peroxisome
Human-GEM MAR11529CC BY 4.0Reference data
FAD+water+decanedioyl-CoAFADH2+MAM03259x
Catalysed by EHHADH & ACOX1 or ECH1 & ACOX1
Reaction pageLeft to rightBalancedEC 1.3.3.6, 4.2.1.17peroxisome
Human-GEM MAR11534CC BY 4.0Reference data
CHEBI:85090CHEBI:85091
Catalysed by ECH1
Reaction pageLeft to rightBalanced
Rhea RHEA:45224CC BY 4.0Reference data
CHEBI:85110CHEBI:85111
Catalysed by ECH1
Reaction pageLeft to rightBalance undeterminable
Rhea RHEA:45240CC BY 4.0Reference data