Proteins/UNIPROT:Q4G176

ACSF3

ProteinUNIPROT:Q4G176Malonate--CoA ligase ACSF3, mitochondrial

Catalyzes the initial reaction in intramitochondrial fatty acid synthesis, by activating malonate and methylmalonate, but not acetate, into their respective CoA thioester. May have some preference toward very-long-chain substrates..

Function from UniProtKB (Q4G176), read 2026-09-23. CC BY 4.0.

Curated compounds routed: 0

What the reference databases state

GenCC submitters' classifications

1 row

combined malonic and methylmalonic acidemia has a germline basis in ACSF3DiseaseGenCC HGNC:27288|MONDO:0013661Inferred only

Statements

32 statements

32 statements · 5 reference databases · Subject: ACSF3 · Sources: Hetionet, Human-GEM, Reactome, Rhea, WikiPathways

Order

ACSF3 covaries with DUSP28ProteinHetionet hetionet:covaries:197322-285193Inferred only
ACSF3 covaries with MVDProteinHetionet hetionet:covaries:197322-4597Inferred only
PIEZO1 covaries with ACSF3ProteinHetionet hetionet:covaries:197322-9780Inferred only
UBC interacts with ACSF3ProteinHetionet hetionet:interacts:197322-7316Inferred only
ACSF3 participates in Fatty acid metabolismPathwayReactome R-HSA-8978868Inferred only
ACSF3 participates in Fatty acyl-CoA biosynthesisPathwayReactome R-HSA-75105Inferred only
ACSF3 participates in Leucine isoleucine and valine metabolismPathwayWikiPathways WP4686Inferred only
ACSF3 participates in MetabolismPathwayReactome R-HSA-1430728Inferred only
ACSF3 participates in Metabolism of lipidsPathwayReactome R-HSA-556833Inferred only
ACSF3 participates in Synthesis of very long-chain fatty acyl-CoAsPathwayReactome R-HSA-75876Inferred only
14,15-epoxy-(5Z,8Z,11Z)-eicosatrienoate is a substrate of ACSF3CompoundHuman-GEM MAR01068Inferred only
CHEBI:1146900 is a substrate of ACSF3CompoundHuman-GEM MAR06486Inferred only

Show the remaining 20

CHEBI:1146900 is a substrate of ACSF3CompoundHuman-GEM MAR06484Inferred only
CHEBI:1146900 is a substrate of ACSF3CompoundHuman-GEM MAR06473Inferred only
CHEBI:1146900 is a substrate of ACSF3CompoundHuman-GEM MAR06472Inferred only
CHEBI:1146900 is a substrate of ACSF3CompoundHuman-GEM MAR06471Inferred only
CHEBI:1146900 is a substrate of ACSF3CompoundHuman-GEM MAR06453Inferred only
CHEBI:1146900 is a substrate of ACSF3CompoundHuman-GEM MAR06451Inferred only
CHEBI:1146900 is a substrate of ACSF3CompoundHuman-GEM MAR06450Inferred only
CHEBI:1146900 is a substrate of ACSF3CompoundHuman-GEM MAR06448Inferred only
CHEBI:1146900 is a substrate of ACSF3CompoundHuman-GEM MAR01065Inferred only
CHEBI:1146900 is a substrate of ACSF3CompoundHuman-GEM MAR01066Inferred only
CHEBI:1146900 is a substrate of ACSF3CompoundHuman-GEM MAR01067Inferred only
CHEBI:1146900 is a substrate of ACSF3CompoundHuman-GEM MAR06435Inferred only
CHEBI:1146900 is a substrate of ACSF3CompoundHuman-GEM MAR06436Inferred only
CHEBI:1146900 is a substrate of ACSF3CompoundHuman-GEM MAR01068Inferred only
CHEBI:1146900 is a substrate of ACSF3CompoundHuman-GEM MAR01069Inferred only
CHEBI:1146900 is a substrate of ACSF3CompoundHuman-GEM MAR01070Inferred only
CHEBI:1146900 is a substrate of ACSF3CompoundHuman-GEM MAR06432Inferred only
CHEBI:1146900 is a substrate of ACSF3CompoundHuman-GEM MAR06433Inferred only
malonate is a substrate of ACSF3CompoundRhea RHEA:32139Inferred only
tetracosanoate is a substrate of ACSF3CompoundRhea RHEA:33639Inferred only

Reactions

21 reactions

MAM00279c+ATP+CHEBI:1146900+2water+NAD(+)+dioxygenMAM01219c+CHEBI:15351+AMP+hydrogen peroxide+NADH(2-)+diphosphate(3-)
Catalysed by ACAA1 or HSD17B10 or HADHA or ACOX3 or ACOT7 or

Other names

1 name

Resolves to: ACSF3

Also called: Acyl-CoA synthetase family member 3

← Every protein Find it in the graph →

ACOT8

or ACOT2

or ECHS1

or SLC27A1

or HSD17B4

or BAAT

or HADHB

and 12 more alternatives

Reaction pageLeft to rightUnbalancedEC 1.1.1.35, 1.3.3.6, 2.3.1.176, 2.3.1.16, 3.1.2.2, 4.2.1.17, 5.1.99.4, 6.2.1.-cytosol

Human-GEM MAR01065CC BY 4.0Reference data

MAM01219c+ATP+CHEBI:1146900+2water+NAD(+)+dioxygenMAM01176c+CHEBI:15351+AMP+2proton+hydrogen peroxide+NADH(2-)+diphosphate(3-)

Catalysed by ACAA1 or HSD17B10 or HADHA or ACOX3 or ACOT7 or ACOT8 or ACOT2 or ECHS1 or SLC27A1 or HSD17B4 or BAAT or HADHB and 12 more alternatives

Reaction pageLeft to rightBalancedEC 1.1.1.35, 1.3.3.6, 2.3.1.176, 2.3.1.16, 3.1.2.2, 4.2.1.17, 5.1.99.4, 6.2.1.-cytosol

Human-GEM MAR01066CC BY 4.0Reference data

MAM01176c+ATP+CHEBI:1146900+2water+NAD(+)+dioxygenMAM01055c+CHEBI:15351+AMP+hydrogen peroxide+NADH(2-)+diphosphate(3-)

Catalysed by ACAA1 or HSD17B10 or HADHA or ACOX3 or ACOT7 or ACOT8 or ACOT2 or ECHS1 or SLC27A1 or HSD17B4 or BAAT or HADHB and 11 more alternatives

Reaction pageLeft to rightUnbalancedEC 1.1.1.35, 1.3.3.6, 2.3.1.176, 2.3.1.16, 3.1.2.2, 4.2.1.17, 5.1.99.4, 6.2.1.-cytosol

Human-GEM MAR01067CC BY 4.0Reference data

14,15-epoxy-(5Z,8Z,11Z)-eicosatrienoate+ATP+CHEBI:1146900+2water+NAD(+)+dioxygenMAM00310c+CHEBI:15351+AMP+hydrogen peroxide+NADH(2-)+diphosphate(3-)

Catalysed by ACAA1 or HSD17B10 or HADHA or ACOX3 or ACOT7 or ACOT8 or ACOT2 or ECHS1 or SLC27A1 or HSD17B4 or BAAT or HADHB and 12 more alternatives

Reaction pageLeft to rightUnbalancedEC 1.1.1.35, 1.3.3.6, 2.3.1.176, 2.3.1.16, 3.1.2.2, 4.2.1.17, 5.1.99.4, 6.2.1.-cytosol

Human-GEM MAR01068CC BY 4.0Reference data

MAM00310c+ATP+CHEBI:1146900+2water+NAD(+)+dioxygenMAM00259c+CHEBI:15351+AMP+2proton+hydrogen peroxide+NADH(2-)+diphosphate(3-)

Catalysed by ACAA1 or HSD17B10 or HADHA or ACOX3 or ACOT7 or ACOT8 or ACOT2 or ECHS1 or SLC27A1 or HSD17B4 or BAAT or HADHB and 12 more alternatives

Reaction pageLeft to rightBalancedEC 1.1.1.35, 1.3.3.6, 2.3.1.176, 2.3.1.16, 3.1.2.2, 4.2.1.17, 5.1.99.4, 6.2.1.-cytosol

Human-GEM MAR01069CC BY 4.0Reference data

MAM00259c+ATP+CHEBI:1146900+proton+2water+NAD(+)+dioxygenMAM01210c+CHEBI:15351+AMP+hydrogen peroxide+NADH(2-)+diphosphate(3-)

Catalysed by ACAA1 or HSD17B10 or HADHA or ACOX3 or ACOT7 or ACOT8 or ACOT2 or ECHS1 or SLC27A1 or HSD17B4 or BAAT or HADHB and 12 more alternatives

Reaction pageLeft to rightUnbalancedEC 1.1.1.35, 1.3.3.6, 2.3.1.176, 2.3.1.16, 3.1.2.2, 4.2.1.17, 5.1.99.4, 6.2.1.-cytosol

Human-GEM MAR01070CC BY 4.0Reference data

MAM00345m+ATP+CHEBI:1146900+2water+NAD(+)MAM00287m+AMP+2proton+NADH(2-)+diphosphate(3-)+propionyl-CoA

Catalysed by ACAA1 or HSD17B10 or HADHA or ACOX3 or ACOT7 or ACOT8 or ACOT2 or ECHS1 or SLC27A1 or HSD17B4 or BAAT or HADHB and 12 more alternatives

Reaction pageLeft to rightBalancedEC 1.1.1.35, 1.3.3.6, 2.3.1.176, 2.3.1.16, 3.1.2.2, 4.2.1.17, 5.1.99.4, 6.2.1.-mitochondrial matrix

Human-GEM MAR06432CC BY 4.0Reference data

MAM00287m+ATP+CHEBI:1146900+2water+NAD(+)+dioxygenMAM01226m+CHEBI:15351+AMP+2proton+hydrogen peroxide+NADH(2-)+diphosphate(3-)

Catalysed by ACAA1 or HSD17B10 or HADHA or ACOX3 or ACOT7 or ACOT8 or DECR1 or ACOT2 or ECHS1 or SLC27A1 or HSD17B4 or BAAT and 16 more alternatives

Reaction pageLeft to rightBalancedEC 1.1.1.35, 1.3.1.34, 1.3.3.6, 2.3.1.16, 3.1.2.2, 3.1.2.27, 4.2.1.17, 5.1.99.4, 5.3.3.8, 6.2.1.-mitochondrial matrix

Human-GEM MAR06433CC BY 4.0Reference data

MAM01186m+ATP+CHEBI:1146900+2water+dioxygenMAM01096m+CHEBI:15351+AMP+proton+hydrogen peroxide+diphosphate(3-)

Catalysed by ACAA1 or HSD17B10 or HADHA or ACOX3 or ACOT7 or ACOT8 or DECR1 or ACOT2 or ECHS1 or SLC27A1 or HSD17B4 or BAAT and 16 more alternatives

Reaction pageLeft to rightBalancedEC 1.1.1.35, 1.3.1.34, 1.3.3.6, 2.3.1.16, 3.1.2.2, 3.1.2.27, 4.2.1.17, 5.1.99.4, 5.3.3.8, 6.2.1.-mitochondrial matrix

Human-GEM MAR06435CC BY 4.0Reference data

MAM01096m+ATP+CHEBI:1146900+2water+NAD(+)+dioxygenMAM00766m+AMP+2proton+hydrogen peroxide+NADH(2-)+diphosphate(3-)+propionyl-CoA

Catalysed by ACAA1 or HSD17B10 or HADHA or ACOX3 or ACOT7 or ACOT8 or ACOT2 or ECHS1 or SLC27A1 or HSD17B4 or BAAT or HADHB and 12 more alternatives

Reaction pageLeft to rightBalancedEC 1.1.1.35, 1.3.3.6, 2.3.1.176, 2.3.1.16, 3.1.2.2, 4.2.1.17, 5.1.99.4, 6.2.1.-mitochondrial matrix

Human-GEM MAR06436CC BY 4.0Reference data

MAM00289m+ATP+CHEBI:1146900+2water+NAD(+)+dioxygenMAM01228m+CHEBI:15351+AMP+2proton+hydrogen peroxide+NADH(2-)+diphosphate(3-)

Catalysed by ACAA1 or HSD17B10 or HADHA or ACOX3 or ACOT7 or ACOT8 or DECR1 or ACOT2 or ECHS1 or SLC27A1 or HSD17B4 or BAAT and 16 more alternatives

Reaction pageLeft to rightBalancedEC 1.1.1.35, 1.3.1.34, 1.3.3.6, 2.3.1.16, 3.1.2.2, 3.1.2.27, 4.2.1.17, 5.1.99.4, 5.3.3.8, 6.2.1.-mitochondrial matrix

Human-GEM MAR06448CC BY 4.0Reference data

MAM01228m+ATP+CHEBI:1146900+2water+NAD(+)MAM01188m+AMP+2proton+NADH(2-)+diphosphate(3-)+propionyl-CoA

Catalysed by ACAA1 or HSD17B10 or HADHA or ACOX3 or ACOT7 or ACOT8 or ACOT2 or ECHS1 or SLC27A1 or HSD17B4 or BAAT or HADHB and 12 more alternatives

Reaction pageLeft to rightBalancedEC 1.1.1.35, 1.3.3.6, 2.3.1.176, 2.3.1.16, 3.1.2.2, 4.2.1.17, 5.1.99.4, 6.2.1.-mitochondrial matrix

Human-GEM MAR06450CC BY 4.0Reference data

Show the remaining 9