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Proteins/UNIPROT:P33897
ATP-dependent transporter of the ATP-binding cassette (ABC) family involved in the transport of very long chain fatty acid (VLCFA)-CoA from the cytosol to the peroxisome lumen. Coupled to the ATP-dependent transporter activity also has a fatty acyl-CoA thioesterase activity (ACOT) and hydrolyzes VLCFA-CoA into VLCFA prior their ATP-dependent transport into peroxisomes, the ACOT activity is essential during this transport process. Thus, plays a role in regulation of VLCFAs and energy metabolism namely, in the degradation and biosynthesis of fatty acids by beta-oxidation, mitochondrial function and microsomal fatty acid elongation. Involved in several processes; namely, controls the active myelination phase by negatively regulating the microsomal fatty acid elongation activity and may also play a role in axon and myelin maintenance. Also controls the cellular response to oxidative stress by regulating mitochondrial functions such as mitochondrial oxidative phosphorylation and depolarization. And finally controls the inflammatory response by positively regulating peroxisomal beta-oxidation of VLCFAs (By similarity)..
Function from UniProtKB (P33897), read 2026-09-23. CC BY 4.0.
Curated compounds routed: 0
5 rows
62 statements
62 statements · 6 reference databases · Subject: ABCD1 · Sources: gene ontology, Hetionet, Human-GEM, Reactome, Rhea, WikiPathways
33 reactions
ATP+CHEBI:15519+waterADP+CHEBI:15519+proton+phosphate(3-)
Transported by IDH3G or PDZD4 or ABCD1 or SRPK3 or BCAP31
Reaction pageLeft to rightBalancedcytosol, peroxisome
Human-GEM MAR01659CC BY 4.0Reference data
MAM00110cMAM00110x
3 names
Resolves to: ABCD1
Transported by ABCD1
Reaction pageReversibleBalancedcytosol, peroxisome
Human-GEM MAR02407CC BY 4.0Reference data
(5Z,8Z,11Z,14Z)-eicosatetraenoate+ATP+waterADP+(5Z,8Z,11Z,14Z)-eicosatetraenoate+proton+phosphate(3-)
Transported by ABCD1
Reaction pageLeft to rightBalancedcytosol, peroxisome
Human-GEM MAR02424CC BY 4.0Reference data
ATP+water+hexadecanoic acidADP+proton+hexadecanoic acid+phosphate(3-)
Transported by ABCD1
Reaction pageLeft to rightBalancedcytosol, peroxisome
Human-GEM MAR02425CC BY 4.0Reference data
(6Z,9Z,12Z,15Z,18Z,21Z)-tetracosahexaenoyl-CoA(6Z,9Z,12Z,15Z,18Z,21Z)-tetracosahexaenoyl-CoA
Transported by ABCD1
Reaction pageReversibleBalancedcytosol, peroxisome
Human-GEM MAR02509CC BY 4.0Reference data
ATP+water+propionyl-CoAADP+proton+phosphate(3-)+propionyl-CoA
Transported by IDH3G or PDZD4 or ABCD1 or BCAP31
Reaction pageLeft to rightBalancedcytosol, peroxisome
Human-GEM MAR03008CC BY 4.0Reference data
ATP+water+palmitoyl-CoAADP+proton+palmitoyl-CoA+phosphate(3-)
Transported by IDH3G or PDZD4 or ABCD1 or SRPK3 or BCAP31
Reaction pageLeft to rightBalancedcytosol, peroxisome
Human-GEM MAR03011CC BY 4.0Reference data
ATP+water+(9Z,12Z)-octadecadienoyl-CoAADP+proton+(9Z,12Z)-octadecadienoyl-CoA+phosphate(3-)
Transported by IDH3G or PDZD4 or ABCD1 or PNCK or SRPK3 or BCAP31
Reaction pageLeft to rightBalancedcytosol, peroxisome
Human-GEM MAR03013CC BY 4.0Reference data
arachidonoyl-CoA+ATP+waterADP+arachidonoyl-CoA+proton+phosphate(3-)
Transported by IDH3G or PDZD4 or ABCD1 or PNCK or BCAP31
Reaction pageLeft to rightBalancedcytosol, peroxisome
Human-GEM MAR03014CC BY 4.0Reference data
(13Z)-eicosenoyl-CoA+ATP+water(13Z)-eicosenoyl-CoA+ADP+proton+phosphate(3-)
Transported by IDH3G or PDZD4 or ABCD1 or PNCK or BCAP31
Reaction pageLeft to rightBalancedcytosol, peroxisome
Human-GEM MAR03017CC BY 4.0Reference data
tricosanoyl-CoAtricosanoyl-CoA
Transported by ABCD1
Reaction pageReversibleBalancedcytosol, peroxisome
Human-GEM MAR03018CC BY 4.0Reference data
tetracosanoyl-CoAtetracosanoyl-CoA
Transported by ABCD1
Reaction pageReversibleBalancedcytosol, peroxisome
Human-GEM MAR03019CC BY 4.0Reference data