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. Has fatty acyl-CoA thioesterase (ACOT) and ATPase activities. Coupled to the ATP-dependent transporter activity has also 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 (By similarity). 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 (PubMed:9126326, PubMed:9418970, PubMed:9256488, PubMed:18854420, PubMed:23123468, PubMed:23604518, PubMed:25255441, PubMed:25583114, PubMed:26108493). 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 (PubMed:11875044, PubMed:15489218, PubMed:26108493). Controls also the cellular response to oxidative stress by regulating mitochondrial functions such as mitochondrial oxidative phosphorylation and depolarization (PubMed:18344354, PubMed:22521832, PubMed:23604518, PubMed:25583114). And finally controls the inflammatory response by positively regulating peroxisomal beta-oxidation of VLCFAs (PubMed:18723473). a very long-chain fatty acyl-CoA + H2O = a very long-chain fatty acid + CoA + H(+) a very long-chain fatty acid(in) + ATP + H2O = a very long-chain fatty acid(out) + ADP + H(+) + phosphate H2O + tetracosanoyl-CoA = CoA + H(+) + tetracosanoate ATP + H2O + tetracosanoate(in) = ADP + H(+) + phosphate + tetracosanoate(out) H2O + hexacosanoyl-CoA = CoA + H(+) + hexacosanoate ATP + H2O + hexacosanoate(in) = ADP + H(+) + hexacosanoate(out) + phosphate docosanoyl-CoA + H2O = CoA + docosanoate + H(+) ATP + docosanoate(in) + H2O = ADP + docosanoate(out) + H(+) + phosphate Can form homodimers and heterodimers with ABCD2 and ABCD3 (PubMed:15276650). Dimerization is necessary to form an active transporter (By similarity). The minimal functional unit is a homodimer but the major oligomeric form in peroxisomal membrane is a homotetramer. Forms heterotramers with ABCD2 (By similarity). Interacts with PEX19; facilitates ABCD1 insertion into the peroxisome membrane (By similarity). Widely expressed at low levels with higher levels in heart, lung, intestine and spleen than in skeletal muscle, brain, liver and kidney. Most abundant in embryo. Gradually decreases during maturation. By dietary fenofibrate. The NH2-terminal transmembrane domaine (TMD) is involved in the recognition of substrates, and undergoes a conformational change upon ATP binding to the COOH-terminal nucleotide binding domain (NBD). Tyrosine-phosphorylated. Abcd1 hemizygous males are viable and apparently healthy and they show no detectable motor defect for at least 4-month-old. Inbreeding homozygous and hemizygous Abcd1-deficient mice show a reduction of fertility. Moreover, among several 6-month-old mice, there is at least one apparently infertile mutant of each sex. The infertile hemizygous male show additionally a severe testicular atrophy (PubMed:9418970). Abcd1 hemizygous mutant male and homozygous mutant mice grow normally, are fer- tile, and appear normal at least up to one year of age (PubMed:9126326). Abcd1 hemizygous mutant male and homozygous mutant mice grow normally, are fer- tile, and appear normal at least up to 6 months of age (PubMed:9256488). At 20 months of age, Abcd1 homozygous mice present an impairment of their locomotor coordination and exploratory abilities (PubMed:11875044, PubMed:15489218). Double Abcd1 and Abcd2 knockout mice exhibit severe impairment of their locomotor coordination and exploratory abilities already at 15 months of age (PubMed:15489218). Belongs to the ABC transporter superfamily. ABCD family. Peroxisomal fatty acyl CoA transporter (TC 3.A.1.203) subfamily.