Broad substrate specificity ATP-dependent transporter of the ATP-binding cassette (ABC) family that catalyzes the transport of long-chain fatty acids (LCFA)-CoA, dicarboxylic acids-CoA, long-branched-chain fatty acids-CoA and bile acids from the cytosol to the peroxisome lumen for beta-oxydation (PubMed:11248239, PubMed:25168382, PubMed:24333844, PubMed:29397936). Has fatty acyl-CoA thioesterase and ATPase activities (PubMed:29397936). Probably hydrolyzes fatty acyl-CoAs into free fatty acids prior to their ATP-dependent transport into peroxisomes (By similarity). Thus, play a role in regulation of LCFAs and energy metabolism namely, in the degradation and biosynthesis of fatty acids by beta-oxidation (PubMed:25944712, PubMed:24333844). 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 a long-chain fatty acyl-CoA + H2O = a long-chain fatty acid + CoA + H(+) a long-chain fatty acid(in) + ATP + H2O = a long-chain fatty acid(out) + ADP + H(+) + phosphate H2O + pristanoyl-CoA = 2,6,10,14-tetramethylpentadecanoate + CoA + H(+) 2,6,10,14-tetramethylpentadecanoate(in) + ATP + H2O = 2,6,10,14-tetramethylpentadecanoate(out) + ADP + H(+) + phosphate H2O + hexadecanedioyl-CoA = CoA + H(+) + hexadecanedioate ATP + H2O + hexadecanedioate(in) = ADP + H(+) + hexadecanedioate(out) + phosphate (5Z,8Z,11Z,14Z,17Z)-eicosapentaenoyl-CoA + H2O = (5Z,8Z,11Z,14Z,17Z)-eicosapentaenoate + CoA + H(+) (5Z,8Z,11Z,14Z,17Z)-eicosapentaenoate(in) + ATP + H2O = (5Z,8Z,11Z,14Z,17Z)-eicosapentaenoate(out) + ADP + H(+) + phosphate (4Z,7Z,10Z,13Z,16Z,19Z)-docosahexaenoyl-CoA + H2O = (4Z,7Z,10Z,13Z,16Z,19Z)-docosahexaenoate + CoA + H(+) (4Z,7Z,10Z,13Z,16Z,19Z)-docosahexaenoate(in) + ATP + H2O = (4Z,7Z,10Z,13Z,16Z,19Z)-docosahexaenoate(out) + ADP + H(+) + phosphate Homodimers (PubMed:17609205). Can form heterodimers with ABCD1 and ABCD2 (PubMed:10777694, PubMed:10551832, PubMed:17609205). Dimerization is necessary to form an active transporter (PubMed:17609205). Interacts with PEX19; mediates the targeting of ABCD3 to peroxisomes (PubMed:10704444, PubMed:16344115, PubMed:17761678). The disease is caused by variants affecting the gene represented in this entry. Belongs to the ABC transporter superfamily. ABCD family. Peroxisomal fatty acyl CoA transporter (TC 3.A.1.203) subfamily. Mutation in ABCD3 have been found in two individuals affected by Zellweger syndrome (PubMed:1301993). Later studies, however, showed unambiguously that a PEX1 defect was the underlying cause of the defect in peroxisome biogenesis in these patients (PubMed:9539740). Database for mutations in ABC proteins