The protein encoded by this gene is a member of the superfamily of ATP-binding cassette (ABC) transporters. Mutation in ABCD3 have been found in two individuals affected by Zellweger syndrome. However, the role of ABCD3 in the causation of Zellweger syndrome remains uncertain. which is involved in peroxisomal import of fatty acids and/or fatty acyl-CoAs in the organelle. All known peroxisomal ABC transporters are half transporters which require a partner half transporter molecule to form a functional homodimeric or heterodimeric transporter. This peroxisomal membrane protein likely plays an important role in peroxisome biogenesis. Probable transporter involved in the transport of branched-chain fatty acids and C27 bile acids into the peroxisome; the latter function is a crucial step in bile acid biosynthesis. The nucleotide-binding fold acts as an ATP-binding subunit with ATPase activity.
ATP-Binding Cassette, Sub-Family D (ALD), Member 3; 70 KDa Peroxisomal Membrane Protein; PXMP1; PMP70; DJ824O18.1 (ATP-Binding Cassette, Sub-Family D (ALD), Member 3 (PMP70, PXMP1)); Peroxisomal Membrane Protein 1 (70kD, Zellweger Syndrome); Peroxisomal Membrane Protein-1 (70kD); ABC43; CBAS5; ZWS2