ABCD3
ABCD3, also known as PMP70, ZWS2 and PXMP1, is an ATP-dependent transporter of the ABC family in the peroxisome membrane. It moves long- and very-long-chain fatty acyl-CoAs from the cytosol into the peroxisome matrix for beta-oxidation. Browse Assay Genie's current range of ABCD3 research reagents below, or read on for the biology behind the target.
ABCD3 is an ATP-binding cassette (ABC) transporter that catalyses the transport of long- and very-long-chain fatty acyl-CoAs, and probably bile acyl-CoAs, from the cytosol to the peroxisome matrix, where they undergo beta-oxidation. It appears to be specifically involved in the peroxisomal transport of branched-chain fatty acyl-CoAs (BCFA-CoAs) and dicarboxylic acyl-CoAs (DCA-CoAs). The protein has both fatty acyl-CoA thioesterase and ATPase activities, and is thought to hydrolyse fatty acyl-CoAs into free fatty acids before their ATP-dependent transport into peroxisomes.
The ABCD3 gene encodes a member of the ABC transporter superfamily, which moves molecules across extra- and intracellular membranes. It belongs to the ALD subfamily (the ABCD family), whose members are involved in peroxisomal import of fatty acids and fatty acyl-CoAs, and to the peroxisomal fatty acyl-CoA transporter subfamily (TC 3.A.1.203). All known peroxisomal ABC transporters are half transporters, so each needs a partner half transporter to form a functional homodimeric or heterodimeric transporter. ABCD1 is another member of this subfamily. Alternative splicing produces multiple transcript variants that encode distinct isoforms. Mouse and rat both have an Abcd3 ortholog.
The ABCD3 protein is likely to play an important role in peroxisome biogenesis. Mutations have been associated with some forms of Zellweger syndrome, a heterogeneous group of peroxisome assembly disorders. This link explains the earlier names, peroxisomal membrane protein 1 (70kD, Zellweger syndrome) and ATP-binding cassette, sub-family D (ALD), member 3. Researchers studying the ABCD3 protein often refer to it by the alias PMP70.
ABCD3 carries long- and very-long-chain fatty acyl-CoAs into the peroxisome matrix for beta-oxidation, making it central to peroxisomal lipid handling.
ABCD3 appears to be specifically involved in peroxisomal transport of branched-chain fatty acyl-CoAs and dicarboxylic acyl-CoAs.
ABCD3 likely contributes to peroxisome biogenesis, and mutations have been associated with some forms of Zellweger syndrome.
As a half transporter that needs a partner to form a homodimer or heterodimer, ABCD3 is a model for peroxisomal ABC transporter function.
ABCD3 is an ATP-dependent transporter in the peroxisome membrane. It moves long- and very-long-chain fatty acyl-CoAs, and probably bile acyl-CoAs, from the cytosol into the peroxisome matrix for beta-oxidation. It also has fatty acyl-CoA thioesterase and ATPase activities.
ABCD3 is also called PMP70, ZWS2 and PXMP1. Earlier names include peroxisomal membrane protein 1 (70kD, Zellweger syndrome). Alternative splicing of the gene gives multiple transcript variants that encode distinct isoforms.
Mutations in ABCD3 have been associated with some forms of Zellweger syndrome, a group of peroxisome assembly disorders. Mouse and rat each have an Abcd3 ortholog; check the datasheet of any product for its validated reactivity.
Other ABCD subfamily peroxisomal transporters with products at Assay Genie.
Gene and protein annotations from HGNC, NCBI Gene and UniProt P28288; product specifications from Assay Genie datasheets.