ABCD3 a member of the superfamily of ATP-binding cassette (ABC) transporters. Likely plays an important role in peroxisome biogenesis. ABC proteins transport various molecules across extra- and intra-cellular membranes. ABC genes are divided into seven distinct subfamilies (ABC1, MDR/TAP, MRP, ALD, OABP, GCN20, White). This protein is a member of the ALD subfamily, 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. Defects in ABCD3 may be the cause of Zellweger syndrome-2 (ZWS-2), an autosomal recessive disorder due to defective import mechanisms for peroxisomal matrix enzymes. Note: This description may include information from UniProtKB.
Protein type: Membrane protein, integral; Membrane protein, multi-pass; Mitochondrial; Transporter; Transporter, ABC family
Chromosomal Location of Human Ortholog: 2q41
Cellular Component:  integral component of membrane; intracellular membrane-bounded organelle; mitochondrion; peroxisomal matrix; peroxisomal membrane; peroxisome
Molecular Function:  ATP binding; ATPase activity; ATPase activity, coupled to transmembrane movement of substances; long-chain fatty acid transporter activity; protein binding; protein homodimerization activity; protein self-association
Biological Process:  fatty acid beta-oxidation; fatty acid biosynthetic process; peroxisomal long-chain fatty acid import; peroxisome organization; response to drug; response to organic cyclic compound; very long-chain fatty acid catabolic process
Reference #:  P16970 (UniProtKB)
Alt. Names/Synonyms: 70 kDa peroxisomal membrane protein; 70-kDa peroxisomal membrane protein; Abcd3; ATP-binding cassette sub-family D member 3; ATP-binding cassette, sub-family D (ALD), member 3; Peroxisomal membrane protein 1; PMP70; Pxmp1
Gene Symbols: Abcd3
Molecular weight: 75,316 Da
Basal Isoelectric point: 9.32  Predict pI for various phosphorylation states
Select Structure to View Below


Protein Structure Not Found.

Cross-references to other databases:  STRING  |  Reactome  |  BioGPS  |  Pfam  |  Phospho.ELM  |  NetworKIN  |  UniProtKB  |  Entrez-Gene  |  Ensembl Gene