BC009712
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UniProt Secondary Accession #
UniProt Related Accession #
Molecular Weight
27,121 Da
NCBI Official Full Name
Homo sapiens ATP-binding cassette, sub-family D (ALD), member 3, mRNA
NCBI Official Synonym Full Names
ATP binding cassette subfamily D member 3
NCBI Official Synonym Symbols
ZWS2; ABC43; CBAS5; PMP70; PXMP1 [Similar Products]
NCBI Protein Information
ATP-binding cassette sub-family D member 3
UniProt Protein Name
ATP-binding cassette sub-family D member 3
UniProt Synonym Protein Names
70 kDa peroxisomal membrane protein; PMP70
UniProt Synonym Gene Names
UniProt Entry Name
ABCD3_HUMAN
NCBI Summary for ABCD3
The protein encoded by this gene is a member of the superfamily of ATP-binding cassette (ABC) transporters. 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. This peroxisomal membrane protein likely plays an important role in peroxisome biogenesis. Mutations have been associated with some forms of Zellweger syndrome, a heterogeneous group of peroxisome assembly disorders. Alternative splicing results in multiple transcript variants encoding distinct isoforms. [provided by RefSeq, Jul 2008]
UniProt Comments for ABCD3
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.
Protein type: Membrane protein, multi-pass; Mitochondrial; Membrane protein, integral; Transporter; Transporter, ABC family
Chromosomal Location of Human Ortholog: 1p21.3
Cellular Component: cytosol; intracellular membrane-bound organelle; membrane; peroxisomal matrix; peroxisomal membrane; peroxisome
Molecular Function: ATP binding; ATPase activity; long-chain fatty acid transporter activity; protein binding; protein homodimerization activity
Biological Process: fatty acid beta-oxidation; fatty acid biosynthetic process; peroxisome organization and biogenesis; transmembrane transport; very-long-chain fatty acid catabolic process
Disease: Bile Acid Synthesis Defect, Congenital, 5
Research Articles on ABCD3
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Pathways associated with ABCD3 cdna clone
Diseases associated with ABCD3 cdna clone
Organs/Tissues associated with ABCD3 cdna clone
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