NP_001116146.1
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NCBI GenBank Nucleotide #
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UniProt Primary Accession #
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UniProt Secondary Accession #
UniProt Related Accession #
Molecular Weight
75,476 Da
NCBI Official Full Name
ATP-binding cassette sub-family D member 3 isoform b
NCBI Official Synonym Full Names
ATP-binding cassette, sub-family D (ALD), member 3
NCBI Protein Information
ATP-binding cassette sub-family D member 3; 70 kDa peroxisomal membrane protein; Peroxisomal membrane protein-1 (70kD); peroxisomal membrane protein 1 (70kD, Zellweger syndrome); dJ824O18.1 (ATP-binding cassette, sub-family D (ALD), member 3 (PMP70, PXMP1))
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
Function: Probable transporter. The nucleotide-binding fold acts as an ATP-binding subunit with ATPase activity. Ref.11
Subunit structure: Can form heterodimers with ABCD1/ALD and ABCD2/ALDR. Dimerization is necessary to form an active transporter. Interacts with PEX19. Ref.8 Ref.9 Ref.10
Subcellular location: Peroxisome membrane; Multi-pass membrane protein Ref.10.
Miscellaneous: 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.
Sequence similarities: Belongs to the ABC transporter superfamily. ABCD family. Peroxisomal fatty acyl CoA transporter (TC 3.A.1.203) subfamily. [View classification]Contains 1 ABC transmembrane type-1 domain.Contains 1 ABC transporter domain.
Research Articles on ABCD3
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Products associated with anti-ABCD3 antibody
Pathways associated with anti-ABCD3 antibody
Diseases associated with anti-ABCD3 antibody
Organs/Tissues associated with anti-ABCD3 antibody
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