NP_001116146.1
[Other Products]
NCBI GenBank Nucleotide #
|
[Other Products]
UniProt Primary Accession #
|
[Other Products]
UniProt Secondary Accession #
UniProt Related Accession #
Molecular Weight
27,121 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
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; Membrane protein, integral; Transporter; Transporter, ABC family; Mitochondrial
Chromosomal Location of Human Ortholog: 1p21.3
Cellular Component: peroxisomal membrane; peroxisomal matrix; intracellular membrane-bound organelle; membrane; mitochondrial inner membrane; integral to membrane; peroxisome; cytosol
Molecular Function: protein binding; protein homodimerization activity; ATPase activity, coupled to transmembrane movement of substances; ATPase activity; ATP binding
Biological Process: fatty acid beta-oxidation; peroxisome organization and biogenesis; very-long-chain fatty acid catabolic process; transmembrane transport
Disease: Bile Acid Synthesis Defect, Congenital, 5
Research Articles on ABCD3
Precautions
All of MyBioSource's Products are for scientific laboratory research purposes and are not for diagnostic, therapeutics, prophylactic or in vivo use. Through your purchase, you expressly represent and warrant to MyBioSource that you will properly test and use any Products purchased from MyBioSource in accordance with industry standards. MyBioSource and its authorized distributors reserve the right to refuse to process any order where we reasonably believe that the intended use will fall outside of our acceptable guidelines.
Disclaimer
While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice. It is the responsibility of the customer to report product performance issues to MyBioSource within 30 days of receipt of the product. Please visit our Terms & Conditions page for more information.
Products associated with ABCD3 blocking peptide
Pathways associated with ABCD3 blocking peptide
Diseases associated with ABCD3 blocking peptide
Organs/Tissues associated with ABCD3 blocking peptide
|