Testing Data
Rabbit ABCD1 Polyclonal Antibody | anti-ABCD1 antibody
ABCD1 Antibody
Testing Data
Testing Data
NCBI and Uniprot Product Information
NCBI Description
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 is likely involved in the peroxisomal transport or catabolism of very long chain fatty acids. Defects in this gene have been identified as the underlying cause of adrenoleukodystrophy, an X-chromosome recessively inherited demyelinating disorder of the nervous system. [provided by RefSeq, Jul 2008]
Uniprot Description
ABCD1: Probable transporter. The nucleotide-binding fold acts as an ATP-binding subunit with ATPase activity. Defects in ABCD1 are the cause of adrenoleukodystrophy X- linked (X-ALD). X-ALD is a peroxisomal metabolic disorder characterized by progressive multifocal demyelination of the central nervous system and by peripheral adrenal insufficiency (Addison disease). It results in mental deterioration, corticospinal tract dysfunction, and cortical blindness. Different clinical manifestations exist like: cerebral childhood ALD (CALD), adult cerebral ALD (ACALD), adrenomyeloneuropathy (AMN) and 'Addison disease only' (ADO) phenotype. The promoter region of ABCD1 is deleted in the chromosome Xq28 deletion syndrome which involves ABCD1 and the neighboring gene BCAP31. Belongs to the ABC transporter superfamily. ABCD family. Peroxisomal fatty acyl CoA transporter (TC 3.A.1.203) subfamily.
Protein type: Membrane protein, integral; Transporter, ABC family; Transporter; Hydrolase; Membrane protein, multi-pass; Mitochondrial
Chromosomal Location of Human Ortholog: Xq28
Cellular Component: peroxisomal membrane; integral to peroxisomal membrane; mitochondrion; membrane; perinuclear region of cytoplasm; cytoplasm; peroxisome; cytosol
Molecular Function: identical protein binding; protein binding; protein homodimerization activity; enzyme binding; peroxisomal fatty acyl CoA transporter activity; transporter activity; ATPase activity, coupled to transmembrane movement of substances; ATPase activity; ATP binding
Biological Process: fatty acid beta-oxidation using acyl-CoA oxidase; fatty acid beta-oxidation; peroxisomal membrane transport; peroxisomal long-chain fatty acid import; very-long-chain fatty acid catabolic process; peroxisome organization and biogenesis; unsaturated fatty acid metabolic process; linoleic acid metabolic process; cellular lipid metabolic process; transmembrane transport; long-chain fatty acid catabolic process
Disease: Adrenoleukodystrophy
Research Articles on ABCD1
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Product Notes
The ABCD1 abcd1 (Catalog #AAA2519721) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The ABCD1 Antibody reacts with Human, Mouse, Rat and may cross-react with other species as described in the data sheet. AAA Biotech's ABCD1 can be used in a range of immunoassay formats including, but not limited to, ELISA (EIA), Western Blot (WB). WB: 1:500-1:5000. Researchers should empirically determine the suitability of the ABCD1 abcd1 for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "ABCD1, Polyclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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