ABCD1, Polyclonal Antibody

ALD; ATP-binding cassette sub-family D member 1; Adrenoleukodystrophy protein; ALDP

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

Rabbit

Recognizes endogenous levels of ABCD1 protein.

The antibody was purified by immunogen affinity chromatography.

Liquid in 0.42% Potassium phosphate, 0.87% Sodium chloride, pH 7.3, 30% glycerol, and 0.01% sodium azide.

Shipped at 4 degree C. Upon delivery aliquot and store at -20 degree C for one year. Avoid freeze/thaw cycles.

Small volumes of anti-ABCD1 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

Rabbit polyclonal antibody to ABCD1

Western Blot (WB)

WB (1/500 - 1/1000)

Western blot analysis of ABCD1 expression in HeLa (A), mouse brain (B), rat kidney (C) whole cell lysates.

82,937 Da

ATP-binding cassette, sub-family D (ALD), member 1

ATP-binding cassette sub-family D member 1

ATP-binding cassette sub-family D member 1

ALD; ALDP  [Similar Products]

ABCD1_HUMAN

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]

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), ***** 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: Transporter; Hydrolase; Membrane protein, multi-pass; Transporter, ABC family; Membrane protein, integral; Mitochondrial

Chromosomal Location of Human Ortholog: Xq28

Cellular Component: peroxisomal membrane; integral to peroxisomal membrane; membrane; mitochondrion; perinuclear region of cytoplasm; cytoplasm; peroxisome; cytosol

Molecular Function: identical protein binding; protein binding; enzyme binding; protein homodimerization activity; peroxisomal fatty acyl CoA transporter activity; ATPase activity, coupled to transmembrane movement of substances; transporter activity; ATPase activity; ATP binding

Biological Process: fatty acid beta-oxidation using acyl-CoA oxidase; peroxisomal membrane transport; fatty acid beta-oxidation; 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

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.

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.