Rabbit anti-Human, Mouse ABCD1 Polyclonal Antibody | anti-ABCD1 antibody

Anti-ABCD1 Antibody

Cat. Num. AAA416373

• Gene Names: ABCD1; ALD; AMN; ALDP; ABC42
• Reactivity: Human, Mouse
• Applications: Western Blot, Immunohistochemistry, ELISA
• Purity: Affinity purified
• Synonyms: ABCD1; Polyclonal Antibody; Anti-ABCD1 Antibody; anti-ABCD1 antibody

• Host: Rabbit
• Reactivity: Human, Mouse
• Clonality: Polyclonal
• Isotype: IgG
• Purity/Purification: Affinity purified
• Form/Format: Liquid
• Concentration: 100ug/100ul (varies by lot)
• Sequence Length: 745

• Applicable Applications for anti-ABCD1 antibody: Western Blot (WB), Immunohistochemistry (IHC), ELISA (EIA)
• Application Notes: Suggested dilutions:; ELISA: 1:20,000~80,000; Western blot: 1:200~1,000; Immunohistochemistry: 1:100~500; Final dilutions to be determined by end user.
• Storage Buffer: PBS, pH 7.4 with 0.02% Sodium Azide
• Immunogen: Recombinant protein
• Preparation and Storage: This product is stable for several weeks at 4 degree C as an undiluted liquid. Dilute only prior to immediate use. For extended storage, aliquot contents and freeze at -20 degree C or below. Avoid cycles of freezing and thawing. Expiration date is one (1) year from date of receipt.

Testing Data

Related Product Information for anti-ABCD1 antibody

Homo sapiens ATP-binding cassette, sub-family D (ALD), member 1 (ABCD1), mRNA - GeneBank Accession no NM_000033

NCBI and Uniprot Product Information

• NCBI GI #: 7262393
• NCBI GeneID: 215
• NCBI Accession #: NP_000024.2
• NCBI GenBank Nucleotide #: NM_000033.3
• Molecular Weight: 82,937 Da
• NCBI Official Full Name: ATP-binding cassette sub-family D member 1
• NCBI Official Synonym Full Names: ATP-binding cassette, sub-family D (ALD), member 1
• NCBI Official Symbol: ABCD1
• NCBI Official Synonym Symbols: ALD; AMN; ALDP; ABC42
• NCBI Protein Information: ATP-binding cassette sub-family D member 1; adrenoleukodystrophy protein
• UniProt Protein Name: ATP-binding cassette sub-family D member 1
• Protein Family: ABC transporter D family
• UniProt Gene Name: ABCD1
• UniProt Entry Name: ABCD1_HUMAN

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

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; 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