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

Anti-ABCD1 Antibody, Rabbit Polyclonal

Cat. Num. AAA8102988

• Gene Names: ABCD1; ALD; AMN; ALDP; ABC42
• Reactivity: Human
• Applications: Immunohistochemistry, Immunocytochemistry, Immunofluorescence
• Purity: Protein A & Antigen Affinity
• Synonyms: ABCD1; Polyclonal Antibody; Anti-ABCD1 Antibody; Rabbit Polyclonal; ABCD1 Antibody; Rabbit PAb; Antigen Affinity Purified; ATP-binding cassette; sub-family D (ALD); member 1; Anti-ABC42 Antibody; Anti-ALD Antibody; Anti-ALDP Antibody; Anti-AMN Antibody; anti-ABCD1 antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Isotype: Rabbit IgG
• Specificity: Human ABCD1
• Purity/Purification: Protein A & Antigen Affinity
• Form/Format: Liquid; PBS, pH7.0 with 0.03% Proclin300

• Applicable Applications for anti-ABCD1 antibody: Immunohistochemistry-Paraffin (IHC-P), Immunocytochemistry (ICC), Immunofluorescence (IF)
• Application Notes: IHC-P: 1:200-1:1000; ICC/IF: 1:200-1:1000
• Immunogen: A synthetic peptide corresponding to the center region of the Human ABCD1
• Conjugation: Unconjugated
• Preparation: Produced in rabbits immunized with a synthetic peptide corresponding to the center region of the Human ABCD1, and purified by antigen affinity chromatography.
• Preparation and Storage: This antibody can be stored at 2-8 degree C for one month without detectable loss of activity. Antibody products are stable for twelve months from date of receipt when stored at -20 degree C to -80 degree C. Avoid repeated freeze-thaw cycles.

Immunohistochemistry (IHC)

(Immunochemical staining of human ABCD1 in human pancreas with rabbit polyclonal antibody at 1:300 dilution, formalin-fixed paraffin embedded sections.)

Immunofluorescence (IF)

(Immunofluorescence staining of ABCD1 in MCF7 cells. Cells were fixed with 4% PFA, permeabilzed with 0.1% Triton X-100 in PBS,blocked with 10% serum, and incubated with rabbit anti-Human ABCD1 polyclonal antibody (dilution ratio 1:500) at 4 degree C overnight. Then cells were stained with the Alexa Fluor488-conjugated Goat Anti-rabbit IgG secondary antibody (green). Positive staining was localized to Cytoplasm.)

NCBI and Uniprot Product Information

• NCBI GI #: 7262393
• NCBI GeneID: 215
• NCBI Accession #: NP_000024.2
• NCBI GenBank Nucleotide #: NM_000033.3
• UniProt Accession #: P33897; Q6GTZ2
• 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 Synonym Gene Names: ALD; ALDP
• 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: Membrane protein, multi-pass; Hydrolase; Mitochondrial; Membrane protein, integral; Transporter; Transporter, ABC family

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; 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; peroxisome organization and biogenesis; peroxisomal long-chain fatty acid import; very-long-chain fatty acid catabolic process; unsaturated fatty acid metabolic process; linoleic acid metabolic process; cellular lipid metabolic process; transmembrane transport; long-chain fatty acid catabolic process

Disease: Adrenoleukodystrophy