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

ABCD1, ID (ABCD1, ALD, ATP-binding cassette sub-family D member 1, Adrenoleukodystrophy protein) (Biotin)

Cat. Num. AAA6270465

• Gene Names: ABCD1; ALD; AMN; ALDP; ABC42
• Reactivity: Human
• Applications: Immunohistochemistry, Western Blot, Flow Cytometry, Functional Assay, ELISA
• Purity: Purified by Protein A and Peptide Affinity Chromatography.
• Synonyms: ABCD1; Polyclonal Antibody; ID (ABCD1; ALD; ATP-binding cassette sub-family D member 1; Adrenoleukodystrophy protein) (Biotin); Adrenoleukodystrophy protein; anti-ABCD1 antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Isotype: IgG
• Specificity: Recognizes human ABCD1.
• Purity/Purification: Purified by Protein A and Peptide Affinity Chromatography.
• Form/Format: Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with Biotin.

• Applicable Applications for anti-ABCD1 antibody: Immunohistochemistry (IHC) Paraffin, Western Blot (WB), Flow Cytometry (FC/FACS), ELISA (EIA)
• Application Notes: Applications are based on unconjugated antibody.
• Immunogen: KLH-conjugated synthetic peptide mapping to a fragment of residues within amino acids 257-285 in the central region of human ABCD1, UniProt Accession #P33897
• Conjugate: Biotin
• Note: Preservative Free
• Preparation and Storage: Store product at 4 degree C if to be used immediately within two weeks. For long-term storage, aliquot to avoid repeated freezing and thawing and store at -20 degree C. Aliquots are stable at -20 degree C for 12 months after receipt. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Western Blot (WB)

(Western Blot analysis in HL-60 cell line lysates (35ug/lane) usingMBS648014. This demonstrates MBS648014 detected the ABCD1 protein (arrow).)

Immunohistochemistry (IHC)

(Immunohistochemistry analysis of formalin fixed and paraffin embedded human hepatocarcinoma using MBS648014 followed by peroxidase conjugation of the secondary antibody and DAB staining. This data demonstrates the use of MBS648014 for immunohistochemistry.)

Flow Cytometry (FC/FACS)

(Flow Cytometric analysis of HL-60 cells (right histogram) using MBS648014 compared to a negative control cell (left histogram).FITC-conjugated goat-anti-rabbit secondary antibodies were used for the analysis.)

Product Categories/Family for anti-ABCD1 antibody

Antibodies; Multi Drug Resistance (MDR)

References

Matsukawa, T., et al. Neurogenetics (2010). In press: Xie, H.H., et al. Zhonghua Yi Xue Yi Chuan Xue Za Zhi 27(2):144-148(2010). Li, J.Y., et al. J. Neurol. Sci. 290 (1-2), 163-165 (2010): Hour, T.C., et al. Int. J. Biol. Markers 24(3):171-178(2009). Shukla, P., et al. J. Child Neurol. 24(7):857-860(2009).

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