antibody

ABCD2, CT (ABCD2, ALD1, ALDL1, ALDR, ALDRP, ATP-binding cassette sub-family D member 2, Adrenoleukodystrophy-like 1, Adrenoleukodystrophy-related protein) (AP)

031481-AP

200 ul

polyclonal

domestic rabbit

AP

ABCD2, CT (ABCD2, ALD1, ALDL1, ALDR, ALDRP, ATP-binding cassette sub-family D member 2, Adrenoleukodystrophy-like 1, Adrenoleukodystrophy-related protein) (AP)

rabbit

Pab

AP

Antibodies

200 ul

IgG

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. The function of this peroxisomal membrane protein is unknown; however this protein is speculated to function as a dimerization partner of ABCD1 and/or other peroxisomal ABC transporters. Mutations in this gene have been observed in patients with adrenoleukodystrophy, a severe demyelinating disease. This gene has been identified as a candidate for a modifier gene, accounting for the extreme variation among adrenoleukodystrophy phenotypes. This gene is also a candidate for a complement group of Zellweger syndrome, a genetically heterogeneous disorder of peroxisomal biogenesis. Applications: Suitable for use in Western Blot, Immunohistochemistry and ELISA. Other applications have not been tested. Recommended Dilution: Immunohistochemistry: Formalin-fixed, paraffin-embedded sections Optimal dilutions to be determined by the researcher. Storage and Stability: Store product at 4°C. DO NOT FREEZE! Stable at 4°C for 12 months after receipt as an undiluted liquid. 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 prior to removing the cap. Note: Applications are based on unconjugated antibody.

Hu

ABCD2, ALD1, ALDL1, ALDR, ALDRP, ATP-binding cassette sub-family D member 2, Adrenoleukodystrophy-like 1, Adrenoleukodystrophy-related protein

KLH-conjugated synthetic peptide mapping to a fragment of residues within amino acids 553-582 in the C-terminal region of human ABCD2.

Recognizes human ABCD2.

Purified by Protein A and peptide affinity chromatography.

Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with Alkaline Phophatase (AP).

As Reported

Product Type: Pab Isotype: IgG Host: rabbit Source: human Concentration: As Reported Form: Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with Alkaline Phophatase (AP). Purity: Purified by Protein A and peptide affinity chromatography. Immunogen: KLH-conjugated synthetic peptide mapping to a fragment of residues within amino acids 553-582 in the C-terminal region of human ABCD2. Specificity: Recognizes human ABCD2. Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications without the expressed written authorization of United States Biological.

E IHC WB

4°C Do Not Freeze

1. Matsukawa, T., et al. Neurogenetics (2010). 2. Saito, A., et al. J. Hum. Genet. 54(6):317-323 (2009). 3. Maier, E.M., et al. Biochem. Biophys. Res. Commun. 377(1):176-180 (2008). 4. Lu, Y., et al. J. Lipid Res. 49(12):2582-2589 (2008). 5. Petroni, A., et al. J. Inherit. Metab. Dis. 30 (5), 828 (2007)

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