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

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£889.00

SKU:: 031481-PE
Purity:: Purified by Protein A and peptide affinity chromatography.
Storage Conditions:: 4°C Do Not Freeze
Supplier:: United States Biological
Host:: Rabbit
Immunogen:: KLH-conjugated synthetic peptide mapping to a fragment of residues within amino acids 553-582 in the C-terminal region of human ABCD2.
Format:: Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with R-Phycoerythrin (PE).
Extra Details:: 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, Flow Cytometry (Not Tested) and FLISA. 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 in the dark. 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. Caution: PE conjugates are sensitive to light. For maximum recovery of product, centrifuge the original vial prior to removing the cap. Note: Applications are based on unconjugated antibody.

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