|Application ||WB, IHC-P, FC, E|
|Calculated MW||83233 Da|
|Antigen Region||553-582 aa|
|Other Names||ATP-binding cassette sub-family D member 2, Adrenoleukodystrophy-like 1, Adrenoleukodystrophy-related protein, hALDR, ABCD2, ALD1, ALDL1, ALDR, ALDRP|
|Target/Specificity||This ABCD2 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 553-582 amino acids from the C-terminal region of human ABCD2.|
|Format||Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification.|
|Storage||Maintain refrigerated at 2-8°C for up to 2 weeks. For long term storage store at -20°C in small aliquots to prevent freeze-thaw cycles.|
|Precautions||ABCD2 Antibody (C-term) is for research use only and not for use in diagnostic or therapeutic procedures.|
|Synonyms||ALD1, ALDL1, ALDR, ALDRP|
|Cellular Location||Peroxisome membrane; Multi-pass membrane protein|
|Tissue Location||Predominantly expressed in brain and heart.|
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Provided below are standard protocols that you may find useful for product applications.
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.
Matsukawa, T., et al. Neurogenetics (2010) In press :
Saito, A., et al. J. Hum. Genet. 54(6):317-323(2009)
Maier, E.M., et al. Biochem. Biophys. Res. Commun. 377(1):176-180(2008)
Lu, Y., et al. J. Lipid Res. 49(12):2582-2589(2008)
Petroni, A., et al. J. Inherit. Metab. Dis. 30 (5), 828 (2007) :
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