|Application ||WB, IHC-P, FC, E|
|Other Accession||P48410, NP_000024.2|
|Calculated MW||82937 Da|
|Antigen Region||257-285 aa|
|Other Names||ATP-binding cassette sub-family D member 1, Adrenoleukodystrophy protein, ALDP, ABCD1, ALD|
|Target/Specificity||This ABCD1 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 257-285 amino acids from the Central region of human ABCD1.|
|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||ABCD1 Antibody (Center) is for research use only and not for use in diagnostic or therapeutic procedures.|
|Function||Probable transporter. The nucleotide-binding fold acts as an ATP-binding subunit with ATPase activity.|
|Cellular Location||Peroxisome membrane; Multi-pass membrane protein|
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Provided below are standard protocols that you may find useful for product applications.
ABCD1 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.
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)
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