|Other Names||ATP-binding cassette sub-family D member 1, Adrenoleukodystrophy protein, ALDP, ABCD1, ALD|
|Format||Synthetic peptide was lyophilized with 100% acetonitrile and is supplied as a powder. Reconstitute with 0.1 ml DI water for a final concentration of 1 mg/ml.|
|Storage||Maintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C.|
|Precautions||This product is for research use only. 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 thesuperfamily of ATP-binding cassette (ABC) transporters. ABCproteins transport various molecules across extra- andintra-cellular membranes. ABC genes are divided into seven distinctsubfamilies (ABC1, MDR/TAP, MRP, ALD, OABP, GCN20, White). Thisprotein is a member of the ALD subfamily, which is involved inperoxisomal import of fatty acids and/or fatty acyl-CoAs in theorganelle. All known peroxisomal ABC transporters are halftransporters which require a partner half transporter molecule toform a functional homodimeric or heterodimeric transporter. Thisperoxisomal membrane protein is likely involved in the peroxisomaltransport or catabolism of very long chain fatty acids. Defects inthis gene have been identified as the underlying cause ofadrenoleukodystrophy, an X-chromosome recessively inheriteddemyelinating 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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