Specifications
Immunogen
ABCD1 (AAH15541, 1 a.a. ~ 100 a.a) partial recombinant protein with GST tag. The sequence is MPVLSRPRPWRGNTLKRTAVLLALAAYGAHKVYPLVRQCLAPARGLQAPAGEPTQEASGV AAAKAGMNRVFLQRLLWLLRLLFPRVLCRETGLLALHSAA
Target
Alternative Names
ABCD1; ATP-binding cassette, sub-family D (ALD), member 1; ALD; AMN; ALDP; ABC42; ATP-binding cassette sub-family D member 1; adrenoleukodystrophy protein;
Product Background
Antigen Description
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. 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.
Pathway
ABC transporters; ABC-family proteins mediated transport; Beta-oxidation of very long chain fatty acids; Metabolism
Citations
Publication ()
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