Article
A mouse model for X-linked adrenoleukodystrophy.
Proceedings of the National Academy of Sciences of the United States of America - 19 Aug 1997
Lu J F, Lawler A M, Watkins P A, Powers J M, Moser A B, Moser H W, Smith K D
Abstract excerpt
X-linked adrenoleukodystrophy (X-ALD) is a peroxisomal disorder with impaired beta-oxidation of very long chain fatty acids (VLCFAs) and reduced function of peroxisomal very long chain fatty acyl-CoA synthetase (VLCS) that leads to severe and progressive neurological disability. The X-ALD gene, identified by positional cloning, encodes a peroxisomal membrane protein (adrenoleukodystrophy protein; ALDP) that...
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