Article
Mouse very long-chain acyl-CoA synthetase in X-linked adrenoleukodystrophy.
The Journal of biological chemistry - 9 Aug 2002
Heinzer Ann K, Kemp Stephan, Lu Jyh-Feng, Watkins Paul A, Smith Kirby D
Abstract excerpt
X-linked adrenoleukodystrophy (X-ALD) is a neurodegenerative disorder characterized by accumulation of very long-chain fatty acids (VLCFA). This accumulation has been attributed to decreased VLCFA beta-oxidation and peroxisomal very long-chain acyl-CoA synthetase (VLCS) activity. The X-ALD gene, ABCD1, encodes a peroxisomal membrane ATP binding cassette transporter, ALDP, that is hypothesized to affect VLCS...
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