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, i...
Topics
- Adrenoleukodystrophy
- Animals
- Disease Models, Animal
- Fatty Acids
- Gene Targeting
- Genetic Linkage
- Humans
- Mice
- Mutation
- Oxidation-Reduction
- X Chromosome