Therapy of X-linked adrenoleukodystrophy.

  title={Therapy of X-linked adrenoleukodystrophy.},
  author={Alexander Semmler and Wolfgang R. K{\"o}hler and Hans H. Jung and Michael Weller and Michael Linnebank},
  journal={Expert review of neurotherapeutics},
  volume={8 9},
X-linked adrenoleukodystrophy (X-ALD; OMIM #300100) is caused by defects of the ABCD1 gene on chromosome Xq28, resulting in an impairment of peroxisomal beta-oxidation and the accumulation of saturated very long chain fatty acids (VLCFAs). Primary manifestations occur in the CNS, the adrenal cortex and the testes' Leydig cells. The clinical presentation shows a marked variability which is not explained by the different X-ALD genotypes. Phenotypes range from rapidly progressive cerebral disease… CONTINUE READING
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Toxic effects of X - linked adrenoleukodystrophyassociated , very long chain fatty acids on glial cells and neurons from rat hippocampus in culture

  • P Schonfeld, S Kahlert, G Reiser
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