Accumulation of very long-chain fatty acids does not affect mitochondrial function in adrenoleukodystrophy protein deficiency.

@article{Oezen2005AccumulationOV,
  title={Accumulation of very long-chain fatty acids does not affect mitochondrial function in adrenoleukodystrophy protein deficiency.},
  author={Iris Oezen and Walter Rossmanith and Sonja Forss-Petter and Stephan Kemp and Till Voigtlaender and Karin Moser-Thier and Ronald J. A. Wanders and Reginald E. Bittner and Johannes Berger},
  journal={Human molecular genetics},
  year={2005},
  volume={14 9},
  pages={1127-37}
}
X-linked adrenoleukodystrophy (X-ALD, OMIM 300100) is a severe inherited neurodegenerative disease, associated with the accumulation of very long-chain fatty acids (VLCFA). The recent unexpected observation that the accumulation of VLCFA in tissues of the Abcd1-deficient mouse model for X-ALD is not due to a deficiency in VLCFA degradation, led to the hypothesis that mitochondrial abnormalities might contribute to X-ALD pathology. Here, we report that in spite of substantial accumulation of… CONTINUE READING

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