Oxidation of unsaturated fatty acids by human fibroblasts with very-long-chain acyl-CoA dehydrogenase deficiency: aspects of substrate specificity and correlation with clinical phenotype.

@article{Roe2001OxidationOU,
  title={Oxidation of unsaturated fatty acids by human fibroblasts with very-long-chain acyl-CoA dehydrogenase deficiency: aspects of substrate specificity and correlation with clinical phenotype.},
  author={Diane S Roe and Christine Vianey-Saban and Seema Sharma and Marie-Th{\'e}r{\`e}se Zabot and Charles R. Roe},
  journal={Clinica chimica acta; international journal of clinical chemistry},
  year={2001},
  volume={312 1-2},
  pages={55-67}
}
The degradation of unsaturated fatty acids was examined in fibroblasts from 16 patients with very-long-chain acyl-CoA dehydrogenase (VLCAD) deficiency. Analysis of acylcarnitine intermediates following incubation of intact human cells with these compounds revealed that the milder clinical phenotypes could be distinguished from the severe cardiomyopathic phenotype. These findings may reflect more effective contributions of alternate pathways in the milder forms of the disease. Incubation of… CONTINUE READING

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