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Mitochondrial very long chain acyl-CoA dehydrogenase deficiency--a new disorder of fatty acid oxidation.
  1. C. Largillière,
  2. C. Vianey-Saban,
  3. M. Fontaine,
  4. C. Bertrand,
  5. N. Kacet,
  6. J. P. Farriaux
  1. Hopital Huriez, Lille, France, Department of Paediatrics, France.


    Very long chain acyl-CoA dehydrogenase is a newly characterised enzyme in mitochondrial fatty acid oxidation. A girl who presented on the second day of life with a sudden and severe illness due to deficiency of this enzyme is reported. There is evidence that some children (and perhaps all) originally diagnosed with a deficiency of long-chain acyl-CoA dehydrogenase, in fact, have a defect involving very long chain acyl-CoA dehydrogenase.

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