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Arch Dis Child Fetal Neonatal Ed


Title:Mitochondrial very long chain acyl-CoA dehydrogenase deficiency--a new disorder of fatty acid oxidation
Author(s):Largilliere, C. Vianey-Saban, C. Fontaine, M. Bertrand, C. Kacet, N. Farriaux, J. P.
Address:Hopital Huriez, Lille, France, Department of Paediatrics, France.
Year:1995 Sep
Journal Title:Arch Dis Child Fetal Neonatal Ed
Page Number:F103-5
Language:eng
Volume:73
Issue:2
ISSN/ISBN:1359-2998 (Print). 1359-2998 (Linking)
Abstract: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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