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Largillière, C.; Vianey-Saban, C.; Fontaine, M.; Bertrand, C.; Kacet, N.; Farriaux, J. P.
Archives of disease in childhood, 09/1995, Letnik: 73, Številka: 2Journal Article
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.
