Abstract
Eight cases of hypoglycaemic,’ non-ketotic’ dicarboxylic aciduria have been proved by specific enzyme assays to be caused by a defect in general (medium chain) acyl-CoA dehydrogenase (Kølvraa et al., 1982; Divry et al., 1983; Rhead et al., 1983; Coates et al., 1982). The acyl-CoA dehydrogenases are flavoenzymes and in one of the cases it was shown that the binding of FAD to the apoenzyme was normal, indicating very strongly that the site of defect is localized to the apoenzyme (Kølvraa et al., 1982).
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© 1984 SSIEM and MTP Press Limited, Queen Square, Lancaster
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Gregersen, N., Kølvraa, S. (1984). Medium Chain Acyl-CoA Dehydrogenase Deficiency: Apparent K m and V max Values for Fibroblast Acyl-CoA Dehydrogenase towards Octanoyl CoA in Patient and Control Cell Lines. In: Addison, G.M., Chalmers, R.A., Divry, P., Harkness, R.A., Pollitt, R.J. (eds) Organic Acidurias. Springer, Dordrecht. https://doi.org/10.1007/978-94-009-5612-4_27
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DOI: https://doi.org/10.1007/978-94-009-5612-4_27
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