Abstract
Among the abnormal biochemical findings, a mild to severe lactic acidaemia (Treem et al 1994) is frequently described in patients with a long-chain fatty acid β-oxidation disorder, together with a markedly increased plasma long-chain acylcarnitine fraction, abnormal excretion of C 6 -C 10 dicarboxylic acids and an accumulation of medium- and long-chain 3-hydroxydicarboxylic acids (Duran et al 1991). Our aim was to explain the lactic acidosis found in these patients and to associate this with the accumulation of long-chain fatty acid derivatives. Previously (Ventura et al 1995) we have reported that long-chain (C 14 -C 20 ) acyl-CoA esters, both saturated and unsaturated, inhibit the synthesis of ATP in digitonin-permeabilized normal human fibroblasts (Wanders et al 1993). Using the same system we investigated in this work the effects of palmitoyl-CoA, α,β-unsaturated palmitoyl-CoA, 3-hydroxy- and 3-ketopalmitoyl-CoA on the oxidative phosphorylation system.
Published Version
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