Inhibitory effect of 3-hydroxyacyl-CoAs and other long-chain fatty acidβ-oxidation intermediates on mitochondrial oxidative phosphorylation (original) (raw)

Among the abnormal biochemical findings, a mild to severe lactic acidaemia is frequently described in patients with a long-chain fatty acid ~oxidation disorder, together with a markedly increased plasma long-chain acylcarnitine fraction, abnormal excretion of C6-Clo 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.