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Savic, T

Protection against the potentially severe toxicity of lithocholic acid is coordinated by the pregnane X receptor which has the potential to switch on all the genes required for its safe metabolism and elimination, including hydroxylation by cytochrome P450 3A, sulphation by sulphotransferase (SULT2A1) and transporter promoted excretion in faeces [17,18] (Fig

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Abnormalities in the acylcarnitine profile are more pronounced during times of metabolic derangement but the acylcarnitine profile can be normal in patients when they are well [36,37,38,39]