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40 Within this cycle, glutamine-derived carbon is converted to oxaloacetate and then to malate, which exits the mitochondria and is decarboxylated to NADPH and pyruvate by cytosolic malic enzyme (ME1), ultimately yielding lactate

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Proteins and genes involved in DIC include BSEP (ABCB11) and MDR3 (ABCB4) [19], which in homozygous form are responsible for paediatric PFIC2 and PFIC3

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