Table 5.

Biochemical changes caused by mitochondrial toxicity associated with drug-induced PLD and by phenylketonuria.

	Mitochondrial toxicity associated with drug-induced PLD	PKU
PAH	Downregulated at mRNA level for drug A and B	Deficit
Blood tyrosine levels	Decreased after high dose of drug A correlated with PAH down regulation at mRNA level	Decreased
TCA intermediates/
Fumarate is a link between TCA and urea cycles	Depletion in e.g. fumarate, malate, 2- oxoglutarate with high dose of drug A	Depletion in fumarate and 2-oxoglutarate
Urine	Low pH with drug A (dose dependent)	Ketone bodies
PAG	High with drug A (dose dependent)	High

PAH: phenylalanine hydroxylase; PLD: phospholipidosis; PKU: phenylketonuria; TCA: tricarboxylic acid