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Abbreviations 2-MCA: 2-methylcitrate ALT: Alanine transaminase AST: Aspartate transaminase CoA: Coenzyme A CPS-1: Carbamyl phosphate synthetase-1 GLN: Glutamine GLU: Glutamate H 2 O: Water HCO 3 : Bicarbonate IV: Intravenous IVA: Isovaleric acidaemia IV-CoA: Isovaleryl-CoA IVD: Isovaleryl-CoA dehydrogenase Leu: Leucine MMA: Methylmalonic acidaemia MM-CoA: Methylmalonyl-CoA MUT: Methylmalonyl-CoA mutase NAG: N-acetylglutamate NAGS: N-acetylglutamate synthase NCG: N-carbamyl-L-glutamate NH 3 : Ammonia OA: Organic acidaemia PA: Propionic acidaemia PC: Pyruvate carboxylase PCC: Propionyl-CoA carboxylase P-CoA: Propionyl-CoA PDH: Pyruvate dehydrogenase complex ROS: Reactive oxygen species TCA: Tricarboxylic acid UCD: Urea cycle disorder VAL: Valine References Dionisi-Vici C, Deodato F, Rschinger W, Rhead W, Wilcken B