Fig. 1
From: Management of late onset urea cycle disorders—a remaining challenge for the intensivist?
Urea cycle disorders: NAGS: N-acetyl glutamate synthetase, CPS1: Carbamyl phosphate synthetase 1, OTC: Ornithine transcarbamylase, ORNT1 mitochondrial ornithine transporter 1, CITRIN: Citrullinemia type II (mitochondrial aspartate/glutamate) carrier, ASS: Arginosuccinic acid synthetase 1, ASL: Arginosuccinic acid lyase