Inborn defects of the mitochondrial portion of the urea cycle.
D000123:Acetyltransferases / Q000172:deficiency; D051046:Amino-Acid N-Acetyltransferase; D000818:Animals; D001693:Biological Transport, Active; D002222:Carbamoyl-Phosphate Synthase (Ammonia) / Q000172:deficiency; D006801:Humans; D008661:Metabolism, Inborn Errors / Q000378:metabolism*; D008928:Mitochondria / Q000378:metabolism*; D009952:Ornithine / Q000378:metabolism; D020163:Ornithine Carbamoyltransferase Deficiency Disease; D014508:Urea / Q000378:metabolism*
D014508:Urea; D009952:Ornithine; D000123:Acetyltransferases; D051046:Amino-Acid N-Acetyltransferase; C494874:NAGS protein, human; D002222:Carbamoyl-Phosphate Synthase (Ammonia)