Protein and ammino acid metabolism is the object of two modules (Modules 6 and 7). In Module 6, the catabolism of C-skeleton of different amino acids with different organic acidurias as a result of multiple enzymatic defects, the oxidative deamination of aminoacids with formation of ammonia and the ammonia detoxification through the urea cycle are discussed. Both the biochemical bases of these metabolic pathways and the pathological conditions associated with their deficits are described with a particular focus on hyperammonemia and urea cycle disorders.
Authors: François Eyskens, Laurence Roosens, Guillem Pintos-Morell, Andrea Bordugo
