Fig. 5.

Effects of hyperammonemia on GLN and BCAA metabolism in skeletal muscle (Holecek et al. 2011). Hyperammonemia enhances GLN synthesis that may enhance the consumption of BCAA, resulting in their deficiency in extracellular fluid. BCAA deficiency may participate in the pathogenesis of muscle wasting and HE. 1 branched-chain aminotransferase; 2 branched-chain α-ketoacid dehydrogenase; 3 GLN synthetase. ECF The extracellular fluid; BCAA Branched-chain amino acids; BCKA Branched-chain keto acids; BCA-CoA Branched-chain acyl-CoA; αnKG α-ketoglutarate; Glu Glutamate; Ala Alanine