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Fig. 5 | Nutrition & Metabolism

Fig. 5

From: Branched-chain amino acids in health and disease: metabolism, alterations in blood plasma, and as supplements

Fig. 5

Pathways of ammonia detoxification to GLN in muscles associated with enhanced consumption of the BCAA and α-KG (cataplerosis) and suggested effects of BCAA and phenylbutyrate in subjects with liver cirrhosis or UCD. Positive effect of BCAA on ammonia detoxification to GLN may be blunted by GLN degradation to ammonia in enterocytes and kidneys. Phenylbutyrate decreases ammonia via enhanced excretion of GLN by urine. An adverse side effect of phenylbutyrate is activation of BCKD resulting in the decrease of the BCAA. ALA, alanine; BCAA, branched-chain amino acids; BCKA, branched-chain keto acids. GLU, glutamate; GLN, glutamine; PYR, pyruvate; TCA cycle, tricarboxylic acid cycle; UCD, urea cycle disorders; α-KG, α-ketoglutarate. 1, branched-chain-amino-acid aminotransferase; 2, branched-chain α-keto acid dehydrogenase; 3, GLN synthetase

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