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Pathways of ammonia detoxification to GLN in muscles connected with enhanced consumption in the BCAA and α-KG (cataplerosis) and prompt results of BCAA and phenylbutyrate in subjects with liver cirrhosis or UCD. Good result of BCAA on ammonia detoxification to GLN might be blunted by GLN degradation to ammonia in enterocytes and kidneys. Phenylbut