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Pathways of ammonia detoxification to GLN in muscles connected with Improved intake of the BCAA and α-KG (cataplerosis) and advised outcomes of BCAA and phenylbutyrate in subjects with liver cirrhosis or UCD. Beneficial outcome of BCAA on ammonia detoxification to GLN might be blunted by GLN degradation to ammonia in enterocytes and kidneys. Pheny