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Pathways of ammonia detoxification to GLN in muscles related to enhanced usage on the BCAA and α-KG (cataplerosis) and instructed consequences of BCAA and phenylbutyrate in topics with liver cirrhosis or UCD. Positive effect of BCAA on ammonia detoxification to GLN could be blunted by GLN degradation to ammonia in enterocytes and kidneys. Phenylbu