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Pathways of ammonia detoxification to GLN in muscles connected with enhanced usage from the BCAA and α-KG (cataplerosis) and instructed effects of BCAA and phenylbutyrate in topics with liver cirrhosis or UCD. Positive impact of BCAA on ammonia detoxification to GLN can be blunted by GLN degradation to ammonia in enterocytes and kidneys. Phenylbut