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Pathways of ammonia detoxification to GLN in muscles related to enhanced usage with the BCAA and α-KG (cataplerosis) and prompt effects of BCAA and phenylbutyrate in subjects with liver cirrhosis or UCD. Good result of BCAA on ammonia detoxification to GLN can be blunted by GLN degradation to ammonia in enterocytes and kidneys. Phenylbutyrate decr