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Pathways of ammonia detoxification to GLN in muscles affiliated with enhanced intake in the BCAA and α-KG (cataplerosis) and prompt results of BCAA and phenylbutyrate in topics with liver cirrhosis or UCD. Positive result of BCAA on ammonia detoxification to GLN may be blunted by GLN degradation to ammonia in enterocytes and kidneys. Phenylbutyrat