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Pathways of ammonia detoxification to GLN in muscles connected with Increased intake of your BCAA and α-KG (cataplerosis) and prompt outcomes of BCAA and phenylbutyrate in subjects with liver cirrhosis or UCD. Good result of BCAA on ammonia detoxification to GLN could be blunted by GLN degradation to ammonia in enterocytes and kidneys. Phenylbutyr