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Pathways of ammonia detoxification to GLN in muscles connected with enhanced use of your BCAA and α-KG (cataplerosis) and prompt consequences of BCAA and phenylbutyrate in subjects with liver cirrhosis or UCD. Good effect of BCAA on ammonia detoxification to GLN might be blunted by GLN degradation to ammonia in enterocytes and kidneys. Phenylbutyr