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Pathways of ammonia detoxification to GLN in muscles connected with Increased usage in the BCAA and α-KG (cataplerosis) and suggested results of BCAA and phenylbutyrate in subjects with liver cirrhosis or UCD. Good outcome of BCAA on ammonia detoxification to GLN might be blunted by GLN degradation to ammonia in enterocytes and kidneys. Phenylbuty