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Pathways of ammonia detoxification to GLN in muscles linked to Increased usage with the BCAA and α-KG (cataplerosis) and recommended outcomes of BCAA and phenylbutyrate in topics with liver cirrhosis or UCD. Good result of BCAA on ammonia detoxification to GLN may be blunted by GLN degradation to ammonia in enterocytes and kidneys. Phenylbutyrate