Pathways of ammonia detoxification to GLN in muscles linked to Improved usage in the BCAA and ?-KG (cataplerosis) and recommended effects of BCAA and phenylbutyrate in topics with liver cirrhosis or UCD. Beneficial influence of BCAA on ammonia detoxification to GLN can be blunted by GLN degradation to ammonia in enterocytes and kidneys. Phenylbutyr