Pathways of ammonia detoxification to GLN in muscles connected to Increased use with the BCAA and ?-KG (cataplerosis) and advised outcomes of BCAA and phenylbutyrate in topics with liver cirrhosis or UCD. Positive impact of BCAA on ammonia detoxification to GLN may be blunted by GLN degradation to ammonia in enterocytes and kidneys. Phenylbutyrate