Pathways of ammonia detoxification to GLN in muscles related to enhanced intake in the BCAA and ?-KG (cataplerosis) and suggested results of BCAA and phenylbutyrate in topics with liver cirrhosis or UCD. Optimistic effect of BCAA on ammonia detoxification to GLN may be blunted by GLN degradation to ammonia in enterocytes and kidneys. Phenylbutyrate