Pathways of ammonia detoxification to GLN in muscles connected with enhanced intake in the BCAA and ?-KG (cataplerosis) and suggested effects of BCAA and phenylbutyrate in topics with liver cirrhosis or UCD. Good influence of BCAA on ammonia detoxification to GLN may be blunted by GLN degradation to ammonia in enterocytes and kidneys. Phenylbutyrat