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