Pathways of ammonia detoxification to GLN in muscles connected with enhanced use from the BCAA and ?-KG (cataplerosis) and suggested results of BCAA and phenylbutyrate in topics with liver cirrhosis or UCD. Optimistic impact of BCAA on ammonia detoxification to GLN can be blunted by GLN degradation to ammonia in enterocytes and kidneys. Phenylbutyr