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Pathways of ammonia detoxification to GLN in muscles linked to Increased intake with the BCAA and α-KG (cataplerosis) and advised effects of BCAA and phenylbutyrate in subjects with liver cirrhosis or UCD. Beneficial impact of BCAA on ammonia detoxification to GLN could be blunted by GLN degradation to ammonia in enterocytes and kidneys. Phenylbut