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Pathways of ammonia detoxification to GLN in muscles linked to enhanced intake of the BCAA and α-KG (cataplerosis) and proposed effects of BCAA and phenylbutyrate in subjects with liver cirrhosis or UCD. Favourable result of BCAA on ammonia detoxification to GLN may very well be blunted by GLN degradation to ammonia in enterocytes and kidneys. Phe