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