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Pathways of ammonia detoxification to GLN in muscles related to enhanced use on the BCAA and α-KG (cataplerosis) and proposed results of BCAA and phenylbutyrate in topics with liver cirrhosis or UCD. Beneficial outcome of BCAA on ammonia detoxification to GLN could be blunted by GLN degradation to ammonia in enterocytes and kidneys. Phenylbutyrate

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