7. A 22-year-old patient suffering argininosuccinic aciduria from little was prescribed the treatment by administration of keto-analogs of valine, leucine, isoleucine and phenylalanine against the...

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Extracted text: 7. A 22-year-old patient suffering argininosuccinic aciduria from little was prescribed the treatment by administration of keto-analogs of valine, leucine, isoleucine and phenylalanine against the background of reduced protein in the diet during two weeks. The doctor noted the decreasc of ammonia concentration in plasma from 0.09 mg/dL to 0.04 mg/dL as a result, and arginine succinate excretion was reduced from 2 to 0.8 g/24h. What is the molecular mechanism of the curative effeet of amino acids keto-analogs? For the answer: a) write down the scheme of ammonia detoxification in the liver; b) explain the cause of argininosuccinic aciduria; c) make up the scheme of present amino acid keto-analogs usage for the reduction of hyperammoniemia.