Methods of modulation of branched chain acids and uses thereof
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United States of America Patent
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Aug 22, 2017
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app pub date -
Jun 19, 2015
filing date -
Jul 24, 2009
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Abstract
A method of modulating plasma levels of branched chain amino acids and branched chain alpha-keto acids is disclosed, wherein an ammonia scavenger compound or a salt thereof, for example phenylbutyrate or an even numbered congener thereof or a salt thereof, is administered to an individual in need thereof. In various methods, a decrease in plasma levels of branched chain amino acids and branched chain alpha-keto acids is effected to treat individuals suffering from an inborn error in metabolism of amino acids, such as Maple Syrup Urine Disease, for example.
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