Phenylketonuria Phenylalanine
the genetic disorder phenylketonuria (pku) inability metabolize phenylalanine because of lack of enzyme phenylalanine hydroxylase. individuals disorder known phenylketonurics , must regulate intake of phenylalanine. phenylketonurics use blood tests monitor amount of phenylalanine in blood. lab results may report phenylalanine levels using either mg/dl , μmol/l. 1 mg/dl of phenylalanine approximately equivalent 60 μmol/l. a (rare) variant form of phenylketonuria called hyperphenylalaninemia caused inability synthesize cofactor called tetrahydrobiopterin, can supplemented. pregnant women hyperphenylalaninemia may show similar symptoms of disorder (high levels of phenylalanine in blood) these indicators disappear @ end of gestation. pregnant women pku must control blood phenylalanine levels if fetus heterozygous defective gene because fetus adversely affected due hepatic immaturity. a non-food source of phenylalanine artificial sweetener aspartame. compound, sold under trade names equ...