Application of stable isotope tracers to examine phenylalanine metabolism and protein requirements in children with phenylketonuria (pku)
Phenylketonuria (PKU) is an inherited inborn error of phenylalanine (PHE) metabolism caused by deficiency of hepatic enzyme phenylalanine hydroxylase (PAH). Therefore, PHE accumulates in plasma leading to mental retardation and developmental delay. Kuvan® (Sapropterin dihydrochloride), a synthetic f...
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Language: | English |
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University of British Columbia
2015
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Online Access: | http://hdl.handle.net/2429/53095 |