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...

Full description

Bibliographic Details
Main Author: Turki, Abrar Mohammed
Language:English
Published: University of British Columbia 2015
Online Access:http://hdl.handle.net/2429/53095