Amino Acid Medical Foods Provide a High Dietary Acid Load and Increase Urinary Excretion of Renal Net Acid, Calcium, and Magnesium Compared with Glycomacropeptide Medical Foods in Phenylketonuria
Introduction
PKU (PKU; OMIM 261600) is an inherited metabolic disorder characterized by high Phe concentrations in blood
due to mutations in the gene which encodes phenylalanine hydroxylase (PAH; EC 1.14.16.1). PAH catalyzes the hepatic conversion of Phe to Tyr using tetrahydrobiopterin as a cofactor [1]. Untreated PKU causes Phe to accumulate in the brain resulting in profound ...
