grantor: University of TorontoPhenylketonuria (PKU) is a disorder of aromatic amino acid metabolism, which results from a genetic mutation of the phenylalanine hydroxylase enzyme (PAH). PAH is the rate limiting enzyme which catalyzes the initial reaction in which phenylalanine is converted to tyrosine, or completely oxidized to CO2 and H2O. Although dietary restriction of phenylalanine is the main treatment in PKU, present estimates of requirements are based on plasma phenylalanine levels and growth. The present study aimed to more precisely determine phenylalanine requirement using Indicator Amino Acid Oxidation (IAAO), with L-[1-13C]lysine as the indicator. Breath 13CO2 production (F13CO2) was used as the endpoint. The mean phe...
Phenylketonuria is the most prevalent disorder caused by an inborn error in aminoacid metabolism. It...
Phenylketonuria (PKU) is an autosomal recessive inborn error of phenylalanine (Phe) metabolism resul...
There is a consensus on the importance of early and life-long treatment for PKU patients. Still, dif...
grantor: University of TorontoPhenylketonuria (PKU) is a disorder of aromatic amino acid m...
grantor: University of TorontoTyrosine requirements and resting energy expenditure (REE) w...
Phenylketonuria (PKU) is an inherited inborn error of phenylalanine (PHE) metabolism caused by defic...
Phenylketonuria (PKU) is the most common autosomal recessive disease. Hyperphenylalaninemia is cause...
Phenylketonuria (PKU; MIM 261600) is an autosomal recessive disorder of phenylalanine metabolism cau...
Purpose: Phenylketonuria (PKU) is an inherited metabolic disease caused by low levels of the enzyme ...
peer reviewedPhenylketonuria is an inherited metabolic disease, of autosomal recessive transmission,...
Phenylketonuria (PKU) is an autosomal recessive inborn error of metabolism caused by a deficiency in...
The same basic principles are used to deliver dietary treatment in PKU that was developed sixty year...
Phenylketonuria is the most common inborn error of amino acid metabolism. The defect is due to mutat...
Hyperphenylalaninaemia (HPA) is an inherited disorder that results in raised plasma phenylalanine le...
Phenylketonuria (PKU) is a metabolic disorder affecting the metabolism of essential aromatic amino a...
Phenylketonuria is the most prevalent disorder caused by an inborn error in aminoacid metabolism. It...
Phenylketonuria (PKU) is an autosomal recessive inborn error of phenylalanine (Phe) metabolism resul...
There is a consensus on the importance of early and life-long treatment for PKU patients. Still, dif...
grantor: University of TorontoPhenylketonuria (PKU) is a disorder of aromatic amino acid m...
grantor: University of TorontoTyrosine requirements and resting energy expenditure (REE) w...
Phenylketonuria (PKU) is an inherited inborn error of phenylalanine (PHE) metabolism caused by defic...
Phenylketonuria (PKU) is the most common autosomal recessive disease. Hyperphenylalaninemia is cause...
Phenylketonuria (PKU; MIM 261600) is an autosomal recessive disorder of phenylalanine metabolism cau...
Purpose: Phenylketonuria (PKU) is an inherited metabolic disease caused by low levels of the enzyme ...
peer reviewedPhenylketonuria is an inherited metabolic disease, of autosomal recessive transmission,...
Phenylketonuria (PKU) is an autosomal recessive inborn error of metabolism caused by a deficiency in...
The same basic principles are used to deliver dietary treatment in PKU that was developed sixty year...
Phenylketonuria is the most common inborn error of amino acid metabolism. The defect is due to mutat...
Hyperphenylalaninaemia (HPA) is an inherited disorder that results in raised plasma phenylalanine le...
Phenylketonuria (PKU) is a metabolic disorder affecting the metabolism of essential aromatic amino a...
Phenylketonuria is the most prevalent disorder caused by an inborn error in aminoacid metabolism. It...
Phenylketonuria (PKU) is an autosomal recessive inborn error of phenylalanine (Phe) metabolism resul...
There is a consensus on the importance of early and life-long treatment for PKU patients. Still, dif...