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Phenylketonuria: An Inborn Error of Phenylalanine Metabolism

Phenylketonuria (PKU) is an autosomal recessive inborn error of phenylalanine (Phe) metabolism resulting from deficiency of phenylalanine hydroxylase (PAH). Most forms of PKU and hyperphenylalaninaemia (HPA) are caused by mutations in the PAH gene on chromosome 12q23.2. Untreated PKU is associated w...

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Détails bibliographiques
Auteurs principaux: Williams, Robin A, Mamotte, Cyril DS, Burnett, John R
Format: Artigo
Langue:Inglês
Publié: The Australian Association of Clinical Biochemists 2008
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Accès en ligne:https://ncbi.nlm.nih.gov/pmc/articles/PMC2423317/
https://ncbi.nlm.nih.gov/pubmed/18566668
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