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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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Detalhes bibliográficos
Main Authors: Williams, Robin A, Mamotte, Cyril DS, Burnett, John R
Formato: Artigo
Idioma:Inglês
Publicado em: The Australian Association of Clinical Biochemists 2008
Assuntos:
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC2423317/
https://ncbi.nlm.nih.gov/pubmed/18566668
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