ThePAH gene, phenylketonuria, and a paradigm shift

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The PAH gene, phenylketonuria, and a paradigm shift.

"Inborn errors of metabolism," first recognized 100 years ago by Garrod, were seen as transforming evidence for chemical and biological individuality. Phenylketonuria (PKU), a Mendelian autosomal recessive phenotype, was identified in 1934 by Asbjörn Fölling. It is a disease with impaired postnatal cognitive development resulting from a neurotoxic effect of hyperphenylalaninemia (HPA). Its meta...

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ژورنال

عنوان ژورنال: Human Mutation

سال: 2007

ISSN: 1059-7794,1098-1004

DOI: 10.1002/humu.20526