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Identification of pharmacological chaperones as potential therapeutic agents to treat phenylketonuria

Phenylketonuria (PKU) is an inborn error of metabolism caused by mutations in phenylalanine hydroxylase (PAH). Over 500 disease-causing mutations have been identified in humans, most of which result in PAH protein misfolding and increased turnover in vivo. The use of pharmacological chaperones to st...

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Autors principals: Pey, Angel L., Ying, Ming, Cremades, Nunilo, Velazquez-Campoy, Adrian, Scherer, Tanja, Thöny, Beat, Sancho, Javier, Martinez, Aurora
Format: Artigo
Idioma:Inglês
Publicat: American Society for Clinical Investigation 2008
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Accés en línia:https://ncbi.nlm.nih.gov/pmc/articles/PMC2441854/
https://ncbi.nlm.nih.gov/pubmed/18596920
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1172/JCI34355
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