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Phenylalanine Hydroxylase Misfolding and Pharmacological Chaperones

Phenylketonuria (PKU) is a loss-of-function inborn error of metabolism. As many other inherited diseases the main pathologic mechanism in PKU is an enhanced tendency of the mutant phenylalanine hydroxylase (PAH) to misfold and undergo ubiquitin-dependent degradation. Recent alternative approaches wi...

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Detalles Bibliográficos
Autores principales:	Underhaug, Jarl, Aubi, Oscar, Martinez, Aurora
Formato:	Online Artículo Texto
Lenguaje:	English
Publicado:	Bentham Science Publishers 2012
Materias:	Article
Acceso en línea:	https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3664513/ https://www.ncbi.nlm.nih.gov/pubmed/23339306 http://dx.doi.org/10.2174/1568026611212220008

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