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Missense Mutations in the N-Terminal Domain of Human Phenylalanine Hydroxylase Interfere with Binding of Regulatory Phenylalanine

Hyperphenylalaninemia due to a deficiency of phenylalanine hydroxylase (PAH) is an autosomal recessive disorder caused by >400 mutations in the PAH gene. Recent work has suggested that the majority of PAH missense mutations impair enzyme activity by causing increased protein instability and aggre...

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Detalhes bibliográficos
Main Authors: Gjetting, Torben, Petersen, Marie, Guldberg, Per, Güttler, Flemming
Formato: Artigo
Idioma:Inglês
Publicado em: The American Society of Human Genetics 2001
Assuntos:
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC1226122/
https://ncbi.nlm.nih.gov/pubmed/11326337
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