PKU mutation p.G46S prevents the stereospecific binding ofl-phenylalanine to the dimer of human phenylalanine hydroxylase regulatory domain

Author:

Leandro João12,Saraste Jaakko1,Leandro Paula2,Flatmark Torgeir1

Affiliation:

1. Department of Biomedicine; University of Bergen; Norway

2. Metabolism and Genetics Group; Research Institute for Medicines and Pharmaceutical Sciences (iMed.UL); Faculty of Pharmacy; University of Lisbon; Portugal

Funder

Fundação para a Ciência e a Tecnologia

Universitetet i Bergen

Publisher

Wiley

Subject

General Biochemistry, Genetics and Molecular Biology

Reference27 articles.

1. Structural insights into the regulation of aromatic amino acid hydroxylation;Fitzpatrick;Curr Opin Struct Biol,2015

2. Kinetic aspectes of rregulation of metabolic protesses. The hysteretic enzyme concept;Frieden;J Biol Chem,1970

3. Substrate activation of phenylalanine hydroxylase. A kinetic characterization;Shiman;J Biol Chem,1980

4. Structural basis of autoregulation of phenylalanine hydroxylase;Kobe;Nat Struct Biol,1999

5. Phenylalanine binding is linked to dimerization of the regulatory domain of phenylalanine hydroxylase;Zhang;Biochemistry,2014

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