Activation of Phenylalanine Hydroxylase Induces Positive Cooperativity toward the Natural Cofactor

Protein misfolding with loss-of-function of the enzyme phenylalanine hydroxylase (PAH) is the molecular basis of phenylketonuria in many individuals carrying missense mutations in the PAH gene. PAH is complexly regulated by its substrate l-Phenylalanine and its natural cofactor 6R-l-erythro-5,6,7,8-...

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Bibliographic Details
Main Authors: Gersting, Søren W., Staudigl, Michael, Truger, Marietta S., Messing, Dunja D., Danecka, Marta K., Sommerhoff, Christian P., Kemter, Kristina F., Muntau, Ania C.
Format: Artigo
Language:Inglês
Published: American Society for Biochemistry and Molecular Biology 2010
Subjects:
Enzymology
Online Access:https://ncbi.nlm.nih.gov/pmc/articles/PMC2945563/
https://ncbi.nlm.nih.gov/pubmed/20667834
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1074/jbc.M110.124016
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