Phenylalanine hydroxylase: Function, structure, and regulation

Mammalian phenylalanine hydroxylase (PAH) catalyzes the rate‐limiting step in the phenylalanine catabolism, consuming about 75% of the phenylalanine input from the diet and protein catabolism under physiological conditions. In humans, mutations in the PAH gene lead to phenylketonuria (PKU), and most...

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Veröffentlicht in:	IUBMB life 2013-04, Vol.65 (4), p.341-349
Hauptverfasser:	Flydal, Marte I., Martinez, Aurora
Format:	Artikel
Sprache:	eng
Schlagworte:	Animals Biopterins - analogs & derivatives Biopterins - chemistry enzymology evolution Humans Kinetics Melanins - biosynthesis Melanins - chemistry Mutation Phenylalanine - chemistry Phenylalanine - metabolism Phenylalanine Hydroxylase - chemistry Phenylalanine Hydroxylase - genetics Phenylalanine Hydroxylase - metabolism Phenylketonurias - genetics Protein Conformation Protein Folding protein function protein structure
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