3-(2-Thienyl)-L-Alanine as a Competitive Substrate Analogue and Activator of Human Phenylalanine Hydroxylase

Abstract

Phenylalanine hydroxylase (PAH) is a non-heme iron enzyme that catalyses the conversion of L-phenylalanine (L-Phe) to form L-tyrosine (L-Tyr) in the presence of the cofactor tetrahydrobiopterin (BH4) and dioxygen. It is well established that PAH is activated by its...