芳香族氨基酸羟化酶家族结构与催化功能

Structure and Catalytic Characters of Aromatic Amino Acid Hydroxylase

芳香族氨基酸羟化酶(AAAH)家族是一类单加氢酶,包括苯丙氨酸羟化酶(PAH)、酪氨酸羟化酶(TH)和色氨酸羟化酶(TPH).在辅因子四氢生物蝶呤、铁原子及氧存在下,分别催化苯丙氨酸、酪氨酸、色氨酸的羟化反应.多种疾病如苯丙酮尿症、帕金森氏病以及神经相关疾病的发病机制均与这类酶有关.本文综述近年来对芳香族氨基酸羟化酶家族蛋白结构功能、底物特异性、催化机制等方面的研究进展,为该类酶的定向进化及功能应用提供新思路.

The tetrahydropterin dependent aromatic amino acid hydroxylase （AAAH） is a family that include phenylalanine hydroxylase, tyrosine hydroxy/ase and tryptophan hydroxylase I or 2, which can catalyze hydroxylation reaction of aromatic amino acid at the presence of tetrahydropterin, iron and oxygen. Many diseases such as phenylketonuria, Parkinson＇ s disease, nervous disease and others are associated with AAAH. In this article, the progress of AAAH protein structure, functional domain, selectivity of substrate and catalytic mechanism are reviewed.