四氢生物素 (BH4) 的合成、回收、 salvage 和调节
中文名称
通路描述
四氢生物素 (BH4) 是芳香族氨基酸羟化酶和甘油醚单加氧酶等必需辅因子，并调节一氧化氮合酶 (NOS) 活性。先天性 BH4 缺乏症会导致苯丙酮尿症和大脑中多巴胺及神经递质缺乏。BH4 维持 L- 色氨酸氧化产生 NO 的酶促耦合。BH4 氧化为 BH2 会导致 NOS 解偶联，产生超氧化物 (O2.-) 而非 NO。超氧化物迅速与 NO 反应产生过氧亚硝酸盐，可进一步解偶联 NOS。
这些活性氧物种 (超氧化物和过氧亚硝酸盐) 可促进内皮细胞氧化应激增加，导致动脉粥样硬化和高血压 (Thony et al. 2000, Crabtree and Channon 2011, Schulz et al. 2008, Schmidt and Alp 2007)。BH4 的合成、回收和效应在此展示。BH4 的从头合成需要三种酶，回收需要两种酶。
这些活性氧物种 (超氧化物和过氧亚硝酸盐) 可促进内皮细胞氧化应激增加，导致动脉粥样硬化和高血压 (Thony et al. 2000, Crabtree and Channon 2011, Schulz et al. 2008, Schmidt and Alp 2007)。BH4 的合成、回收和效应在此展示。BH4 的从头合成需要三种酶，回收需要两种酶。
英文描述
Tetrahydrobiopterin (BH4) synthesis, recycling, salvage and regulation Tetrahydrobiopterin (BH4) is an essential co-factor for the aromatic amino acid hydroxylases and glycerol ether monooxygenase and it regulates nitric oxide synthase (NOS) activity. Inherited BH4 deficiency leads to hyperphenylalaninemia, and dopamine and neurotransmitter deficiency in the brain. BH4 maintains enzymatic coupling to L-arginine oxidation to produce NO. Oxidation of BH4 to BH2 results in NOS uncoupling, resulting in superoxide (O2.-) formation rather than NO. Superoxide rapidly reacts with NO to produce peroxynitrite which can further uncouple NOS.
These reactive oxygen species (superoxide and peroxynitrite) can contribute to increased oxidative stress in the endothelium leading to atherosclerosis and hypertension (Thony et al. 2000, Crabtree and Channon 2011,Schulz et al. 2008, Schmidt and Alp 2007). The synthesis, recycling and effects of BH4 are shown here. Three enzymes are required for the de novo biosynthesis of BH4 and two enzymes for the recycling of BH4.
These reactive oxygen species (superoxide and peroxynitrite) can contribute to increased oxidative stress in the endothelium leading to atherosclerosis and hypertension (Thony et al. 2000, Crabtree and Channon 2011,Schulz et al. 2008, Schmidt and Alp 2007). The synthesis, recycling and effects of BH4 are shown here. Three enzymes are required for the de novo biosynthesis of BH4 and two enzymes for the recycling of BH4.
所含基因
9 个基因