中文摘要：

四氢生物蝶呤（BH4）属于芳香族氨基酸羟化酶的辅酶,是一氧化氮合酶的重要辅因子。在人体内中,BH4具有抗氧化和清除活性氮氧化物的功能,并在体内一系列生理和病理过程中起关键作用。研究表明,在糖尿病、肺动脉高压以及病理性心肌重塑等疾病的发生发展中,BH4生物利用度下降及内皮型一氧化氮合酶脱偶联所导致的血管内皮功能障碍扮演了重要角色。本文就目前BH4在上述疾病中的作用进行综述。

英文摘要：

Tetrahydrobiopterin（ BH4） is an essential cofactor for aromatic amino acid hydroxylases and all three nitric oxide synthase（ NOS） isoforms. It has protective effects as an antioxidant and scavenger of reactive nitrogen and oxygen species. It also serves as a cofactor in both normal physiologic and pathological states. In conditions of BH4 deficiency,endothelial nitric oxide synthase（ e NOS） becomes ＇uncoupled＇,which leads to endothelial dysfunction in diabetes,pulmonary hypertension and pathologic cardiac remodeling. In this review,we will discuss the pathophysiological role of BH4 in those diseases mentioned above.