title: [Tetrahydrobiopterin Synthesis and Biological Function].
abstract: 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 (eNOS) 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.
authors.forename: Zhuo-fei
authors.initials: ZF
authors.lastname: Wang
authors.lastname: Chen Jin-Wen
authors.forename: Lu
authors.initials: L
authors.lastname: Tie
authors.forename: Xue-Jun
authors.initials: XJ
authors.lastname: Li
mesh_terms: D000975:Antioxidants; D001708:Biopterins / Q000031:analogs & derivatives* / Q000096:biosynthesis; D003920:Diabetes Mellitus; D006321:Heart; D006801:Humans; D006976:Hypertension, Pulmonary; D019001:Nitric Oxide Synthase; D052250:Nitric Oxide Synthase Type III
chemical_list: D000975:Antioxidants; D001708:Biopterins; C496314:NOS3 protein, human; D019001:Nitric Oxide Synthase; D052250:Nitric Oxide Synthase Type III; C003402:sapropterin