Lam A A J, Heales S J R
Neurometabolic Unit, National Hospital, Queen Square & Department of Molecular Neuroscience Institute of Neurology (UCL), Queen Square, London, UK.
Ann Clin Biochem. 2007 Jul;44(Pt 4):394-6. doi: 10.1258/000456307780945741.
Assessment of total neopterin and tetrahydrobioterin (BH4) concentrations in cerebrospinal fluid (CSF) can be used to identify potential disorders of BH4 biosynthesis. In this study, we demonstrate that exposure of CSF to nitric oxide leads to an accelerated degradation of BH4 but does not affect the total neopterin concentration. These data suggest that in those conditions associated with increased nitric oxide formation, perturbation of the total neopterin to BH4 ratio could occur. Under such circumstances a putative diagnosis of a defect in BH4 biosynthesis may erroneously be proposed. Assessment of central nitric oxide generation may therefore be a useful adjunct to the determination of CSF pterin status.
评估脑脊液（CSF）中总蝶呤和四氢生物蝶呤（BH4）的浓度可用于识别BH4生物合成的潜在紊乱。在本研究中，我们证明脑脊液暴露于一氧化氮会导致BH4加速降解，但不影响总蝶呤浓度。这些数据表明，在与一氧化氮生成增加相关的情况下，总蝶呤与BH4的比例可能会受到干扰。在这种情况下，可能会错误地提出BH4生物合成缺陷的假定诊断。因此，评估中枢一氧化氮的生成可能是确定脑脊液蝶呤状态的有用辅助手段。
