Abstract
We investigated CSF concentrations of nitrite and nitrate as indicators of nitric oxide (NO) production in patients with tetrahydrobiopterin (BH4) deficiencies. Patients with 6-pyruvoyl-tetrahydropterin synthase, sepiapterin reductase and dihydropteridine reductase deficiencies exhibited decreased CSF nitrite + nitrate levels compared with healthy control subjects. Reduced levels of nitrite + nitrate were not influenced by oral administration of 2.5-5.0 mg/kg tetrahydrobiopterin. Our data indicate impaired NO synthase function in patients with BH4 deficiency and suggest possible involvement in the neuronal cell dysfunction.
Original language | English |
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Pages (from-to) | 362-364 |
Number of pages | 3 |
Journal | Journal of Neurochemistry |
Volume | 80 |
Issue number | 2 |
DOIs | |
Publication status | Published - 2002 |
Keywords
- 6-Pyruvoyl-tetrahydropterin
- Dihydropteridine
- Neurotoxicity
- Nitric oxide synthase
- Peroxynitrite
- Sepiapterin
ASJC Scopus subject areas
- Biochemistry
- Cellular and Molecular Neuroscience