Reduced nitric oxide metabolites in CSF of patients with tetrahydrobiopterin deficiency

Giovanna Zorzi; Beat Thöny; Nenad Blau

doi:10.1046/j.0022-3042.2001.00710.x

Reduced nitric oxide metabolites in CSF of patients with tetrahydrobiopterin deficiency

Giovanna Zorzi, Beat Thöny, Nenad Blau

Fondazione IRCCS Istituto Neurologico "C. Besta"

Research output: Contribution to journal › Article › peer-review

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
Pages (from-to)	362-364
Number of pages	3
Journal	Journal of Neurochemistry
Volume	80
Issue number	2
DOIs	https://doi.org/10.1046/j.0022-3042.2001.00710.x
Publication status	Published - 2002

Keywords

6-Pyruvoyl-tetrahydropterin
Dihydropteridine
Neurotoxicity
Nitric oxide synthase
Peroxynitrite
Sepiapterin

ASJC Scopus subject areas

Biochemistry
Cellular and Molecular Neuroscience

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10.1046/j.0022-3042.2001.00710.x

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title = "Reduced nitric oxide metabolites in CSF of patients with tetrahydrobiopterin deficiency",

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.",

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AU - Zorzi, Giovanna

AU - Thöny, Beat

AU - Blau, Nenad

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N2 - 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.

AB - 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.

KW - 6-Pyruvoyl-tetrahydropterin

KW - Dihydropteridine

KW - Neurotoxicity

KW - Nitric oxide synthase

KW - Peroxynitrite

KW - Sepiapterin

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Reduced nitric oxide metabolites in CSF of patients with tetrahydrobiopterin deficiency

Abstract

Keywords

ASJC Scopus subject areas

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