Reduced nitric oxide metabolites in CSF of patients with tetrahydrobiopterin deficiency.
J Neurochem
; 80(2): 362-4, 2002 Jan.
Article
in En
| MEDLINE
| ID: mdl-11902126
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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Collection:
01-internacional
Database:
MEDLINE
Main subject:
Phenylketonurias
/
Biopterins
/
Nitric Oxide
Limits:
Adolescent
/
Adult
/
Child
/
Child, preschool
/
Humans
/
Infant
Language:
En
Journal:
J Neurochem
Year:
2002
Document type:
Article
Affiliation country:
Suiza