Article
Tetrahydrobiopterin availability, nitric oxide metabolism and glutathione status in the hph-1 mouse; implications for the pathogenesis and treatment of tetrahydrobiopterin deficiency states.
Journal of inherited metabolic disease - 1 Apr 2007
Lam A A J, Hyland K, Heales S J R
Abstract excerpt
Tetrahydrobiopterin (BH4) is an essential cofactor for all isoforms of nitric oxide synthase. While it is well established that BH4 deficiency states are associated with impairment of dopamine, serotonin and phenylalanine metabolism, less is known with regard to the effects of deficiency of the cofactor upon nitric oxide (NO) metabolism. In this study, we have evaluated the effects of partial BH4 deficiency upon...
Topics
- Animals
- Biological Availability
- Biopterins
- Brain
- Carrier Proteins
- GTP Cyclohydrolase
- Glutathione
- Kidney
- Lipopolysaccharides
- Liver
- Metabolism, Inborn Errors
- Mice
- Mutation
- Nitrates
- Nitric Oxide