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Article

Tetrahydrobiopterin deficiency and brain nitric oxide synthase in the hph1 mouse.

Journal of inherited metabolic disease - 1 Jan 1995

Brand M P, Heales S J, Land J M, Clark J B

Abstract excerpt

Tetrahydrobiopterin (BH4) is the cofactor for the aromatic amino acid monoxygenase group of enzymes and for all known isoforms of nitric oxide synthase (NOS). Inborn errors of BH4 metabolism lead to hyperphenylalaninaemia and impaired catecholamine and serotonin turnover. The effects of BH4 deficiency on brain nitric oxide (NO) metabolism are not known. In this study we have used the hph-1 mouse, which displays...

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Identifiers

PMID: 7542713
DOI: 10.1007/BF00711370

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-08.1.

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Tetrahydrobiopterin deficiency and brain nitric oxide synthase in the hph1 mouse.PMID 7542713Read ↗

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Potential therapeutic applications of tetrahydrobiopterin: from inherited hyperphenylalaninemia to mitochondrial diseases.Semantic proximity Read ↗

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Mildly compromised tetrahydrobiopterin cofactor biosynthesis due to Pts variants leads to unusual body fat distribution and abdominal obesity in mice.Semantic proximity Read ↗

In vivo regulation of phenylalanine hydroxylase in the genetic mutant hph-1 mouse model.Semantic proximity Read ↗

Anxiety- and depression-like phenotype of hph-1 mice deficient in tetrahydrobiopterin.Semantic proximity Read ↗

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Erythropoietin increases bioavailability of tetrahydrobiopterin and protects cerebral microvasculature against oxidative stress induced by eNOS uncoupling.Semantic proximity Read ↗

The N-terminal peptide of mammalian GTP cyclohydrolase I is an autoinhibitory control element and contributes to binding the allosteric regulatory protein GFRP.Semantic proximity Read ↗

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