Tetrahydrobiopterin

used in the degradation of amino acid phenylalanine and in the biosynthesis of the neurotransmitters serotonin (5-hydroxytryptamine, 5-HT), melatonin, dopamine ...

Tetrahydrobiopterin: Beyond Its Traditional Role as a Cofactor - PMC

Tetrahydrobiopterin (BH4) is an endogenous cofactor for some enzymatic conversions of essential biomolecules, including nitric oxide, and monoamine ...

Tetrahydrobiopterin - an overview | ScienceDirect Topics

Tetrahydrobiopterin (Fig. 1) is an endogenous co-factor of eight human enzymes: four aromatic amino acid hydroxylases, three nitric oxide synthases and ...

Consensus guideline for the diagnosis and treatment of ...

Tetrahydrobiopterin (BH4) deficiencies comprise a group of six rare neurometabolic disorders characterized by insufficient synthesis of the ...

Tetrahydrobiopterin deficiency - Genetics - MedlinePlus

Tetrahydrobiopterin deficiency is a rare disorder characterized by a shortage (deficiency) of a molecule called tetrahydrobiopterin or BH4.

Tetrahydrobiopterin - an overview | ScienceDirect Topics

Tetrahydrobiopterin (BH4) is an essential cofactor required for the activity of endothelial nitric oxide (NO) synthase. Suboptimal concentrations of BH4 in the ...

Tetrahydrobiopterin: biochemistry and pathophysiology

During the catalytic event of aromatic amino acid hydroxylation, molecular oxygen is transferred to the corresponding amino acid and BH4 is ...

Tetrahydrobiopterin: biochemistry and pathophysiology - PubMed

BH4 (6R-L-erythro-5,6,7,8-tetrahydrobiopterin) is an essential cofactor of a set of enzymes that are of central metabolic importance, ...

Tetrahydrobiopterin Deficiency - Symptoms, Causes, Treatment

When tetrahydrobiopterin is deficient, the chemical balance within the body is upset. In most of these disorders, there are abnormally high levels of the amino ...

Sapropterin: Uses, Interactions, Mechanism of Action - DrugBank

Sapropterin (tetrahydrobiopterin or BH4) is a cofactor in the synthesis of nitric oxide. It is also essential in the conversion of phenylalanine to tyrosine ...

Showing metabocard for Tetrahydrobiopterin (HMDB0000027)

Showing metabocard for Tetrahydrobiopterin (HMDB0000027) enzymes (15) Show 15 proteins Record Information Show more accession numbers

Hyperphenylalaninemia due to tetrahydrobiopterin deficiency

A disorder of pterin metabolism characterized by tetrahydrobiopterin (BH4) biosynthesis or recycling deficiencies, leading to central dopamine and serotonin ...

Intestinal microbiota as a tetrahydrobiopterin exogenous source in ...

Tetrahydrobiopterin (BH4) is a cofactor of a number of regulatory enzymes. Although there are no known BH4 exogenous sources, ...

Tetrahydrobiopterin ((Rac)-Sapropterin) | Co-factor

Tetrahydrobiopterin ((Rac)-Sapropterin) is a cofactor of the aromatic amino acid hydroxylases enzymes and also acts as an essential cofactor for all nitric ...

Definition of tetrahydrobiopterin - NCI Drug Dictionary

Find technical definitions and synonyms by letter for drugs/agents used to treat patients with cancer or conditions related to cancer.

FFTEN - Overview: Tetrahydrobiopterin and Neopterin Profile (BH4, N)

Tetrahydrobiopterin and Neopterin Profile (BH4, N)

BH4 Deficiency (Tetrahydrobiopterin Deficiency)

Tetrahydrobiopterin (BH4) deficiencies are disorders that affect phenylalanine (Phe or F) homeostasis, as well as brain biosynthesis of ...

Tetrahydrobiopterin deficiency - Wikipedia

Tetrahydrobiopterin deficiency ... Tetrahydrobiopterin deficiency (THBD, BH4D) is a rare metabolic disorder that increases the blood levels of phenylalanine.

(6R)-5,6,7,8-Tetrahydrobiopterin 69056-38-8 - Sigma-Aldrich

98% (HPLC), powder CAS Number: 69056-38-8; Synonyms: (6R)-BH4 at Sigma-Aldrich.

Tetrahydrobiopterin and Cardiovascular Disease - AHA Journals

Oral sapropterin hydrochloride, the synthetic form of 6R-BH4, at 2 mg/kg causes a 3-fold increase in BH4 after 3 hours, returning to baseline at ...