Tetrahydrobiopterin
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| Tetrahydrobiopterin | |
|---|---|
| Image:Tetrahydrobiopterin1.png | |
| Identifiers | |
| CAS number | |
| PubChem | |
| MeSH | |
| Properties | |
| Molecular formula | C9H15N5O3 |
| Molar mass | 241.247 |
| Except where noted otherwise, data are given for materials in their standard state (at 25 °C, 100 kPa) Infobox disclaimer and references | |
Tetrahydrobiopterin or BH4 is a cofactor in the synthesis of nitric oxide. It is also essential in the conversion of phenylalanine to tyrosine by the enzyme phenylalanine-4-hydroxylase; the conversion of tyrosine to L-dopa by the enzyme tyrosine hydroxylase; and conversion of tryptophan to 5-hydroxytryptophan via tryptophan hydroxylase.
Clinical significance
A defect in BH4 production and/or a defect in the enzyme dihydropteridine reductase (DHPR) causes phenylketonuria type IV, as well as dopa-responsive dystonias.
Synthesis
Tetrahydrobiopterin is synthesized from GTP via two intermediates.
References
Thony B, Auerbach G, Blau N (2000). "Tetrahydrobiopterin biosynthesis, regeneration and functions". Biochem J 347 Pt 1: 1-16. PMID 10727395.
See also
- Tetrahydrobiopterin deficiency This can also cause mental retardation as seen in patients with Phenylketonuria (PKU).
External links
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| Major families of biochemicals | ||
| Peptides | Amino acids | Nucleic acids | Carbohydrates | Nucleotide sugars | Lipids | Terpenes | Carotenoids | Tetrapyrroles | Enzyme cofactors | Steroids | Flavonoids | Alkaloids | Polyketides | Glycosides | ||
| Analogues of nucleic acids: | Types of enzyme cofactors | Analogues of nucleic acids: |
| Coenzymes | NAD+ | NADP+ | Coenzyme A | Tetrahydrofolic acid | Menaquinone | Ascorbic acid | Coenzyme F420 | Adenosine triphosphate | S-Adenosyl methionine | 3'-Phosphoadenosine-5'-phosphosulfate | Coenzyme Q | Tetrahydrobiopterin | Cytidine triphosphate | Glutathione | Coenzyme M | Coenzyme B | Methanofuran | Tetrahydromethanopterin | |
|---|---|---|
| Prosthetic groups | ||
| Organic groups: | Flavin mononucleotide | Flavin adenine dinucleotide | Pyrroloquinoline quinone | Pyridoxal phosphate | Biotin | Methylcobalamin | Cobamamide | Thiamine pyrophosphate | Heme | Molybdopterin | Lipoic acid | |
| Metals: | Calcium | Copper | Iron | Magnesium | Manganese | Nickel | Zinc | |
Acknowledgement and Attribution Regarding Sources of Content
Some of the initial content on this page may be incorporated in part from copyleft sources in the public domain including wikis such as Wikipedia and AskDrWiki. Drug information for patients came from the The National Library of Medicine. Infectious disease information may have come from the Centers for Disease Control (CDC). Differential Diagnoses are drawn from clinicians as well as an amalgamation of 3 sources: 1.The Disease Database; 2. Kahan, Scott, Smith, Ellen G. In A Page: Signs and Symptoms. Malden, Massachusetts: Blackwell Publishing, 2004:3; 3. Sailer, Christian, Wasner, Susanne. Differential Diagnosis Pocket. Hermosa Beach, CA: Borm Bruckmeir Publishing LLC, 2002:7 .
