Showing metabocard for Dihydrobiopterin (HMDB00038)

General function:

Involved in oxidoreductase activity

Specific function:

This is a copper-containing oxidase that functions in the formation of pigments such as melanins and other polyphenolic compounds. Catalyzes the rate-limiting conversions of tyrosine to DOPA, DOPA to DOPA-quinone and possibly 5,6-dihydroxyindole to indole-5,6 quinone.

Gene Name:

TYR

Uniprot ID:

P14679

Molecular weight:

60392.69

References

1. Wood JM, Chavan B, Hafeez I, Schallreuter KU: Regulation of tyrosinase by tetrahydropteridines and H2O2. Biochem Biophys Res Commun. 2004 Dec 24;325(4):1412-7. [PubMed:15555584 ]

General function:

Involved in monooxygenase activity

Specific function:

Plays an important role in the physiology of adrenergic neurons.

Gene Name:

TH

Uniprot ID:

P07101

Molecular weight:

55611.26

References

1. Goodwill KE, Sabatier C, Stevens RC: Crystal structure of tyrosine hydroxylase with bound cofactor analogue and iron at 2.3 A resolution: self-hydroxylation of Phe300 and the pterin-binding site. Biochemistry. 1998 Sep 29;37(39):13437-45. [PubMed:9753429 ]

General function:

Involved in oxidoreductase activity

Specific function:

Catalyzes the final one or two reductions in tetra-hydrobiopterin biosynthesis to form 5,6,7,8-tetrahydrobiopterin.

Gene Name:

SPR

Uniprot ID:

P35270

Molecular weight:

28048.13

Reactions

References

1. Curtius HC, Heintel D, Ghisla S, Kuster T, Leimbacher W, Niederwieser A: Tetrahydrobiopterin biosynthesis. Studies with specifically labeled (2H)NAD(P)H and 2H2O and of the enzymes involved. Eur J Biochem. 1985 May 2;148(3):413-9. [PubMed:3888618 ]

General function:

Involved in amino acid binding

Specific function:

Not Available

Gene Name:

TPH1

Uniprot ID:

P17752

Molecular weight:

50984.725

References

1. Wang L, Erlandsen H, Haavik J, Knappskog PM, Stevens RC: Three-dimensional structure of human tryptophan hydroxylase and its implications for the biosynthesis of the neurotransmitters serotonin and melatonin. Biochemistry. 2002 Oct 22;41(42):12569-74. [PubMed:12379098 ]

General function:

Involved in oxidoreductase activity

Specific function:

The product of this enzyme, tetrahydrobiopterin (BH-4), is an essential cofactor for phenylalanine, tyrosine, and tryptophan hydroxylases.

Gene Name:

QDPR

Uniprot ID:

P09417

Molecular weight:

25789.295

General function:

Involved in dihydrofolate reductase activity

Specific function:

Key enzyme in folate metabolism. Contributes to the de novo mitochondrial thymidylate biosynthesis pathway. Catalyzes an essential reaction for de novo glycine and purine synthesis, and for DNA precursor synthesis. Binds its own mRNA and that of DHFRL1.

Gene Name:

DHFR

Uniprot ID:

P00374

Molecular weight:

21452.61

References

1. Bowers SW, Duch DS: In vivo measurement of dihydrofolate reductase and its inhibition by antifolates. Anal Biochem. 1988 Jul;172(1):169-75. [PubMed:3189762 ]

General function:

Involved in oxidoreductase activity

Specific function:

Produces nitric oxide (NO) which is a messenger molecule with diverse functions throughout the body. In the brain and peripheral nervous system, NO displays many properties of a neurotransmitter. Probably has nitrosylase activity and mediates cysteine S-nitrosylation of cytoplasmic target proteins such SRR.

Gene Name:

NOS1

Uniprot ID:

P29475

Molecular weight:

160969.095

References

1. Li H, Igarashi J, Jamal J, Yang W, Poulos TL: Structural studies of constitutive nitric oxide synthases with diatomic ligands bound. J Biol Inorg Chem. 2006 Sep;11(6):753-68. Epub 2006 Jun 28. [PubMed:16804678 ]

General function:

Involved in oxidoreductase activity

Specific function:

Produces nitric oxide (NO) which is implicated in vascular smooth muscle relaxation through a cGMP-mediated signal transduction pathway. NO mediates vascular endothelial growth factor (VEGF)-induced angiogenesis in coronary vessels and promotes blood clotting through the activation of platelets. Isoform eNOS13C: Lacks eNOS activity, dominant-negative form that may down-regulate eNOS activity by forming heterodimers with isoform 1.

Gene Name:

NOS3

Uniprot ID:

P29474

Molecular weight:

133273.59

References

1. Lowe ER, Everett AC, Lee AJ, Lau M, Dunbar AY, Berka V, Tsai AL, Osawa Y: Time-dependent inhibition and tetrahydrobiopterin depletion of endothelial nitric-oxide synthase caused by cigarettes. Drug Metab Dispos. 2005 Jan;33(1):131-8. Epub 2004 Oct 6. [PubMed:15470159 ]

General function:

Involved in 4-alpha-hydroxytetrahydrobiopterin dehydratase activity

Specific function:

Involved in tetrahydrobiopterin biosynthesis. Seems to both prevent the formation of 7-pterins and accelerate the formation of quinonoid-BH2. Coactivator for HNF1A-dependent transcription. Regulates the dimerization of homeodomain protein HNF1A and enhances its transcriptional activity.

Gene Name:

PCBD1

Uniprot ID:

P61457

Molecular weight:

11999.515

Reactions

General function:

Involved in 4-alpha-hydroxytetrahydrobiopterin dehydratase activity

Specific function:

Involved in tetrahydrobiopterin biosynthesis. Seems to both prevent the formation of 7-pterins and accelerate the formation of quinonoid-BH2 (By similarity). Regulates the dimerization of homeodomain protein HNF-1-alpha and enhances its transcriptional activity.

Gene Name:

PCBD2

Uniprot ID:

Q9H0N5

Molecular weight:

14365.325

Reactions

General function:

Not Available

Specific function:

Glyceryl-ether monooxygenase that cleaves the O-alkyl bond of ether lipids. Ether lipids are essential components of brain membranes.

Gene Name:

AGMO

Uniprot ID:

Q6ZNB7

Molecular weight:

51499.41

Reactions