FComEx: Biopterin (PC000396)

Record Information

Version

1.0

Creation date

2015-10-09 22:30:26 UTC

Update date

2017-01-19 02:36:22 UTC

FoodComEx ID

PC000396

FoodDB Record

FDB022060

Chemical Information

Name

Biopterin

Description

Biopterin concentrations in cerebrospinal fluid from patients with Parkinson's disease, in which the nigrostriatal dopamine neurons degenerate, are lower than those from age-matched older controls. In hereditary progressive dystonia/DOPA-responsive dystonia, which is a dopamine deficiency caused by mutations in GTP cyclohydrolase I without neuronal cell death (Segawa's disease), biopterin in cerebrospinal fluid decrease in parallel owing to the decreased activity in GTP cyclohydrolase I (EC 3.5.4.16, is an enzyme that is part of the folate and biopterin biosynthesis pathways. It is responsible for the hydrolysis of guanosine triphosphate (GTP) to form 7,8-dihydroneopterin 3'-triphosphate. (Pteridines (1999), 10(1), 5-13.) Lowered levels of urinary biopterin concomitant with elevated serum phenylalanine concentration occur in a variant type of hyperphenylalaninemia caused by a deficiency of tetrahydrobiopterin (BH4), the obligatory cofactor for phenylalanine hydroxylase. The most frequent form of this cofactor deficiency is due to lack of 6-pyruvoyl-tetrahydropterin synthase (PTPS) activity, the second enzyme in the biosynthetic pathway for BH4. (PMID 8178819) The hepatic phenylalanine hydroxylating system consists of 3 essential components, phenylalanine hydroxylase, dihydropteridine reductase, and the nonprotein coenzyme, tetrahydrobiopterin. The reductase and the pterin coenzyme are also essential components of the tyrosine and tryptophan hydroxylating systems. There are 3 distinct forms of phenylketonuria or hyperphenylalaninemia, each caused by lack of 1 of these essential components. The variant forms of the disease that are caused by the lack of dihydropteridine reductase or tetrahydrobiopterin are characterized by severe neurol. deterioration, impaired functioning of tyrosine and tryptophan hydroxylases, and the resultant deficiency of tyrosine- and tryptophan-derived monoamine neurotransmitters in brain. (PMID 3930837) [HMDB]

CAS Number

22150-76-1

Structure

MOL SDF PDB SMILES InChI

Synonyms

Synonym	Source
(-)-Biopterin	hmdb
(1'R,1'S) Biopterin	hmdb
[S-(R,S)]-2-amino-6-(1,2-dihydroxypropyl)-4(1H)-Pteridinone	hmdb
2-amino-6-(L-erythro-1,2-dihydroxypropyl)-4(3H)-Pteridinone	hmdb
6-Biopterin	hmdb
Biopterin	hmdb
L-Biopterin	hmdb
L-erythro-Biopterin	hmdb
Pterin H B2	hmdb

Chemical Formula

C9H11N5O3

IUPAC name

2-amino-6-[(1R,2S)-1,2-dihydroxypropyl]-4,8-dihydropteridin-4-one

InChI Identifier

InChI=1S/C9H11N5O3/c1-3(15)6(16)4-2-11-7-5(12-4)8(17)14-9(10)13-7/h2-3,6,15-16H,1H3,(H3,10,11,13,14,17)/t3-,6-/m0/s1

InChI Key

LHQIJBMDNUYRAM-DZSWIPIPSA-N

Isomeric SMILES

C[C@H](O)[C@H](O)C1=NC2=C(NC(N)=NC2=O)N=C1

Average Molecular Weight

237.2153

Monoisotopic Molecular Weight

237.086189243

Chemical Taxonomy

Description

Belongs to the class of organic compounds known as biopterins and derivatives. These are coenzymes containing a 2-amino-pteridine-4-one derivative. They are mainly synthesized in several parts of the body, including the pineal gland.

Kingdom

Organic compounds

Super Class

Organoheterocyclic compounds

Class

Pteridines and derivatives

Sub Class

Pterins and derivatives

Direct Parent

Biopterins and derivatives

Alternative Parents