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Identification Taxonomy Ontology Physical properties Spectra Biological properties Concentrations Links References enzymes (11) Show 11 proteins XML

enzymes (11) Show 11 proteins

Record Information

Version

5.0

Status

Detected and Quantified

Creation Date

2005-11-16 15:48:42 UTC

Update Date

2021-09-14 15:40:00 UTC

HMDB ID

HMDB0000038

Secondary Accession Numbers

HMDB00038

Metabolite Identification

Common Name

Dihydrobiopterin

Description

Dihydrobiopterin, also known as BH2, 7,8-dihydrobiopterin, L-erythro-7,8-dihydrobiopterin, quinonoid dihydrobiopterin or q-BH2, belongs to the class of organic compounds known as biopterins and derivatives. These are coenzymes containing a 2-amino-pteridine-4-one derivative. Dihydrobiopterin is also classified as a pteridine. Pteridines are aromatic compounds composed of fused pyrimidine and pyrazine rings. Dihydrobiopterin is produced during the synthesis of neurotransmitters L-DOPA, dopamine, norepinephrine and epinephrine. It is restored to the required cofactor tetrahydrobiopterin via the NADPH-dependant reduction of dihydrobiopterin reductase. Dihydrobiopterin can also be converted to tetrahydrobiopterin by nitric oxide synthase (NOS) which is catalyzed by the flavoprotein "diaphorase" activity of NOS. This activity is located on the reductase (C-terminal) domain of NOS, whereas the high affinity tetrahydrobiopterin site involved in NOS activation is located on the oxygenase (N-terminal) domain (PMID: 8626754 ). Sepiapterin reductase (SPR) is another enzyme that plays a role in the production of dihydrobiopterin. SPR catalyzes the reduction of sepiapterin to dihydrobiopterin (BH2), the precursor for tetrahydrobiopterin (BH4). BH4 is a cofactor critical for nitric oxide biosynthesis and alkylglycerol and aromatic amino acid metabolism (PMID: 25550200 ). Dihydrobiopterin is known to be synthesized in several parts of the body, including the pineal gland. Dihydrobiopterin exists in all eukaryotes, ranging from yeast to humans. In humans, dihydrobiopterin is involved in several metabolic disorders including dihydropteridine reductase (DHPR) deficiency. DHPR deficiency is a severe form of hyperphenylalaninemia (HPA) due to impaired regeneration of tetrahydrobiopterin (BH4) leading to decreased levels of neurotransmitters (dopamine, serotonin) and folate in cerebrospinal fluid, and causing neurological symptoms such as psychomotor delay, hypotonia, seizures, abnormal movements, hypersalivation, and swallowing difficulties. Dihydrobiopterin is also associated with another metabolic disorder known as sepiapterin reductase deficiency (SRD). Sepiapterin reductase catalyzes the (NADP-dependent) reduction of carbonyl derivatives, including pteridines, and plays an important role in tetrahydrobiopterin biosynthesis. Low dihydrofolate reductase activity in the brain leads to the accumulation of dihydrobiopterin, which in turn, inhibits tyrosine and tryptophan hydroxylases. This uncouples neuronal nitric oxide synthase, leading to neurotransmitter deficiencies and neuronal cell death. SRD is characterized by low cerebrospinal fluid neurotransmitter levels and the presence of elevated cerebrospinal fluid dihydrobiopterin. SRD is characterized by motor delay, axial hypotonia, language delay, diurnal fluctuation of symptoms, dystonia, weakness, oculogyric crises, dysarthria, parkinsonian signs and hyperreflexia.

Structure

MOL 3D MOL SDF 3D SDF PDB 3D PDB SMILES InChI

HMDB0000038
     RDKit          3D
Dihydrobiopterin
 30 31  0  0  0  0  0  0  0  0999 V2000
   -3.1628    0.5544   -0.8718 C   0  0  0  0  0  0  0  0  0  0  0  0
   -3.1898   -0.5983    0.1175 C   0  0  2  0  0  0  0  0  0  0  0  0
   -4.4621   -0.6454    0.6850 O   0  0  0  0  0  0  0  0  0  0  0  0
   -2.1627   -0.4093    1.2061 C   0  0  2  0  0  0  0  0  0  0  0  0
   -2.3523    0.7550    1.9177 O   0  0  0  0  0  0  0  0  0  0  0  0
   -0.7630   -0.4274    0.6192 C   0  0  0  0  0  0  0  0  0  0  0  0
   -0.0479    0.6178    0.7798 N   0  0  0  0  0  0  0  0  0  0  0  0
    1.2711    0.7944    0.3113 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.8309   -0.2579   -0.3798 C   0  0  0  0  0  0  0  0  0  0  0  0
    3.0824   -0.2078   -0.8756 N   0  0  0  0  0  0  0  0  0  0  0  0
    3.8047    0.9081   -0.6871 C   0  0  0  0  0  0  0  0  0  0  0  0
    5.1393    0.9583   -1.2171 N   0  0  0  0  0  0  0  0  0  0  0  0
    3.3027    1.9641   -0.0183 N   0  0  0  0  0  0  0  0  0  0  0  0
    2.0373    1.9233    0.4873 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.5460    2.8814    1.1089 O   0  0  0  0  0  0  0  0  0  0  0  0
    1.0817   -1.4225   -0.5784 N   0  0  0  0  0  0  0  0  0  0  0  0
   -0.2493   -1.5737   -0.0945 C   0  0  0  0  0  0  0  0  0  0  0  0
   -2.1282    0.6178   -1.2570 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.5073    1.4878   -0.3858 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.8008    0.2568   -1.7289 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.0539   -1.5140   -0.4541 H   0  0  0  0  0  0  0  0  0  0  0  0
   -4.5421   -1.3494    1.3475 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.2233   -1.2568    1.9161 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.4575    1.5575    1.3833 H   0  0  0  0  0  0  0  0  0  0  0  0
    3.4817   -1.0182   -1.3946 H   0  0  0  0  0  0  0  0  0  0  0  0
    5.9433    1.0784   -0.5698 H   0  0  0  0  0  0  0  0  0  0  0  0
    5.2493    0.8705   -2.2505 H   0  0  0  0  0  0  0  0  0  0  0  0
    1.5482   -2.2311   -1.1240 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.2695   -2.5262    0.4918 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.9459   -1.7877   -0.9617 H   0  0  0  0  0  0  0  0  0  0  0  0
  1  2  1  0
  2  3  1  0
  2  4  1  0
  4  5  1  0
  4  6  1  0
  6  7  2  0
  7  8  1  0
  8  9  2  0
  9 10  1  0
 10 11  1  0
 11 12  1  0
 11 13  2  0
 13 14  1  0
 14 15  2  0
  9 16  1  0
 16 17  1  0
 17  6  1  0
 14  8  1  0
  1 18  1  0
  1 19  1  0
  1 20  1  0
  2 21  1  6
  3 22  1  0
  4 23  1  1
  5 24  1  0
 10 25  1  0
 12 26  1  0
 12 27  1  0
 16 28  1  0
 17 29  1  0
 17 30  1  0
M  END


  Mrv1652303182019222D          

 17 18  0  0  0  0            999 V2000
10023.383810026.0447    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
10026.236010025.2178    0.0000 C   0  0  2  0  0  0  0  0  0  0  0  0
10026.951710024.8031    0.0000 C   0  0  2  0  0  0  0  0  0  0  0  0
10027.665210025.2178    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
10026.951710023.9791    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
10026.236010026.0427    0.0000 O   0  0  0  0  0  0  0  0  0  0  0  0
10021.956710023.5613    0.0000 N   0  0  0  0  0  0  0  0  0  0  0  0
10023.383810025.2132    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
10022.669410024.8007    0.0000 N   0  0  0  0  0  0  0  0  0  0  0  0
10022.669410023.9757    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
10023.383810023.5633    0.0000 N   0  0  0  0  0  0  0  0  0  0  0  0
10024.810010025.2132    0.0000 N   0  0  0  0  0  0  0  0  0  0  0  0
10024.095510024.8007    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
10024.095510023.9757    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
10024.810010023.5633    0.0000 N   0  0  0  0  0  0  0  0  0  0  0  0
10025.524310023.9757    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
10025.524310024.8007    0.0000 C   0  0  0  0  0  0  0  0  0  0  0  0
  2  3  1  0  0  0  0
  2  6  1  1  0  0  0
  3  4  1  0  0  0  0
  3  5  1  6  0  0  0
  8  9  1  0  0  0  0
  9 10  2  0  0  0  0
 10 11  1  0  0  0  0
  1  8  2  0  0  0  0
 10  7  1  0  0  0  0
 13 14  2  0  0  0  0
 16 17  1  0  0  0  0
 12 13  1  0  0  0  0
 12 17  2  0  0  0  0
 14 15  1  0  0  0  0
 15 16  1  0  0  0  0
 17  2  1  0  0  0  0
 11 14  1  0  0  0  0
  8 13  1  0  0  0  0
M  END
> <DATABASE_ID>
HMDB0000038

> <DATABASE_NAME>
hmdb

> <SMILES>
C[C@H](O)[C@H](O)C1=NC2=C(NC1)NC(N)=NC2=O

> <INCHI_IDENTIFIER>
InChI=1S/C9H13N5O3/c1-3(15)6(16)4-2-11-7-5(12-4)8(17)14-9(10)13-7/h3,6,15-16H,2H2,1H3,(H4,10,11,13,14,17)/t3-,6-/m0/s1

> <INCHI_KEY>
FEMXZDUTFRTWPE-DZSWIPIPSA-N

> <FORMULA>
C9H13N5O3

> <MOLECULAR_WEIGHT>
239.2312

> <EXACT_MASS>
239.101839307

> <JCHEM_ACCEPTOR_COUNT>
8

> <JCHEM_ATOM_COUNT>
30

> <JCHEM_AVERAGE_POLARIZABILITY>
23.106840817612913

> <JCHEM_BIOAVAILABILITY>
1

> <JCHEM_DONOR_COUNT>
5

> <JCHEM_FORMAL_CHARGE>
0

> <JCHEM_GHOSE_FILTER>
0

> <JCHEM_IUPAC>
2-amino-6-[(1R,2S)-1,2-dihydroxypropyl]-1,4,7,8-tetrahydropteridin-4-one

> <ALOGPS_LOGP>
-1.43

> <JCHEM_LOGP>
-2.2885038219999996

> <ALOGPS_LOGS>
-2.17

> <JCHEM_MDDR_LIKE_RULE>
0

> <JCHEM_NUMBER_OF_RINGS>
2

> <JCHEM_PHYSIOLOGICAL_CHARGE>
0

> <JCHEM_PKA>
13.521765739396816

> <JCHEM_PKA_STRONGEST_ACIDIC>
9.910706687149771

> <JCHEM_PKA_STRONGEST_BASIC>
0.40083109372051184

> <JCHEM_POLAR_SURFACE_AREA>
132.32999999999998

> <JCHEM_REFRACTIVITY>
68.1133

> <JCHEM_ROTATABLE_BOND_COUNT>
2

> <JCHEM_RULE_OF_FIVE>
1

> <ALOGPS_SOLUBILITY>
1.63e+00 g/l

> <JCHEM_TRADITIONAL_IUPAC>
dihydrobiopterin

> <JCHEM_VEBER_RULE>
0

$$$$

HMDB0000038
     RDKit          3D
Dihydrobiopterin
 30 31  0  0  0  0  0  0  0  0999 V2000
   -3.1628    0.5544   -0.8718 C   0  0  0  0  0  0  0  0  0  0  0  0
   -3.1898   -0.5983    0.1175 C   0  0  2  0  0  0  0  0  0  0  0  0
   -4.4621   -0.6454    0.6850 O   0  0  0  0  0  0  0  0  0  0  0  0
   -2.1627   -0.4093    1.2061 C   0  0  2  0  0  0  0  0  0  0  0  0
   -2.3523    0.7550    1.9177 O   0  0  0  0  0  0  0  0  0  0  0  0
   -0.7630   -0.4274    0.6192 C   0  0  0  0  0  0  0  0  0  0  0  0
   -0.0479    0.6178    0.7798 N   0  0  0  0  0  0  0  0  0  0  0  0
    1.2711    0.7944    0.3113 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.8309   -0.2579   -0.3798 C   0  0  0  0  0  0  0  0  0  0  0  0
    3.0824   -0.2078   -0.8756 N   0  0  0  0  0  0  0  0  0  0  0  0
    3.8047    0.9081   -0.6871 C   0  0  0  0  0  0  0  0  0  0  0  0
    5.1393    0.9583   -1.2171 N   0  0  0  0  0  0  0  0  0  0  0  0
    3.3027    1.9641   -0.0183 N   0  0  0  0  0  0  0  0  0  0  0  0
    2.0373    1.9233    0.4873 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.5460    2.8814    1.1089 O   0  0  0  0  0  0  0  0  0  0  0  0
    1.0817   -1.4225   -0.5784 N   0  0  0  0  0  0  0  0  0  0  0  0
   -0.2493   -1.5737   -0.0945 C   0  0  0  0  0  0  0  0  0  0  0  0
   -2.1282    0.6178   -1.2570 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.5073    1.4878   -0.3858 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.8008    0.2568   -1.7289 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.0539   -1.5140   -0.4541 H   0  0  0  0  0  0  0  0  0  0  0  0
   -4.5421   -1.3494    1.3475 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.2233   -1.2568    1.9161 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.4575    1.5575    1.3833 H   0  0  0  0  0  0  0  0  0  0  0  0
    3.4817   -1.0182   -1.3946 H   0  0  0  0  0  0  0  0  0  0  0  0
    5.9433    1.0784   -0.5698 H   0  0  0  0  0  0  0  0  0  0  0  0
    5.2493    0.8705   -2.2505 H   0  0  0  0  0  0  0  0  0  0  0  0
    1.5482   -2.2311   -1.1240 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.2695   -2.5262    0.4918 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.9459   -1.7877   -0.9617 H   0  0  0  0  0  0  0  0  0  0  0  0
  1  2  1  0
  2  3  1  0
  2  4  1  0
  4  5  1  0
  4  6  1  0
  6  7  2  0
  7  8  1  0
  8  9  2  0
  9 10  1  0
 10 11  1  0
 11 12  1  0
 11 13  2  0
 13 14  1  0
 14 15  2  0
  9 16  1  0
 16 17  1  0
 17  6  1  0
 14  8  1  0
  1 18  1  0
  1 19  1  0
  1 20  1  0
  2 21  1  6
  3 22  1  0
  4 23  1  1
  5 24  1  0
 10 25  1  0
 12 26  1  0
 12 27  1  0
 16 28  1  0
 17 29  1  0
 17 30  1  0
M  END

HEADER    PROTEIN                                 18-MAR-20   NONE
TITLE     NULL                                                        
COMPND    NULL                                                        
SOURCE    NULL                                                        
KEYWDS    NULL                                                        
EXPDTA    NULL                                                        
AUTHOR    Marvin                                                      
REVDAT   1   18-MAR-20         0                                  
HETATM    1  O   UNK     0    8710.3168715.283   0.000  0.00  0.00           O+0
HETATM    2  C   UNK     0    8715.6418713.740   0.000  0.00  0.00           C+0
HETATM    3  C   UNK     0    8716.9778712.966   0.000  0.00  0.00           C+0
HETATM    4  C   UNK     0    8718.3088713.740   0.000  0.00  0.00           C+0
HETATM    5  O   UNK     0    8716.9778711.428   0.000  0.00  0.00           O+0
HETATM    6  O   UNK     0    8715.6418715.280   0.000  0.00  0.00           O+0
HETATM    7  N   UNK     0    8707.6538710.648   0.000  0.00  0.00           N+0
HETATM    8  C   UNK     0    8710.3168713.731   0.000  0.00  0.00           C+0
HETATM    9  N   UNK     0    8708.9838712.961   0.000  0.00  0.00           N+0
HETATM   10  C   UNK     0    8708.9838711.421   0.000  0.00  0.00           C+0
HETATM   11  N   UNK     0    8710.3168710.651   0.000  0.00  0.00           N+0
HETATM   12  N   UNK     0    8712.9798713.731   0.000  0.00  0.00           N+0
HETATM   13  C   UNK     0    8711.6458712.961   0.000  0.00  0.00           C+0
HETATM   14  C   UNK     0    8711.6458711.421   0.000  0.00  0.00           C+0
HETATM   15  N   UNK     0    8712.9798710.651   0.000  0.00  0.00           N+0
HETATM   16  C   UNK     0    8714.3128711.421   0.000  0.00  0.00           C+0
HETATM   17  C   UNK     0    8714.3128712.961   0.000  0.00  0.00           C+0
CONECT    1    8                                                            
CONECT    2    3    6   17                                                  
CONECT    3    2    4    5                                                  
CONECT    4    3                                                            
CONECT    5    3                                                            
CONECT    6    2                                                            
CONECT    7   10                                                            
CONECT    8    9    1   13                                                  
CONECT    9    8   10                                                       
CONECT   10    9   11    7                                                  
CONECT   11   10   14                                                       
CONECT   12   13   17                                                       
CONECT   13   14   12    8                                                  
CONECT   14   13   15   11                                                  
CONECT   15   14   16                                                       
CONECT   16   17   15                                                       
CONECT   17   16   12    2                                                  
MASTER        0    0    0    0    0    0    0    0   17    0   36    0
END

COMPND    HMDB0000038
HETATM    1  C1  UNL     1      -3.163   0.554  -0.872  1.00  0.00           C  
HETATM    2  C2  UNL     1      -3.190  -0.598   0.118  1.00  0.00           C  
HETATM    3  O1  UNL     1      -4.462  -0.645   0.685  1.00  0.00           O  
HETATM    4  C3  UNL     1      -2.163  -0.409   1.206  1.00  0.00           C  
HETATM    5  O2  UNL     1      -2.352   0.755   1.918  1.00  0.00           O  
HETATM    6  C4  UNL     1      -0.763  -0.427   0.619  1.00  0.00           C  
HETATM    7  N1  UNL     1      -0.048   0.618   0.780  1.00  0.00           N  
HETATM    8  C5  UNL     1       1.271   0.794   0.311  1.00  0.00           C  
HETATM    9  C6  UNL     1       1.831  -0.258  -0.380  1.00  0.00           C  
HETATM   10  N2  UNL     1       3.082  -0.208  -0.876  1.00  0.00           N  
HETATM   11  C7  UNL     1       3.805   0.908  -0.687  1.00  0.00           C  
HETATM   12  N3  UNL     1       5.139   0.958  -1.217  1.00  0.00           N  
HETATM   13  N4  UNL     1       3.303   1.964  -0.018  1.00  0.00           N  
HETATM   14  C8  UNL     1       2.037   1.923   0.487  1.00  0.00           C  
HETATM   15  O3  UNL     1       1.546   2.881   1.109  1.00  0.00           O  
HETATM   16  N5  UNL     1       1.082  -1.422  -0.578  1.00  0.00           N  
HETATM   17  C9  UNL     1      -0.249  -1.574  -0.094  1.00  0.00           C  
HETATM   18  H1  UNL     1      -2.128   0.618  -1.257  1.00  0.00           H  
HETATM   19  H2  UNL     1      -3.507   1.488  -0.386  1.00  0.00           H  
HETATM   20  H3  UNL     1      -3.801   0.257  -1.729  1.00  0.00           H  
HETATM   21  H4  UNL     1      -3.054  -1.514  -0.454  1.00  0.00           H  
HETATM   22  H5  UNL     1      -4.542  -1.349   1.347  1.00  0.00           H  
HETATM   23  H6  UNL     1      -2.223  -1.257   1.916  1.00  0.00           H  
HETATM   24  H7  UNL     1      -2.458   1.557   1.383  1.00  0.00           H  
HETATM   25  H8  UNL     1       3.482  -1.018  -1.395  1.00  0.00           H  
HETATM   26  H9  UNL     1       5.943   1.078  -0.570  1.00  0.00           H  
HETATM   27  H10 UNL     1       5.249   0.871  -2.251  1.00  0.00           H  
HETATM   28  H11 UNL     1       1.548  -2.231  -1.124  1.00  0.00           H  
HETATM   29  H12 UNL     1      -0.269  -2.526   0.492  1.00  0.00           H  
HETATM   30  H13 UNL     1      -0.946  -1.788  -0.962  1.00  0.00           H  
CONECT    1    2   18   19   20
CONECT    2    3    4   21
CONECT    3   22
CONECT    4    5    6   23
CONECT    5   24
CONECT    6    7    7   17
CONECT    7    8
CONECT    8    9    9   14
CONECT    9   10   16
CONECT   10   11   25
CONECT   11   12   13   13
CONECT   12   26   27
CONECT   13   14
CONECT   14   15   15
CONECT   16   17   28
CONECT   17   29   30
END

HMDB0000038
     RDKit          3D
Dihydrobiopterin
 30 31  0  0  0  0  0  0  0  0999 V2000
   -3.1628    0.5544   -0.8718 C   0  0  0  0  0  0  0  0  0  0  0  0
   -3.1898   -0.5983    0.1175 C   0  0  2  0  0  0  0  0  0  0  0  0
   -4.4621   -0.6454    0.6850 O   0  0  0  0  0  0  0  0  0  0  0  0
   -2.1627   -0.4093    1.2061 C   0  0  2  0  0  0  0  0  0  0  0  0
   -2.3523    0.7550    1.9177 O   0  0  0  0  0  0  0  0  0  0  0  0
   -0.7630   -0.4274    0.6192 C   0  0  0  0  0  0  0  0  0  0  0  0
   -0.0479    0.6178    0.7798 N   0  0  0  0  0  0  0  0  0  0  0  0
    1.2711    0.7944    0.3113 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.8309   -0.2579   -0.3798 C   0  0  0  0  0  0  0  0  0  0  0  0
    3.0824   -0.2078   -0.8756 N   0  0  0  0  0  0  0  0  0  0  0  0
    3.8047    0.9081   -0.6871 C   0  0  0  0  0  0  0  0  0  0  0  0
    5.1393    0.9583   -1.2171 N   0  0  0  0  0  0  0  0  0  0  0  0
    3.3027    1.9641   -0.0183 N   0  0  0  0  0  0  0  0  0  0  0  0
    2.0373    1.9233    0.4873 C   0  0  0  0  0  0  0  0  0  0  0  0
    1.5460    2.8814    1.1089 O   0  0  0  0  0  0  0  0  0  0  0  0
    1.0817   -1.4225   -0.5784 N   0  0  0  0  0  0  0  0  0  0  0  0
   -0.2493   -1.5737   -0.0945 C   0  0  0  0  0  0  0  0  0  0  0  0
   -2.1282    0.6178   -1.2570 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.5073    1.4878   -0.3858 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.8008    0.2568   -1.7289 H   0  0  0  0  0  0  0  0  0  0  0  0
   -3.0539   -1.5140   -0.4541 H   0  0  0  0  0  0  0  0  0  0  0  0
   -4.5421   -1.3494    1.3475 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.2233   -1.2568    1.9161 H   0  0  0  0  0  0  0  0  0  0  0  0
   -2.4575    1.5575    1.3833 H   0  0  0  0  0  0  0  0  0  0  0  0
    3.4817   -1.0182   -1.3946 H   0  0  0  0  0  0  0  0  0  0  0  0
    5.9433    1.0784   -0.5698 H   0  0  0  0  0  0  0  0  0  0  0  0
    5.2493    0.8705   -2.2505 H   0  0  0  0  0  0  0  0  0  0  0  0
    1.5482   -2.2311   -1.1240 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.2695   -2.5262    0.4918 H   0  0  0  0  0  0  0  0  0  0  0  0
   -0.9459   -1.7877   -0.9617 H   0  0  0  0  0  0  0  0  0  0  0  0
  1  2  1  0
  2  3  1  0
  2  4  1  0
  4  5  1  0
  4  6  1  0
  6  7  2  0
  7  8  1  0
  8  9  2  0
  9 10  1  0
 10 11  1  0
 11 12  1  0
 11 13  2  0
 13 14  1  0
 14 15  2  0
  9 16  1  0
 16 17  1  0
 17  6  1  0
 14  8  1  0
  1 18  1  0
  1 19  1  0
  1 20  1  0
  2 21  1  6
  3 22  1  0
  4 23  1  1
  5 24  1  0
 10 25  1  0
 12 26  1  0
 12 27  1  0
 16 28  1  0
 17 29  1  0
 17 30  1  0
M  END

View in JSmol View Stereo Labels

Synonyms

Value	Source
7,8-DIHYDROBIOPTERIN	ChEBI
L-Erythro-Q-dihydrobiopterin	ChEBI
Q-BH2	ChEBI
Quinonoid dihydrobiopterin	ChEBI
L-Erythro-7,8-dihydrobiopterin	Kegg
L-Erythro-dihydrobiopterin	HMDB
7,8-Dihydro-L-biopterin	HMDB
BH2	HMDB
Dihydrobiopterin	ChEBI

Chemical Formula

C₉H₁₃N₅O₃

Average Molecular Weight

239.2312

Monoisotopic Molecular Weight

239.101839307

IUPAC Name

2-amino-6-[(1R,2S)-1,2-dihydroxypropyl]-1,4,7,8-tetrahydropteridin-4-one

Traditional Name

dihydrobiopterin

CAS Registry Number

6779-87-9

SMILES

C[C@H](O)[C@H](O)C1=NC2=C(NC1)NC(N)=NC2=O

InChI Identifier

InChI=1S/C9H13N5O3/c1-3(15)6(16)4-2-11-7-5(12-4)8(17)14-9(10)13-7/h3,6,15-16H,2H2,1H3,(H4,10,11,13,14,17)/t3-,6-/m0/s1

InChI Key

FEMXZDUTFRTWPE-DZSWIPIPSA-N

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

Substituents

Biopterin
Secondary aliphatic/aromatic amine
Hydroxypyrimidine
Pyrimidine
Heteroaromatic compound
1,2-diol
Ketimine
Secondary alcohol
Azacycle
Organic 1,3-dipolar compound
Propargyl-type 1,3-dipolar organic compound
Secondary amine
Hydrocarbon derivative
Organic oxygen compound
Organooxygen compound
Organonitrogen compound
Organopnictogen compound
Imine
Organic nitrogen compound
Amine
Alcohol
Aromatic heteropolycyclic compound

Molecular Framework

Aromatic heteropolycyclic compounds

External Descriptors

7,8-dihydrobiopterin (CHEBI:43029 )

Ontology

Physiological effect

Adverse health effect

Digestive system disorder

Renal disorder

Kidney disease - Elevated in human blood (PMID: 12032193 ).

Nervous system disorder

Tyrosine hydroxylase deficiency

Genetic disorder

Inborn errors of metabolism

Sepiapterin reductase deficiency

Disposition

Biological location

Excreta

Feces

Cellular substructure

Macromolecular complex

Cytoplasm

Ingestion

Process

Naturally occurring process

Biological process

Biochemical pathway

Metabolic pathway

Physical Properties

State

Solid

Experimental Molecular Properties

Property	Value	Reference
Melting Point	Not Available	Not Available
Boiling Point	Not Available	Not Available
Water Solubility	Not Available	Not Available
LogP	Not Available	Not Available

Experimental Spectral Properties

Experimental Collision Cross Sections

Predictor	Adduct Type	Data Source	CCS Value (Å²)	Reference
AllCCS	[M+H]+	Not Available	154.268	http://allccs.zhulab.cn/database/detail?ID=AllCCS00000461

Predicted Molecular Properties

Property	Value	Source
Water Solubility	1.63 g/L	ALOGPS
logP	10^(-1.4) g/L	ALOGPS
logP	10^(-2.3) g/L	ChemAxon
logS	10^(-2.2) g/L	ALOGPS
pKa (Strongest Acidic)	9.91	ChemAxon
pKa (Strongest Basic)	0.4	ChemAxon
Physiological Charge	0	ChemAxon
Hydrogen Acceptor Count	8	ChemAxon
Hydrogen Donor Count	5	ChemAxon
Polar Surface Area	132.33 Å²	ChemAxon
Rotatable Bond Count	2	ChemAxon
Refractivity	68.11 m³·mol⁻¹	ChemAxon
Polarizability	23.11 Å³	ChemAxon
Number of Rings	2	ChemAxon
Bioavailability	Yes	ChemAxon
Rule of Five	Yes	ChemAxon
Ghose Filter	No	ChemAxon
Veber's Rule	No	ChemAxon
MDDR-like Rule	No	ChemAxon

Predicted Spectral Properties

Predicted Kovats Retention Indices

Derivatized

Derivative Name / Structure	SMILES	Kovats RI Value	Column Type	Reference
Dihydrobiopterin,1TMS,#1	C[C@H](O[Si](C)(C)C)[C@H](O)C1=NC2=C(NC1)[NH]C(N)=NC2=O	2388.3	Semi standard non polar	https://arxiv.org/abs/1905.12712
Dihydrobiopterin,1TMS,#2	C[C@H](O)[C@H](O[Si](C)(C)C)C1=NC2=C(NC1)[NH]C(N)=NC2=O	2373.3	Semi standard non polar	https://arxiv.org/abs/1905.12712
Dihydrobiopterin,1TMS,#3	C[C@H](O)[C@H](O)C1=NC2=C(NC1)[NH]C(N[Si](C)(C)C)=NC2=O	2453.6	Semi standard non polar	https://arxiv.org/abs/1905.12712
Dihydrobiopterin,1TMS,#4	C[C@H](O)[C@H](O)C1=NC2=C([NH]C(N)=NC2=O)N([Si](C)(C)C)C1	2307.3	Semi standard non polar	https://arxiv.org/abs/1905.12712
Dihydrobiopterin,1TMS,#5	C[C@H](O)[C@H](O)C1=NC2=C(NC1)N([Si](C)(C)C)C(N)=NC2=O	2381.1	Semi standard non polar	https://arxiv.org/abs/1905.12712
Dihydrobiopterin,2TMS,#1	C[C@H](O[Si](C)(C)C)[C@H](O[Si](C)(C)C)C1=NC2=C(NC1)[NH]C(N)=NC2=O	2330.3	Semi standard non polar	https://arxiv.org/abs/1905.12712
Dihydrobiopterin,2TMS,#2	C[C@H](O[Si](C)(C)C)[C@H](O)C1=NC2=C(NC1)[NH]C(N[Si](C)(C)C)=NC2=O	2348.6	Semi standard non polar	https://arxiv.org/abs/1905.12712
Dihydrobiopterin,2TMS,#3	C[C@H](O[Si](C)(C)C)[C@H](O)C1=NC2=C(NC1)N([Si](C)(C)C)C(N)=NC2=O	2316.6	Semi standard non polar	https://arxiv.org/abs/1905.12712
Dihydrobiopterin,2TMS,#4	C[C@H](O[Si](C)(C)C)[C@H](O)C1=NC2=C([NH]C(N)=NC2=O)N([Si](C)(C)C)C1	2253.6	Semi standard non polar	https://arxiv.org/abs/1905.12712
Dihydrobiopterin,2TMS,#5	C[C@H](O)[C@H](O[Si](C)(C)C)C1=NC2=C(NC1)[NH]C(N[Si](C)(C)C)=NC2=O	2337.8	Semi standard non polar	https://arxiv.org/abs/1905.12712
Dihydrobiopterin,2TMS,#6	C[C@H](O)[C@H](O[Si](C)(C)C)C1=NC2=C(NC1)N([Si](C)(C)C)C(N)=NC2=O	2315.7	Semi standard non polar	https://arxiv.org/abs/1905.12712
Dihydrobiopterin,2TMS,#7	C[C@H](O)[C@H](O[Si](C)(C)C)C1=NC2=C([NH]C(N)=NC2=O)N([Si](C)(C)C)C1	2236.7	Semi standard non polar	https://arxiv.org/abs/1905.12712
Dihydrobiopterin,2TMS,#8	C[C@H](O)[C@H](O)C1=NC2=C(NC1)N([Si](C)(C)C)C(N[Si](C)(C)C)=NC2=O	2343.2	Semi standard non polar	https://arxiv.org/abs/1905.12712
Dihydrobiopterin,2TMS,#9	C[C@H](O)[C@H](O)C1=NC2=C(NC1)[NH]C(N([Si](C)(C)C)[Si](C)(C)C)=NC2=O	2407.4	Semi standard non polar	https://arxiv.org/abs/1905.12712
Dihydrobiopterin,2TMS,#10	C[C@H](O)[C@H](O)C1=NC2=C([NH]C(N[Si](C)(C)C)=NC2=O)N([Si](C)(C)C)C1	2313.7	Semi standard non polar	https://arxiv.org/abs/1905.12712
Dihydrobiopterin,2TMS,#11	C[C@H](O)[C@H](O)C1=NC2=C(N([Si](C)(C)C)C1)N([Si](C)(C)C)C(N)=NC2=O	2288.6	Semi standard non polar	https://arxiv.org/abs/1905.12712
Dihydrobiopterin,1TBDMS,#1	C[C@H](O[Si](C)(C)C(C)(C)C)[C@H](O)C1=NC2=C(NC1)[NH]C(N)=NC2=O	2616.6	Semi standard non polar	https://arxiv.org/abs/1905.12712
Dihydrobiopterin,1TBDMS,#2	C[C@H](O)[C@H](O[Si](C)(C)C(C)(C)C)C1=NC2=C(NC1)[NH]C(N)=NC2=O	2607.2	Semi standard non polar	https://arxiv.org/abs/1905.12712
Dihydrobiopterin,1TBDMS,#3	C[C@H](O)[C@H](O)C1=NC2=C(NC1)[NH]C(N[Si](C)(C)C(C)(C)C)=NC2=O	2636.4	Semi standard non polar	https://arxiv.org/abs/1905.12712
Dihydrobiopterin,1TBDMS,#4	C[C@H](O)[C@H](O)C1=NC2=C([NH]C(N)=NC2=O)N([Si](C)(C)C(C)(C)C)C1	2540.5	Semi standard non polar	https://arxiv.org/abs/1905.12712
Dihydrobiopterin,1TBDMS,#5	C[C@H](O)[C@H](O)C1=NC2=C(NC1)N([Si](C)(C)C(C)(C)C)C(N)=NC2=O	2601.3	Semi standard non polar	https://arxiv.org/abs/1905.12712
Dihydrobiopterin,2TBDMS,#1	C[C@H](O[Si](C)(C)C(C)(C)C)[C@H](O[Si](C)(C)C(C)(C)C)C1=NC2=C(NC1)[NH]C(N)=NC2=O	2760.9	Semi standard non polar	https://arxiv.org/abs/1905.12712
Dihydrobiopterin,2TBDMS,#2	C[C@H](O[Si](C)(C)C(C)(C)C)[C@H](O)C1=NC2=C(NC1)[NH]C(N[Si](C)(C)C(C)(C)C)=NC2=O	2756.2	Semi standard non polar	https://arxiv.org/abs/1905.12712
Dihydrobiopterin,2TBDMS,#3	C[C@H](O[Si](C)(C)C(C)(C)C)[C@H](O)C1=NC2=C(NC1)N([Si](C)(C)C(C)(C)C)C(N)=NC2=O	2762.5	Semi standard non polar	https://arxiv.org/abs/1905.12712
Dihydrobiopterin,2TBDMS,#4	C[C@H](O[Si](C)(C)C(C)(C)C)[C@H](O)C1=NC2=C([NH]C(N)=NC2=O)N([Si](C)(C)C(C)(C)C)C1	2669.0	Semi standard non polar	https://arxiv.org/abs/1905.12712
Dihydrobiopterin,2TBDMS,#5	C[C@H](O)[C@H](O[Si](C)(C)C(C)(C)C)C1=NC2=C(NC1)[NH]C(N[Si](C)(C)C(C)(C)C)=NC2=O	2753.5	Semi standard non polar	https://arxiv.org/abs/1905.12712
Dihydrobiopterin,2TBDMS,#6	C[C@H](O)[C@H](O[Si](C)(C)C(C)(C)C)C1=NC2=C(NC1)N([Si](C)(C)C(C)(C)C)C(N)=NC2=O	2751.6	Semi standard non polar	https://arxiv.org/abs/1905.12712
Dihydrobiopterin,2TBDMS,#7	C[C@H](O)[C@H](O[Si](C)(C)C(C)(C)C)C1=NC2=C([NH]C(N)=NC2=O)N([Si](C)(C)C(C)(C)C)C1	2656.8	Semi standard non polar	https://arxiv.org/abs/1905.12712
Dihydrobiopterin,2TBDMS,#8	C[C@H](O)[C@H](O)C1=NC2=C(NC1)N([Si](C)(C)C(C)(C)C)C(N[Si](C)(C)C(C)(C)C)=NC2=O	2786.6	Semi standard non polar	https://arxiv.org/abs/1905.12712
Dihydrobiopterin,2TBDMS,#9	C[C@H](O)[C@H](O)C1=NC2=C(NC1)[NH]C(N([Si](C)(C)C(C)(C)C)[Si](C)(C)C(C)(C)C)=NC2=O	2785.3	Semi standard non polar	https://arxiv.org/abs/1905.12712
Dihydrobiopterin,2TBDMS,#10	C[C@H](O)[C@H](O)C1=NC2=C([NH]C(N[Si](C)(C)C(C)(C)C)=NC2=O)N([Si](C)(C)C(C)(C)C)C1	2711.2	Semi standard non polar	https://arxiv.org/abs/1905.12712
Dihydrobiopterin,2TBDMS,#11	C[C@H](O)[C@H](O)C1=NC2=C(N([Si](C)(C)C(C)(C)C)C1)N([Si](C)(C)C(C)(C)C)C(N)=NC2=O	2708.9	Semi standard non polar	https://arxiv.org/abs/1905.12712

Spectra

GC-MS Spectra

Spectrum Type	Description	Splash Key	Deposition Date	Source	View
Predicted GC-MS	Predicted GC-MS Spectrum - Dihydrobiopterin GC-MS (Non-derivatized) - 70eV, Positive	splash10-01ox-8930000000-836b0568b603906f549d	2017-08-28	Wishart Lab	View Spectrum
Predicted GC-MS	Predicted GC-MS Spectrum - Dihydrobiopterin GC-MS (Non-derivatized) - 70eV, Positive	Not Available	2021-10-12	Wishart Lab	View Spectrum

MS/MS Spectra

Spectrum Type	Description	Splash Key	Deposition Date	Source	View
Experimental LC-MS/MS	LC-MS/MS Spectrum - Dihydrobiopterin Quattro_QQQ 10V, Positive-QTOF (Annotated)	splash10-000i-0190000000-b23b7cc50e3b7bda01be	2020-02-10	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Dihydrobiopterin Quattro_QQQ 25V, Positive-QTOF (Annotated)	splash10-0092-3900000000-a352eac2af4690fb47f1	2020-02-10	HMDB team, MONA	View Spectrum
Experimental LC-MS/MS	LC-MS/MS Spectrum - Dihydrobiopterin Quattro_QQQ 40V, Positive-QTOF (Annotated)	splash10-014i-9400000000-054ffe617ee964dda203	2020-02-10	HMDB team, MONA	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - Dihydrobiopterin 10V, Positive-QTOF	splash10-006x-0190000000-d7c2e475a8d3f7b4994f	2017-07-25	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - Dihydrobiopterin 20V, Positive-QTOF	splash10-022c-0890000000-00cee7b9f9a6dd10e984	2017-07-25	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - Dihydrobiopterin 40V, Positive-QTOF	splash10-03k9-1900000000-0095b515a0b62e12a2a2	2017-07-25	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - Dihydrobiopterin 10V, Negative-QTOF	splash10-000i-0390000000-4091667943de90fcbcad	2017-07-26	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - Dihydrobiopterin 20V, Negative-QTOF	splash10-0006-1930000000-034022c1ca4ad39363fd	2017-07-26	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - Dihydrobiopterin 40V, Negative-QTOF	splash10-0006-9400000000-4251e4ef4f844b6962ad	2017-07-26	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - Dihydrobiopterin 10V, Negative-QTOF	splash10-0006-0900000000-4f635c9ed114df17ae1f	2021-09-23	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - Dihydrobiopterin 20V, Negative-QTOF	splash10-002f-0900000000-c1bc8529741d404ca90e	2021-09-23	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - Dihydrobiopterin 40V, Negative-QTOF	splash10-0006-9500000000-3171f93c753525fc16a9	2021-09-23	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - Dihydrobiopterin 10V, Positive-QTOF	splash10-0006-0090000000-3376871b80c100bbe6d8	2021-09-24	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - Dihydrobiopterin 20V, Positive-QTOF	splash10-0g4l-0490000000-cb426b9d705889933601	2021-09-24	Wishart Lab	View Spectrum
Predicted LC-MS/MS	Predicted LC-MS/MS Spectrum - Dihydrobiopterin 40V, Positive-QTOF	splash10-0002-3900000000-7ba50f111b0f14f6fbdc	2021-09-24	Wishart Lab	View Spectrum

NMR Spectra

Spectrum Type	Description	Deposition Date	Source	View
Predicted 1D NMR	¹³C NMR Spectrum (1D, 100 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 100 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 1000 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 1000 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 200 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 200 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 300 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 300 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 400 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 400 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 500 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 500 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 600 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 600 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 700 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 700 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 800 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 800 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹³C NMR Spectrum (1D, 900 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Predicted 1D NMR	¹H NMR Spectrum (1D, 900 MHz, D₂O, predicted)	2021-09-29	Wishart Lab	View Spectrum
Experimental 1D NMR	¹H NMR Spectrum (1D, 600 MHz, DMSO, experimental)	2021-10-10	Wishart Lab	View Spectrum
Experimental 1D NMR	¹H NMR Spectrum (1D, 600 MHz, DMSO, experimental)	2021-10-10	Wishart Lab	View Spectrum
Experimental 2D NMR	[¹H, ¹³C]-HSQC NMR Spectrum (2D, 600 MHz, 100%_DMSO, experimental)	2020-02-10	Wishart Lab	View Spectrum

Biological Properties

Cellular Locations

Cytoplasm

Biospecimen Locations

Blood
Cerebrospinal Fluid (CSF)
Feces
Saliva

Tissue Locations

Not Available

Pathways

Name	SMPDB/PathBank	KEGG
Pterine Biosynthesis
Tyrosine metabolism	Not Available
Aromatic L-Aminoacid Decarboxylase Deficiency		Not Available
Tyrosine hydroxylase deficiency		Not Available
Dopa-responsive dystonia		Not Available

Normal Concentrations

Biospecimen	Status	Value	Age	Sex	Condition	Reference	Details
Blood	Detected and Quantified	0.006 +/- 0.0003 uM	Adult (>18 years old)	Both	Normal	12032193	details
Cerebrospinal Fluid (CSF)	Detected and Quantified	0.0004-0.014 uM	Adult (>18 years old)	Both	Normal	12032193	details
Saliva	Detected and Quantified	8.25 +/- 8.34 uM	Adult (>18 years old)	Female	Normal	Sugimoto et al. (...	details
Saliva	Detected and Quantified	1.22 +/- 3.60 uM	Adult (>18 years old)	Not Specified	Normal	Sugimoto et al. (...	details
Saliva	Detected and Quantified	10.8 +/- 14.7 uM	Adult (>18 years old)	Not Specified	Normal	Sugimoto et al. (...	details
Saliva	Detected and Quantified	23.6 +/- 28.7 uM	Adult (>18 years old)	Both	Normal	Sugimoto et al. (...	details

Abnormal Concentrations

Biospecimen	Status	Value	Age	Sex	Condition	Reference	Details
Blood	Detected and Quantified	0.0635 +/- 0.0041 uM	Adult (>18 years old)	Both	Kidney disease	12032193	details
Feces	Detected but not Quantified	Not Quantified	Children (1-13 years old)	Both	Enthesitis-related arthritis	27786174	details
Feces	Detected but not Quantified	Not Quantified	Adult (>18 years old)	Both	Colorectal Cancer	27275383	details

Associated Disorders and Diseases

Disease References

Kidney disease
Yokoyama K, Tajima M, Yoshida H, Nakayama M, Tokutome G, Sakagami H, Hosoya T: Plasma pteridine concentrations in patients with chronic renal failure. Nephrol Dial Transplant. 2002 Jun;17(6):1032-6. [PubMed:12032193 ]
Colorectal cancer
Brown DG, Rao S, Weir TL, O'Malia J, Bazan M, Brown RJ, Ryan EP: Metabolomics and metabolic pathway networks from human colorectal cancers, adjacent mucosa, and stool. Cancer Metab. 2016 Jun 6;4:11. doi: 10.1186/s40170-016-0151-y. eCollection 2016. [PubMed:27275383 ]

Associated OMIM IDs

114500 (Colorectal cancer)

External Links

DrugBank ID

DB04400

Phenol Explorer Compound ID

Not Available

FooDB ID

FDB021884

KNApSAcK ID

Not Available

Chemspider ID

106382

KEGG Compound ID

C02953

BioCyc ID

CPD-15159

BiGG ID

Not Available

Wikipedia Link

Dihydrobiopterin

METLIN ID

Not Available

PubChem Compound

119055

PDB ID

Not Available

ChEBI ID

43029

Food Biomarker Ontology

Not Available

VMH ID

CE2705

MarkerDB ID

MDB00000019

Good Scents ID

Not Available

References

Synthesis Reference

Gal E M. Synthesis and quantitative aspects of dihydrobiopterin control of cerebral serotonin levels. Advances in experimental medicine and biology (1981), 133 197-206.

Material Safety Data Sheet (MSDS)

Download (PDF)

General References

Bonafe L, Thony B, Penzien JM, Czarnecki B, Blau N: Mutations in the sepiapterin reductase gene cause a novel tetrahydrobiopterin-dependent monoamine-neurotransmitter deficiency without hyperphenylalaninemia. Am J Hum Genet. 2001 Aug;69(2):269-77. Epub 2001 Jul 6. [PubMed:11443547 ]
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 ]
Leeming RJ, Blair JA, Melikian V, O'Gorman DJ: Biopterin derivatives in human body fluids and tissues. J Clin Pathol. 1976 May;29(5):444-51. [PubMed:932231 ]
Witteveen CF, Giovanelli J, Kaufman S: Reduction of quinonoid dihydrobiopterin to tetrahydrobiopterin by nitric oxide synthase. J Biol Chem. 1996 Feb 23;271(8):4143-7. [PubMed:8626754 ]
Niederwieser A, Curtius HC, Bettoni O, Bieri J, Schircks B, Viscontini M, Schaub J: Atypical phenylketonuria caused by 7, 8-dihydrobiopterin synthetase deficiency. Lancet. 1979 Jan 20;1(8108):131-3. [PubMed:84153 ]
Topal G, Brunet A, Millanvoye E, Boucher JL, Rendu F, Devynck MA, David-Dufilho M: Homocysteine induces oxidative stress by uncoupling of NO synthase activity through reduction of tetrahydrobiopterin. Free Radic Biol Med. 2004 Jun 15;36(12):1532-41. [PubMed:15182855 ]
Yokoyama K, Tajima M, Yoshida H, Nakayama M, Tokutome G, Sakagami H, Hosoya T: Plasma pteridine concentrations in patients with chronic renal failure. Nephrol Dial Transplant. 2002 Jun;17(6):1032-6. [PubMed:12032193 ]
Hagedoorn PL, Schmidt PP, Andersson KK, Hagen WR, Flatmark T, Martinez A: The effect of substrate, dihydrobiopterin, and dopamine on the EPR spectroscopic properties and the midpoint potential of the catalytic iron in recombinant human phenylalanine hydroxylase. J Biol Chem. 2001 Jun 22;276(25):22850-6. Epub 2001 Apr 11. [PubMed:11301319 ]
Howells DW, Hyland K: Direct analysis of tetrahydrobiopterin in cerebrospinal fluid by high-performance liquid chromatography with redox electrochemistry: prevention of autoxidation during storage and analysis. Clin Chim Acta. 1987 Jul 30;167(1):23-30. [PubMed:3665086 ]
Shinozaki K, Hirayama A, Nishio Y, Yoshida Y, Ohtani T, Okamura T, Masada M, Kikkawa R, Kodama K, Kashiwagi A: Coronary endothelial dysfunction in the insulin-resistant state is linked to abnormal pteridine metabolism and vascular oxidative stress. J Am Coll Cardiol. 2001 Dec;38(7):1821-8. [PubMed:11738280 ]
Yang S, Jan YH, Mishin V, Richardson JR, Hossain MM, Heindel ND, Heck DE, Laskin DL, Laskin JD: Sulfa drugs inhibit sepiapterin reduction and chemical redox cycling by sepiapterin reductase. J Pharmacol Exp Ther. 2015 Mar;352(3):529-40. doi: 10.1124/jpet.114.221572. Epub 2014 Dec 30. [PubMed:25550200 ]

Only showing the first 10 proteins. There are 11 proteins in total.

Show all enzymes and transporters

Enzymes

Enzyme Details

1. Tyrosinase

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

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 ]

Enzyme Details

2. Tyrosine 3-monooxygenase

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

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 ]

Enzyme Details

3. Sepiapterin reductase

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

Dihydrobiopterin + NADP → Sepiapterin + NADPH	details

References

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 ]

Enzyme Details

4. Tryptophan 5-hydroxylase 1

General function:: Involved in amino acid binding
Specific function:: Not Available
Gene Name:: TPH1
Uniprot ID:: P17752
Molecular weight:: 50984.725

References

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 ]

Enzyme Details

5. Dihydropteridine reductase

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

Enzyme Details

6. Dihydrofolate reductase

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

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 ]

Enzyme Details

7. Nitric oxide synthase, brain

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

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 ]

Enzyme Details

8. Nitric oxide synthase, endothelial

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

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 ]

Enzyme Details

9. Pterin-4-alpha-carbinolamine dehydratase

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

(6R)-6-(L-erythro-1,2-dihydroxypropyl)-5,6,7,8-tetrahydro-4a-hydroxypterin → Dihydrobiopterin + Water	details

Enzyme Details

10. Pterin-4-alpha-carbinolamine dehydratase 2

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

(6R)-6-(L-erythro-1,2-dihydroxypropyl)-5,6,7,8-tetrahydro-4a-hydroxypterin → Dihydrobiopterin + Water	details

Only showing the first 10 proteins. There are 11 proteins in total.

Show all enzymes and transporters

Showing metabocard for Dihydrobiopterin (HMDB0000038)

MOL for HMDB0000038 (Dihydrobiopterin)

3D MOL for HMDB0000038 (Dihydrobiopterin)

3D SDF for HMDB0000038 (Dihydrobiopterin)

3D-SDF for HMDB0000038 (Dihydrobiopterin)

PDB for HMDB0000038 (Dihydrobiopterin)

3D PDB for HMDB0000038 (Dihydrobiopterin)

SMILES for HMDB0000038 (Dihydrobiopterin)

INCHI for HMDB0000038 (Dihydrobiopterin)

Structure for HMDB0000038 (Dihydrobiopterin)

3D Structure for HMDB0000038 (Dihydrobiopterin)

Experimental Collision Cross Sections

Predicted Kovats Retention Indices

Derivatized

GC-MS Spectra

MS/MS Spectra

NMR Spectra

Enzymes

References

References

Reactions

References

References

References

References

References

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