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Chemical Compound Review

q-BH2     2-amino-6-[(1S,2S)-1,2- dihydroxypropyl]-7...

Synonyms: AG-K-92613, CHEBI:43029, CTK8D6639, ZINC18181336, AR-1D8539, ...
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Disease relevance of dihydrobiopterin


High impact information on dihydrobiopterin


Biological context of dihydrobiopterin

  • The crystal structure of the dimeric catalytic domain (residues 118-424) of human PheOH (hPheOH), cocrystallized with the oxidized form of the cofactor (7,8-dihydro-L-biopterin, BH(2)), has been determined at 2.0 A resolution [7].
  • Of particular interest was the reactivity demonstrated by 7,8-dihydrobiopterin because of the potential physiological significance of this reaction both in the regeneration of tetrahydrobiopterin, a cofactor for various biological hydroxylations, and as a step in the biosynthesis of this compound from GTP [8].

Anatomical context of dihydrobiopterin


Associations of dihydrobiopterin with other chemical compounds


Gene context of dihydrobiopterin


Analytical, diagnostic and therapeutic context of dihydrobiopterin


  1. Biopterin defect in a normal-appearing child affected by a transient phenylketonuria. Rey, F., Leeming, R.J., Blair, J.A., Rey, J. Arch. Dis. Child. (1980) [Pubmed]
  2. Three-dimensional structure of human tryptophan hydroxylase and its implications for the biosynthesis of the neurotransmitters serotonin and melatonin. Wang, L., Erlandsen, H., Haavik, J., Knappskog, P.M., Stevens, R.C. Biochemistry (2002) [Pubmed]
  3. 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. Goodwill, K.E., Sabatier, C., Stevens, R.C. Biochemistry (1998) [Pubmed]
  4. 6,6-Dimethylpterins: stable quinoid dihydropterin substrate for dihydropteridine reductase and tetrahydropterin cofactor for phenylalanine hydroxylase. Bailey, S.W., Ayling, J.E. Biochemistry (1983) [Pubmed]
  5. Molecular analysis of 16 Turkish families with DHPR deficiency using denaturing gradient gel electrophoresis (DGGE). Romstad, A., Kalkanoğlu, H.S., Coşkun, T., Demirkol, M., Tokatli, A., Dursun, A., Baykal, T., Ozalp, I., Guldberg, P., Güttler, F. Hum. Genet. (2000) [Pubmed]
  6. Determination of quinonoid dihydrobiopterin by high-performance liquid chromatography and electrochemical detection. Heales, S., Hyland, K. J. Chromatogr. (1989) [Pubmed]
  7. Crystal structure and site-specific mutagenesis of pterin-bound human phenylalanine hydroxylase. Erlandsen, H., Bjørgo, E., Flatmark, T., Stevens, R.C. Biochemistry (2000) [Pubmed]
  8. Comparative activity of rat liver dihydrofolate reductase with 7,8-dihydrofolate and other 7,8-dihydropteridines. Webber, S., Whiteley, J.M. Arch. Biochem. Biophys. (1985) [Pubmed]
  9. Tetrahydro-sepiapterin is an intermediate in tetrahydrobiopterin biosynthesis. Milstien, S., Kaufman, S. Biochem. Biophys. Res. Commun. (1983) [Pubmed]
  10. Multiple forms of rat-liver dihydropteridine reductase identified by their differing isoelectric points. Webber, S., Hural, J.A., Whiteley, J.M. Arch. Biochem. Biophys. (1986) [Pubmed]
  11. Studies on biologically active pteridines. III. The absolute configuration at the C-6 chiral center of tetrahydrobiopterin cofactor and related compounds. Matsuura, S., Sugimoto, T., Hasegawa, H., Imaizumi, S., Ichiyama, A. J. Biochem. (1980) [Pubmed]
  12. A sensitive high-performance liquid chromatographic-fluorometric assay for dihydrofolate reductase in adult rat brain, using 7,8-dihydrobiopterin as substrate. Reinhard, J.F., Chao, J.Y., Smith, G.K., Duch, D.S., Nichol, C.A. Anal. Biochem. (1984) [Pubmed]
  13. Determination of quinonoid dihydrobiopterin by liquid chromatography and electrochemical detection. Lunte, C.E., Kissinger, P.T. J. Chromatogr. (1984) [Pubmed]
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