1.0 2011-09-21 00:07:47 UTC 2015-10-09 22:30:26 UTC FDB022060 Biopterin 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] (-)-Biopterin (1'R,1'S) Biopterin [S-(R*,S*)]-2-amino-6-(1,2-dihydroxypropyl)-4(1H)-Pteridinone 2-amino-6-(L-erythro-1,2-dihydroxypropyl)-4(3H)-Pteridinone 6-Biopterin Biopterin L-Biopterin L-erythro-Biopterin Pterin H B2 C9H11N5O3 237.2153 237.086189243 2-amino-6-[(1R,2S)-1,2-dihydroxypropyl]-4,8-dihydropteridin-4-one 2-amino-6-[(1R,2S)-1,2-dihydroxypropyl]-8H-pteridin-4-one 22150-76-1 C[C@H](O)[C@H](O)C1=NC2=C(NC(N)=NC2=O)N=C1 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 LHQIJBMDNUYRAM-DZSWIPIPSA-N 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. Biopterins and derivatives Organic compounds Organoheterocyclic compounds Pteridines and derivatives Pterins and derivatives Aromatic heteropolycyclic compounds 1,2-diols Aminopyrimidines and derivatives Aromatic alcohols Azacyclic compounds Heteroaromatic compounds Hydrocarbon derivatives Organic oxides Organopnictogen compounds Primary amines Pyrazines Pyrimidones Secondary alcohols Vinylogous amides 1,2-diol Alcohol Amine Aminopyrimidine Aromatic alcohol Aromatic heteropolycyclic compound Azacycle Biopterin Heteroaromatic compound Hydrocarbon derivative Organic nitrogen compound Organic oxide Organic oxygen compound Organonitrogen compound Organooxygen compound Organopnictogen compound Primary amine Pyrazine Pyrimidine Pyrimidone Secondary alcohol Vinylogous amide biopterin Solid logp -1.41 ALOGPS logs -2.48 ALOGPS solubility 7.87e-01 g/l ALOGPS logp -2.2 ChemAxon pka_strongest_acidic 8.18 ChemAxon pka_strongest_basic -1.4 ChemAxon iupac 2-amino-6-[(1R,2S)-1,2-dihydroxypropyl]-4,8-dihydropteridin-4-one ChemAxon average_mass 237.2153 ChemAxon mono_mass 237.086189243 ChemAxon smiles C[C@H](O)[C@H](O)C1=NC2=C(NC(N)=NC2=O)N=C1 ChemAxon formula C9H11N5O3 ChemAxon inchi 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 ChemAxon inchikey LHQIJBMDNUYRAM-DZSWIPIPSA-N ChemAxon polar_surface_area 132.66 ChemAxon refractivity 57.81 ChemAxon polarizability 22.47 ChemAxon rotatable_bond_count 2 ChemAxon acceptor_count 8 ChemAxon donor_count 4 ChemAxon physiological_charge 0 ChemAxon formal_charge 0 ChemAxon Specdb::NmrTwoD 1327 Specdb::CMs 555 Specdb::CMs 22218 Specdb::CMs 30489 Specdb::CMs 37552 Specdb::CMs 149609 Specdb::CMs 1063089 Specdb::CMs 1063091 Specdb::CMs 1063093 Specdb::CMs 1063095 Specdb::CMs 1063097 Specdb::CMs 1063099 Specdb::CMs 1063101 Specdb::CMs 1063103 Specdb::CMs 1063105 Specdb::CMs 1063107 Specdb::CMs 1063109 Specdb::CMs 1063111 Specdb::CMs 1063112 Specdb::CMs 1063114 Specdb::CMs 1063117 Specdb::CMs 1063118 Specdb::CMs 1063120 Specdb::CMs 1063122 Specdb::CMs 1063124 Specdb::CMs 1063126 Specdb::NmrOneD 1383 Specdb::NmrOneD 144090 Specdb::NmrOneD 144091 Specdb::NmrOneD 144092 Specdb::NmrOneD 144093 Specdb::NmrOneD 144094 Specdb::NmrOneD 144095 Specdb::NmrOneD 144096 Specdb::NmrOneD 144097 Specdb::NmrOneD 144098 Specdb::NmrOneD 144099 Specdb::NmrOneD 144100 Specdb::NmrOneD 144101 Specdb::NmrOneD 144102 Specdb::NmrOneD 144103 Specdb::NmrOneD 144104 Specdb::NmrOneD 144105 Specdb::NmrOneD 144106 Specdb::NmrOneD 144107 Specdb::NmrOneD 144108 Specdb::NmrOneD 144109 Specdb::NmrOneD 166334 Specdb::NmrOneD 166673 Specdb::MsMs 680 Specdb::MsMs 681 Specdb::MsMs 682 Specdb::MsMs 315337 Specdb::MsMs 315338 Specdb::MsMs 315339 Specdb::MsMs 361279 Specdb::MsMs 361280 Specdb::MsMs 361281 Specdb::MsMs 2236580 Specdb::MsMs 2236813 Specdb::MsMs 2238711 Specdb::MsMs 2238865 Specdb::MsMs 2240839 Specdb::MsMs 2240869 Specdb::MsMs 2242872 Specdb::MsMs 2242960 Specdb::MsMs 2249081 Specdb::MsMs 2249292 Specdb::MsMs 2256696 Specdb::MsMs 2257881 Specdb::MsMs 2258666 Specdb::MsMs 2259916 Specdb::MsMs 2349502 Specdb::MsMs 2349503 HMDB00468 15373 1B66 #<Reference:0x000055ce32c1b2a8> #<Reference:0x000055ce32c1b0f0> #<Reference:0x000055ce32c1af10> #<Reference:0x000055ce32c1acb8> #<Reference:0x000055ce32c1ab00> #<Reference:0x000055ce32c1a948> #<Reference:0x000055ce32c1a790> #<Reference:0x000055ce32c1a5d8> #<Reference:0x000055ce32c1a3d0> #<Reference:0x000055ce32c1a1a0> #<Reference:0x000055ce32c19f20> #<Reference:0x000055ce32c19cc8> #<Reference:0x000055ce32c19a98> #<Reference:0x000055ce32c19778> #<Reference:0x000055ce32c19480> #<Reference:0x000055ce32c192c8> #<Reference:0x000055ce32c19110> #<Reference:0x000055ce32c18f58> #<Reference:0x000055ce32c18da0> #<Reference:0x000055ce32c18be8> #<Reference:0x000055ce32c18a30> Anatidae Type 1 specific Anatidae 8830 Beefalo Type 1 specific Bos taurus X Bison bison 297284 Bison Type 1 specific Bison bison 9901 Buffalo Type 1 specific Bubalus bubalis 89462 Cattle (Beef, Veal) Type 1 specific Bos taurus 9913 Chicken Type 1 specific Gallus gallus 9031 Columbidae (Dove, Pigeon) Type 1 specific Columbidae 8930 Deer Type 1 specific Cervidae 9850 Domestic goat Type 1 specific Capra aegagrus hircus 9925 Domestic pig Type 1 specific Sus scrofa domestica 9825 Elk Type 1 specific Cervus canadensis 1574408 Emu Type 1 specific Dromaius novaehollandiae 8790 European rabbit Type 1 specific Oryctolagus 9984 Greylag goose Type 1 specific Anser anser 8843 Guinea hen Type 1 specific Numida meleagris 8996 Horse Type 1 specific Equus caballus 9796 Mallard duck Type 1 specific Anas platyrhynchos 8839 Mountain hare Type 1 specific Lepus timidus 62621 Mule deer Type 1 specific Odocoileus 9871 Ostrich Type 1 specific Struthio camelus 8801 Pheasant Type 1 specific Phasianus colchicus 9054 Quail Type 1 specific Phasianidae 9005 Rabbit Type 1 specific Leporidae 9979 Rock ptarmigan Type 1 specific Lagopus muta 64668 Sheep (Mutton, Lamb) Type 1 specific Ovis aries 9940 Squab Type 1 specific Columba 8931 Turkey Type 1 specific Meleagris gallopavo 9103 Velvet duck Type 1 specific Melanitta fusca 371864 Wild boar Type 1 specific Sus scrofa 9823