Tìm theo
Tetrahydrobiopterin
Các tên gọi khác (7 ) :
  • (6R)-2-Amino-6-[(1R,2S)-1,2-dihydroxypropyl]-5,6,7,8-tetrahydropteridin-4(1H)-one
  • 2-Amino-6-(1,2-dihydroxypropyl)-5,6,7,8-tetrahydoro-4(1H)-pteridinone
  • 5,6,7,8 Tetrahydrobiopterin
  • 5,6,7,8-Tetrahydrobiopterin
  • BH4
  • sapropterin
  • Tetrahydrobiopterin
dietary supplements, supplements
Thuốc Gốc
Small Molecule
CAS: 17528-72-2
ATC: A16AX07
ĐG : BioMarin Pharmaceuticals Inc. , http://www.bmrn.com
CTHH: C9H15N5O3
PTK: 241.2471
Tetrahydrobiopterin or BH4 is a cofactor in the synthesis of nitric oxide. It is also essential in the conversion of phenylalanine to tyrosine by the enzyme phenylalanine-4-hydroxylase; the conversion of tyrosine to L-dopa by the enzyme tyrosine hydroxylase; and conversion of tryptophan to 5-hydroxytryptophan via tryptophan hydroxylase. [Wikipedia]
Nhận Dạng Quốc Tế & Đặc Tính Hóa Học
Công thức hóa học
C9H15N5O3
Phân tử khối
241.2471
Monoisotopic mass
241.117489371
InChI
InChI=1S/C9H15N5O3/c1-3(15)6(16)4-2-11-7-5(12-4)8(17)14-9(10)13-7/h3-4,6,12,15-16H,2H2,1H3,(H4,10,11,13,14,17)
InChI Key
InChIKey=FNKQXYHWGSIFBK-UHFFFAOYSA-N
IUPAC Name
2-amino-6-(1,2-dihydroxypropyl)-1,4,5,6,7,8-hexahydropteridin-4-one
Traditional IUPAC Name
2-amino-6-(1,2-dihydroxypropyl)-5,6,7,8-tetrahydro-1H-pteridin-4-one
SMILES
CC(O)C(O)C1CNC2=C(N1)C(=O)N=C(N)N2
Độ tan chảy
250-255 °C (hydrochloride salt)
Độ hòa tan
>20 mg/mL (dichloride salt)
logP
-1.7
logS
-2
pKa (strongest acidic)
10.01
pKa (Strongest Basic)
3.58
PSA
132 Å2
Refractivity
68.43 m3·mol-1
Polarizability
23.4 Å3
Rotatable Bond Count
2
H Bond Acceptor Count
8
H Bond Donor Count
6
Physiological Charge
0
Number of Rings
2
Bioavailability
1
Dược Lực Học : Tetrahydrobiopterin (BH4) is used to convert several amino acids, including phenylalanine, to other essential molecules in the body including neurotransmitters. Tetrahydrobiopterin deficiency can be caused by mutations in GTP cyclohydrolase 1 (GCH1), 6-pyruvoyl-tetrahydropterin synthase/dimerization cofactor of hepatocyte nuclear factor 1 alpha (PCBD1), 6-pyruvoyltetrahydropterin synthase (PTS), and quinoid dihydropteridine reductase (QDPR) genes. These genes make the enzymes that are critical for producing and recycling tetrahydrobiopterin. If one of the enzymes fails to function correctly because of a gene mutation, little or no tetrahydrobiopterin is produced. As a result, phenylalanine from the diet builds up in the bloodstream and other tissues and can damage nerve cells in the brain. High levels of phenylalanine can result in signs and symptoms ranging from temporary low muscle tone to mental retardation, movement disorders, difficulty swallowing, seizures, behavioral problems, progressive problems with development, and an inability to control body temperature.
Cơ Chế Tác Dụng : Tetrahydrobiopterin or BH4 is a cofactor in the synthesis of nitric oxide. It is also essential in the conversion of phenylalanine to tyrosine by the enzyme phenylalanine-4-hydroxylase; the conversion of tyrosine to L-dopa by the enzyme tyrosine hydroxylase; and conversion of tryptophan to 5-hydroxytryptophan via tryptophan hydroxylase. [Wikipedia] Tetrahydrobiopterin (BH4) is a natural co-factor or co-enzyme for phenylalanine-4-hydroxylase (PAH),Tetrahydrobiopterine, and tryptophan-5-hydroxylase. Tetrahydrobiopterin is also a natural co-factor for nitrate oxide synthase. Therefore BH4 is required for the conversion of phenylalanine to tyrosine, for the production of epinephrine (adrenaline) and the synthesis of the monoamine neuro-transmitters, serotonin, dopamine, and norepinephrine (noradrenaline). It is also involved in apoptosis and other cellular events mediated by nitric oxide production. As a coenzyme, BH4 reacts with molecular oxygen to form an active oxygen intermediate that can hydroxylate substrates. In the hydroxylation process, the co-enzyme loses two electrons and is regenerated in vivo in an NADH-dependent reaction. As a co-factor for PAH, tetrahydrobiopterin allows the conversion of phenylalanine to tyrosine and reduces the level of phenylalanine in the bloodstream, thereby reducing the toxic effects of of this amino acid. Normal serum concentrations of phenylalanine are 100 micomolar, while elevated (toxic) levels are typically >1200 micromolar. Individuals with a deficiency in tetrahydrobiopterin are not able to efficiently convert phenylalanine to tyrosine. The excess levels provided by tetrahydrobiopterin supplementation help improve enzyme efficiency. As a co-factor for tyrosine hydroxylase, BH4 facilitates the conversion of tyrosine to L-dopa while as a co-factor for tryptophan hydroxylase, BH4 allows the conversion of tryptophan to 5-hydroxytryptophan, which is then converted to serotonin.
Chỉ Định : For the treatment of tetrahydrobiopterin (BH4) deficiency.
Dữ Kiện Thương Mại
Giá thị trường
  • Biệt dược thương mại : Kuvan 100 mg tablet
    Giá bán buôn : USD >36.5
    Đơn vị tính : tablet
Nhà Sản Xuất
  • Công ty : Excelsior
    Sản phẩm biệt dược : BH4
  • Công ty : Daiichi Sankyo
    Sản phẩm biệt dược : Biopten
  • Công ty : Merck
    Sản phẩm biệt dược : Kuvan
Đóng gói
Tài Liệu Tham Khảo Thêm
drugbank
http://www.drugbank.ca/drugs/DB00360
PubChem Compound
http://pubchem.ncbi.nlm.nih.gov/summary/summary.cgi?sid=1125
PubChem Substance
http://pubchem.ncbi.nlm.nih.gov/summary/summary.cgi?sid=46508597
KEGG Compound
http://www.genome.jp/dbget-bin/www_bget?cpd:C00272
ChemSpider
http://www.chemspider.com/Chemical-Structure.1093.html
National Drug Code Directory
http://www.hipaaspace.com/Medical_Billing/Coding/National.Drug.Codes/PDF/68135-300-01
PharmGKB
http://www.pharmgkb.org/drug/PA161990676
Wikipedia
http://en.wikipedia.org/wiki/Tetrahydrobiopterin
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