(6R)-5,6,7,8-Tetrahydro-L-biopterin dihydrochloride

Names

[ Name ]:
(6R)-5,6,7,8-Tetrahydro-L-biopterin dihydrochloride

[Synonym ]:
(6R)-2-amino-6-[(1R,2S)-1,2-dihydroxypropyl]-5,6,7,8-tetrahydropteridin-4(1H)-one dihydrochloride
(6R,1'R,2'S)-6-(1',2'-dihydroxypropyl)-5,6,7,8-tetrahydrobiopterin
Dapropterin
(6R)-2-Amino-6-[(1R,2S)-1,2-dihydroxypropyl]-5,6,7,8-tetrahydro-4(1H)-pteridinone
(6R)-5,6,7,8-tetrahydro-L-biopterin
(6R)-Tetrahydrobiopterin
6R-tetahydrobiopterin
Dapropterin dihydrochloride
Biopten
(6R)-5,6,7,8-TETRAHYDRO-L-BIOPTERIN DIHYDROCHLORIDE
4(1H)-Pteridinone, 2-amino-6-[(1R,2S)-1,2-dihydroxypropyl]-5,6,7,8-tetrahydro-, (6R)-, hydrochloride (1:2)
sapropterinum
(6R)-2-Amino-6-[(1R,2S)-1,2-dihydroxypropyl]-5,6,7,8-tetrahydropteridin-4(1H)-one
R-THBP
MFCD00891665
(R)-2-Amino-6-((1R,2S)-1,2-dihydroxypropyl)-5,6,7,8-tetrahydropteridin-4(3H)-one dihydrochloride
(6R)-5,6,7,8-Tetrahydrobiopterin
(6R)-tetrahydrobiopterin dihydrochloride
4(1H)-Pteridinone, 2-amino-6-[(1R,2S)-1,2-dihydroxypropyl]-5,6,7,8-tetrahydro-, (6R)-
[6R-[6R*(1R*,2S*)]]-2-amino-6-(1,2-dihydroxypropyl)-5,6,7,8-tetrahydro-4(1H)-pteridinone
(6R)-Tetrahydrobiopterin hydrochloride
(6R)-2-Amino-6-[(1R,2S)-1,2-dihydroxypropyl]-5,6,7,8-tetrahydro-4(1H)-pteridinone dihydrochloride
Sapropterin
kuvan
(6R)-5,6,7,8-Tetrahydrobiopterin dihydrochloride
6b-5,6,7,8-Tetrahydro-L-biopterin
(6R)-2-amino-6-[(1R,2S)-1,2-dihydroxypropyl]-5,6,7,8-tetrahydro-1H-pteridin-4-one,dihydrochloride
6R-Tetrahydro-L-biopterin
(6R-(6R*(1R*,2S*)))-5,6,7,8-Tetrahydro-2-amino-6-(1,2-dihydroxypropyl)-4(1H)-pteridinone dihydrochloride
R-THBP6R-BH4
(6R)-L-erythro-5,6,7,8-Tetrahydrobiopterin
sapropterina
(6R)-L-erythro-Tetrahydrobiopterin
4(1H)-pteridinone, 2-amino-6-[(1R,2S)-1,2-dihydroxypropyl]-5,6,7,8-tetrahydro-, (6R)-, dihydrochloride
Sapropterin dihydrochloride
Sapropterin (dihydrochloride)

Biological Activity

[Description]:

Sapropterin dihydrochloride is a synthetic form of BH4 that is approved for the treatment of BH4 responsive PKU. (1) Sapropterin dihydrochloride can stimulate TH and TPH activities leading to improved dopamine and serotonin synthesis despite persistently elevated brain phenylalanine.(2) Sapropterin dihydrochloride is used to lower blood phenylalanine levels in tetrahydrobiopterin-responsive phenylketonuria in conjunction with a phenylalanine-restricted diet.

[Related Catalog]:

Signaling Pathways >> Others >> Others

Research Areas >> Cardiovascular Disease

Natural Products >> Alkaloid

[References]

[1]. Winn SR et al. High dose sapropterin dihydrochloride therapy improves monoamine neurotransmitter turnover in murine phenylketonuria (PKU). Mol Genet Metab. 2016 Jan;117(1):5-11.

[2]. Somaraju UR et al. Sapropterin dihydrochloride for phenylketonuria. Cochrane Database Syst Rev. 2015 Mar 27

[3]. Jurecki ER et al. Sapropterin Dihydrochloride Mixed With Common Foods and Beverages. Top Clin Nutr. 2014 Oct;29(4):325-331.

[4]. Grange DK et al. Sapropterin dihydrochloride use in pregnant women with phenylketonuria: an interim report of the PKU MOMS sub-registry. Mol Genet Metab. 2014 May;112(1):9-16

[Related Small Molecules]

Chemical & Physical Properties

[ Density]:
1.9±0.1 g/cm3

[ Boiling Point ]:
506.6±60.0 °C at 760 mmHg

[ Molecular Formula ]:
C₉H₁₇Cl₂N₅O₃

[ Molecular Weight ]:
314.17

[ Flash Point ]:
260.2±32.9 °C

[ PSA ]:
136.29000

[ LogP ]:
-4.22

[ Vapour Pressure ]:
0.0±3.0 mmHg at 25°C

[ Index of Refraction ]:
1.822

[ Storage condition ]:
−20°C

MSDS

Click to get detail MSDS

Toxicological Information

CHEMICAL IDENTIFICATION

RTECS NUMBER :: UO3516500

CHEMICAL NAME :: 4(1H)-Pteridinone, 5,6,7,8-tetrahydro-2-amino-6-(1,2-dihydroxypropyl)-, dihydrochloride, (6R-(6R*(1R*,2S*)))-

CAS REGISTRY NUMBER :: 69056-38-8

LAST UPDATED :: 199603

DATA ITEMS CITED :: 6

MOLECULAR FORMULA :: C9-H15-N5-O3.2Cl-H

MOLECULAR WEIGHT :: 314.21

HEALTH HAZARD DATA

ACUTE TOXICITY DATA

TYPE OF TEST :: LD50 - Lethal dose, 50 percent kill

ROUTE OF EXPOSURE :: Oral

SPECIES OBSERVED :: Rodent - rat

DOSE/DURATION :: 1 gm/kg

TOXIC EFFECTS :: Details of toxic effects not reported other than lethal dose value

REFERENCE :: YKYUA6 Yakkyoku. Pharmacy. (Nanzando, 4-1-11, Yushima, Bunkyo-ku, Tokyo, Japan) V.1- 1950- Volume(issue)/page/year: 43,1191,1992

TYPE OF TEST :: LD50 - Lethal dose, 50 percent kill

ROUTE OF EXPOSURE :: Subcutaneous

SPECIES OBSERVED :: Rodent - rat

DOSE/DURATION :: 1084 mg/kg

TOXIC EFFECTS :: Details of toxic effects not reported other than lethal dose value

REFERENCE :: YKYUA6 Yakkyoku. Pharmacy. (Nanzando, 4-1-11, Yushima, Bunkyo-ku, Tokyo, Japan) V.1- 1950- Volume(issue)/page/year: 43,1191,1992

TYPE OF TEST :: LD50 - Lethal dose, 50 percent kill

ROUTE OF EXPOSURE :: Intravenous

SPECIES OBSERVED :: Rodent - rat

DOSE/DURATION :: 150 mg/kg

TOXIC EFFECTS :: Details of toxic effects not reported other than lethal dose value

REFERENCE :: YKYUA6 Yakkyoku. Pharmacy. (Nanzando, 4-1-11, Yushima, Bunkyo-ku, Tokyo, Japan) V.1- 1950- Volume(issue)/page/year: 43,1191,1992

TYPE OF TEST :: LD50 - Lethal dose, 50 percent kill

ROUTE OF EXPOSURE :: Oral

SPECIES OBSERVED :: Rodent - mouse

DOSE/DURATION :: 1 gm/kg

TOXIC EFFECTS :: Details of toxic effects not reported other than lethal dose value

REFERENCE :: YKYUA6 Yakkyoku. Pharmacy. (Nanzando, 4-1-11, Yushima, Bunkyo-ku, Tokyo, Japan) V.1- 1950- Volume(issue)/page/year: 43,1191,1992

TYPE OF TEST :: LD50 - Lethal dose, 50 percent kill

ROUTE OF EXPOSURE :: Subcutaneous

SPECIES OBSERVED :: Rodent - mouse

DOSE/DURATION :: 239 mg/kg

TOXIC EFFECTS :: Details of toxic effects not reported other than lethal dose value

REFERENCE :: YKYUA6 Yakkyoku. Pharmacy. (Nanzando, 4-1-11, Yushima, Bunkyo-ku, Tokyo, Japan) V.1- 1950- Volume(issue)/page/year: 43,1191,1992

TYPE OF TEST :: LD50 - Lethal dose, 50 percent kill

ROUTE OF EXPOSURE :: Intravenous

SPECIES OBSERVED :: Rodent - mouse

DOSE/DURATION :: 150 mg/kg

TOXIC EFFECTS :: Details of toxic effects not reported other than lethal dose value

REFERENCE :: YKYUA6 Yakkyoku. Pharmacy. (Nanzando, 4-1-11, Yushima, Bunkyo-ku, Tokyo, Japan) V.1- 1950- Volume(issue)/page/year: 43,1191,1992

Safety Information

[ Symbol ]:

GHS07

[ Signal Word ]:
Warning

[ Hazard Statements ]:
H302

[ Hazard Codes ]:
Xn

[ Risk Phrases ]:
R22

[ RIDADR ]:
NONH for all modes of transport

[ WGK Germany ]:
3

[ RTECS ]:
UO3516500

[ HS Code ]:
2933990090

Synthetic Route

Click to get detail synthetic route

Precursor & DownStream

Precursor

DownStream

Customs

[ HS Code ]: 2933990090

[ Summary ]:
2933990090. heterocyclic compounds with nitrogen hetero-atom(s) only. VAT:17.0%. Tax rebate rate:13.0%. . MFN tariff:6.5%. General tariff:20.0%

Articles

Tetrahydrobiopterin and cytokines.

Proc. Soc. Exp. Biol. Med. 203 , 1, (1993)

Biosynthesis of tetrahydrobiopterin starts from guanosine triphosphate by the action of guanosine triphosphate cyclohydrolase I, which yields the first intermediate, 7,8-dihydroneopterin triphosphate....

Intravenous treatment of experimental Parkinson's disease in the mouse with an IgG-GDNF fusion protein that penetrates the blood-brain barrier.

Brain Res. 1352 , 208-13, (2010)

Glial-derived neurotrophic factor (GDNF) is a trophic factor for the nigra-striatal tract in experimental Parkinson's disease (PD). The neurotrophin must be administered by intra-cerebral injection, b...

A new resorufin-based alpha-glucosidase assay for high-throughput screening.

Anal. Biochem. 390 , 79-84, (2009)

Mutations in alpha-glucosidase cause accumulation of glycogen in lysosomes, resulting in Pompe disease, a lysosomal storage disorder. Small molecule chaperones that bind to enzyme proteins and correct...