MiMeDB: Showing metabocard for L-erythro-tetrahydrobiopterin (MMDBc0032102)

Record Information

Version

1.0

Status

Detected and Quantified

Creation Date

2021-11-18 02:02:25 UTC

Update Date

2024-10-15 20:03:48 UTC

Metabolite ID

MMDBc0032102

Metabolite Identification

Common Name

L-erythro-tetrahydrobiopterin

Description

NULL

Structure

MOL SDF PDB SMILES InChI

Synonyms

Value	Source
(-)-(6R)-2-Amino-6-((1R,2S)-1,2-dihydroxypropyl)-5,6,7,8-tetrahydro-4(3H)-pteridinone	ChEBI
(6R)-L-Erythro-5,6,7,8-tetrahydrobiopterin	ChEBI
(6R)-L-Erythro-tetrahydrobiopterin	ChEBI
2-Amino-6-(1,2-dihydroxypropyl)-5,6,7,8-tetrahydoro-4(1H)-pteridinone	ChEBI
5,6,7,8-Tetrahydrobiopterin	ChEBI
6R-5,6,7,8-Tetrahydrobiopterin	ChEBI
6R-BH4	ChEBI
6R-L-5,6,7,8-Tetrahydrobiopterin	ChEBI
R-THBP	ChEBI
Sapropterina	ChEBI
Sapropterinum	ChEBI
Tetrahydrobiopterin	ChEBI
5,6,7,8-erythro-Tetrahydrobiopterin	MeSH, HMDB
5,6,7,8-tetrahydro-L-Erythrobiopterin	MeSH, HMDB
5,6,7,8-Tetrahydrobiopterin, (S-(r,s))-isomer	MeSH, HMDB
5,6,7,8-Tetrahydrodictyopterin	MeSH, HMDB
6R-L-erythro-5,6,7,8-Tetrahydrobiopterin	MeSH, HMDB
BPH4	MeSH, HMDB
D-threo-Tetrahydrobiopterin	MeSH, HMDB
THBP	MeSH, HMDB
Kuvan	MeSH, HMDB
Phenylalanine hydroxylase cofactor	MeSH, HMDB
Sapropterin dihydrochloride	MeSH, HMDB
tetrahydro-6-Biopterin	MeSH, HMDB
2',4',5'-Trihydroxybutyrophenone	MeSH
Sapropterin	MeSH
Trihydroxybutyrophenone	MeSH
1-Butanone, 1-(2,4,5-trihydroxyphenyl)	MeSH
2,4,5-Trihydroxybutyrophenone	MeSH
(6R)-5,6,7,8-Tetrahydro-L-biopterin	HMDB
(6R)-5,6,7,8-Tetrahydrobiopterin	HMDB
(6R)-Tetrahydrobiopterin	HMDB
2-Amino-6-(1,2-dihydroxypropyl)-5,6,7,8-tetrahydro-4(3H)-pteridinone	HMDB
6R-Tetrahydro-L-biopterin	HMDB
6beta-5,6,7,8-Tetrahydro-L-biopterin	HMDB
6β-5,6,7,8-Tetrahydro-L-biopterin	HMDB
L-erythro-Tetrahydrobiopterin	HMDB
(6R)-2-Amino-6-[(1R,2S)-1,2-dihydroxypropyl]-5,6,7,8-tetrahydro-4(1H)-pteridinone	HMDB

Molecular Formula

C₉H₁₅N₅O₃

Average Mass

241.2471

Monoisotopic Mass

241.117489371

IUPAC Name

Not Available

Traditional Name

Not Available

CAS Registry Number

Not Available

SMILES

Not Available

InChI Identifier

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)/t3-,4+,6-/m0/s1

InChI Key

FNKQXYHWGSIFBK-RPDRRWSUSA-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
Aminopyrimidine
Pyrimidone
Secondary aliphatic/aromatic amine
Pyrimidine
1,3-aminoalcohol
Vinylogous amide
Heteroaromatic compound
Secondary alcohol
1,2-diol
1,2-aminoalcohol
Secondary amine
Azacycle
Hydrocarbon derivative
Organic oxide
Organopnictogen compound
Primary amine
Organooxygen compound
Organonitrogen compound
Organic oxygen compound
Organic nitrogen compound
Amine
Alcohol
Aromatic heteropolycyclic compound

Molecular Framework

Aromatic heteropolycyclic compounds

External Descriptors

5,6,7,8-tetrahydrobiopterin (CHEBI:59560 )
Coenzymes (C00272 )

Functional Ontology

Not Available

Physical Properties

State

Expected Solid

Predicted Properties

Not Available

Spectra

Not Available

Biological Properties

Cellular Locations

Cytoplasm
Nucleus

Biospecimen Locations

All Tissues
Blood
Cerebrospinal Fluid (CSF)
Feces

Tissue Locations

All Tissues

Associated OMIM IDs

114500 (Colorectal cancer)
610247 (Eosinophilic esophagitis)

Human Proteins and Enzymes

Protein ID	Protein Name	Protein Type	Uniprot ID	Species
MMDBp0191760	Tyrosinase	Unknown	P14679	Homo sapiens
MMDBp0191943	Tyrosine 3-monooxygenase	Enzyme	P07101	Homo sapiens
MMDBp0191950	Phenylalanine-4-hydroxylase	Enzyme	P00439	Homo sapiens
MMDBp0192101	6-pyruvoyl tetrahydrobiopterin synthase	Unknown	Q03393	Homo sapiens
MMDBp0192102	Sepiapterin reductase	Enzyme	P35270	Homo sapiens
MMDBp0192127	Tryptophan 5-hydroxylase 1	Enzyme	P17752	Homo sapiens
MMDBp0192129	Tryptophan 5-hydroxylase 2	Enzyme	Q8IWU9	Homo sapiens
MMDBp0192192	Dihydropteridine reductase	Unknown	P09417	Homo sapiens
MMDBp0192544	Nitric oxide synthase, inducible	Unknown	P35228	Homo sapiens
MMDBp0192546	Nitric oxide synthase, brain	Unknown	P29475	Homo sapiens
MMDBp0192547	Nitric oxide synthase, endothelial	Unknown	P29474	Homo sapiens
MMDBp0192556	GTP cyclohydrolase 1	Unknown	P30793	Homo sapiens
MMDBp0193353	GTP cyclohydrolase 1 feedback regulatory protein	Unknown	P30047	Homo sapiens
MMDBp0193707	Pterin-4-alpha-carbinolamine dehydratase	Enzyme	P61457	Homo sapiens
MMDBp0196269	Calcium-calmodulin independent nitric oxide synthase iNOS protein	Enzyme	Q9UM94	Homo sapiens

Human Pathways

Pathways

Name	SMPDB/PathBank
Nitric Oxide Signaling Pathway
Ion Channels and Their Functional Role in Vascular Endothelium
Succinate Signalling During Inflammation
Immunometabolism Pathway (Bacterial Activation)
Immunometabolism Pathway (Viral Activation)

Metabolic Reactions

Reactions

Reaction ID	Precursor	Product	Enzyme	Data Source	Reference
MMDBr0000683	5-Methyltetrahydrofolic acid	5,10-Methylene-THF	Not Available	VMH	3735413
MMDBr0001935	L-Phenylalanine	4a-Hydroxytetrahydrobiopterin	Not Available	VMH; KEGG	30371894
MMDBr0002160	Dyspropterin	L-erythro-tetrahydrobiopterin	sepiapterin reductase (7,8-dihydrobiopterin:NADP+ oxidoreductase)	VMH; KEGG	30371894
MMDBr0002162	Dyspropterin	L-erythro-tetrahydrobiopterin	sepiapterin reductase (7,8-dihydrobiopterin:NADP+ oxidoreductase)	VMH	10727395 14729142
MMDBr0002276	L-erythro-tetrahydrobiopterin	5-Hydroxy-L-tryptophan	tryptophan hydroxylase 1	VMH	10727395 12379098
MMDBr0002295	L-erythro-tetrahydrobiopterin	L-Dopa	tyrosine hydroxylase	VMH	10727395 9920892
MMDBr0002538	L-Phenylalanine	4a-Carbinolamine tetrahydrobiopterin	phenylalanine hydroxylase	VMH	3298976 4074362
MMDBr0002540	6-Lactoyltetrahydropterin	L-erythro-tetrahydrobiopterin	sepiapterin reductase (7,8-dihydrobiopterin:NADP+ oxidoreductase)	VMH; KEGG	30371894
MMDBr0002541	L-erythro-tetrahydrobiopterin	5-Hydroxy-L-tryptophan	tryptophan hydroxylase 1	VMH	30371894
MMDBr0007314	4a-Carbinolamine tetrahydrobiopterin	L-erythro-tetrahydrobiopterin	Not Available	VMH	30371894
MMDBr0007609	4a-Carbinolamine tetrahydrobiopterin	L-erythro-tetrahydrobiopterin	Not Available	VMH	30371894
MMDBr0008334	L-Phenylalanine	4a-Carbinolamine tetrahydrobiopterin	Not Available	VMH	30371894
MMDBr0010407	L-erythro-tetrahydrobiopterin	L-Dopa	tyrosine hydroxylase	VMH; KEGG	30371894

Health Effects and Bioactivity

Health Outcome/Bioactivity	Metabolite Response/Effect	Evidence Type	Host and Biospecimen	Data Source	Reference
Epilepsy		Association	Human Cerebrospinal Fluid (CSF)	HMDB	7869898
Myocardial infarction	Decreased	Association	Human Blood	HMDB	17726257
Septic shock		Association	Human Blood	HMDB	11573638

Microbial Sources

Superkingdom/Kingdom	Phylum	Total species	Hosts and Body Sites	Microbial Links
Bacteria	Proteobacteria	147	Human ; Human feces; Human sputum; Human unknown; Pig ; Human pleural fluid plaque; Human dental plaque; Human nasal swab; Human stool; Human feces; pleural fluid plaque; bal	Unknown; Metabolic reconstruction
Bacteria	Firmicutes	136	Human feces; Human ; Cattle ; Human subgingival plaque	Metabolic reconstruction
Bacteria	Verrucomicrobia	1	Human ; Human feces	Metabolic reconstruction
Bacteria	Actinobacteria	7	Human ; Human feces	Metabolic reconstruction
Bacteria	Bacteroidetes	16	Human feces; Human ; Human supragingival plaque; Human subgingival plaque; Human saliva	Metabolic reconstruction
Bacteria	Fusobacteria	3	Human feces; Human	Metabolic reconstruction
Bacteria	Planctomycetes	1	Human feces	Metabolic reconstruction

Exposure Sources

Source Type	Activity	Data Source	Reference
Drugs	Antimicrobial	U.S. Environmental Protection Agency	CompTox
Cosmetics	Not Available	U.S. Environmental Protection Agency	CompTox
Domestics	Not Available	U.S. Environmental Protection Agency	CompTox

Host Biospecimen and Location

Host and Biospecimen	Status	Mean	Std	Min	Max	Units	Age	Sex	Health Condition	Data Source	Reference
Human Feces	Detected but not Quantified									HMDB
Human Cerebrospinal fluid (csf)	Detected and Quantified	14400				uM	Newborn (0-30 days old)	Both	Generalized seizures	HMDB	7869898
Human Blood	Detected and Quantified	0.0044	0.0007			uM	Adult (>18 years old)	Both	Myocardial infarction	HMDB	17726257
Human Blood	Detected and Quantified	0.16		0.0	5.8	uM	Adult (>18 years old)	Both	Septic shock	HMDB	11573638
Human Blood	Detected and Quantified	1.2		0.8	14.9	uM	Adult (>18 years old)	Both	Septic shock	HMDB	11573638
Human Blood	Detected and Quantified	0.0099	0.0008			uM	Adult (>18 years old)	Both	Smoking	HMDB	10636262
Human Blood	Detected and Quantified	0.0093	0.0005			uM	Adult (>18 years old)	Both	Normal	HMDB	17726257
Human Blood	Detected and Quantified	0.0110	0.0008			uM	Adult (>18 years old)	Both	Normal	HMDB	10636262
Human Cerebrospinal fluid (csf)	Detected and Quantified	0.026		0.014	0.038	uM	Adult (>18 years old)	Both	Normal	HMDB	16168596
Human All tissues	Detected but not Quantified						Not Specified	Not Specified	Normal	HMDB	34986597

External Links

HMDB ID

HMDB0059658

DrugBank ID

DB00360

Phenol Explorer Compound ID

Not Available

FooDB ID

Not Available

KNApSAcK ID

Not Available

Chemspider ID

40270

KEGG Compound ID

C00272

BioCyc ID

CPD-14053

BiGG ID

Not Available

Wikipedia Link

Sapropterin

METLIN ID

Not Available

PubChem Compound

44257

PDB ID

Not Available

ChEBI ID

59560

Food Biomarker Ontology

Not Available

References

Synthesis Reference

Not Available

General References

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. doi: 10.1136/jcp.29.5.444. [PubMed:932231 ]
Kaufman S: Biopterin-responsive hyperphenylalaninemia. J Nutr Sci Vitaminol (Tokyo). 1992;Spec No:601-6. doi: 10.3177/jnsv.38.special_601. [PubMed:1297822 ]
Takikawa S, Curtius HC, Redweik U, Leimbacher W, Ghisla S: Biosynthesis of tetrahydrobiopterin. Purification and characterization of 6-pyruvoyl-tetrahydropterin synthase from human liver. Eur J Biochem. 1986 Dec 1;161(2):295-302. doi: 10.1111/j.1432-1033.1986.tb10446.x. [PubMed:3536512 ]
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. doi: 10.1111/j.1432-1033.1985.tb08855.x. [PubMed:3888618 ]
Dhondt JL, Cotton RG, Danks DM: Liver enzyme activities in hyperphenylalaninaemia due to a defective synthesis of tetrahydrobiopterin. J Inherit Metab Dis. 1985;8(2):47-8. doi: 10.1007/BF01801660. [PubMed:3939528 ]
Kaufman S, Kapatos G, Rizzo WB, Schulman JD, Tamarkin L, Van Loon GR: Tetrahydropterin therapy for hyperphenylalaninemia caused by defective synthesis of tetrahydrobiopterin. Ann Neurol. 1983 Sep;14(3):308-15. doi: 10.1002/ana.410140309. [PubMed:6139056 ]
Komori H, Matsuishi T, Yamada S, Yamashita Y, Ohtaki E, Kato H: Cerebrospinal fluid biopterin and biogenic amine metabolites during oral R-THBP therapy for infantile autism. J Autism Dev Disord. 1995 Apr;25(2):183-93. doi: 10.1007/BF02178503. [PubMed:7559284 ]
Cady EB, Lorek A, Penrice J, Reynolds EO, Iles RA, Burns SP, Coutts GA, Cowan FM: Detection of propan-1,2-diol in neonatal brain by in vivo proton magnetic resonance spectroscopy. Magn Reson Med. 1994 Dec;32(6):764-7. doi: 10.1002/mrm.1910320611. [PubMed:7869898 ]
Furukawa Y, Kish SJ, Bebin EM, Jacobson RD, Fryburg JS, Wilson WG, Shimadzu M, Hyland K, Trugman JM: Dystonia with motor delay in compound heterozygotes for GTP-cyclohydrolase I gene mutations. Ann Neurol. 1998 Jul;44(1):10-6. doi: 10.1002/ana.410440107. [PubMed:9667588 ]
Schallreuter KU, Moore J, Wood JM, Beazley WD, Peters EM, Marles LK, Behrens-Williams SC, Dummer R, Blau N, Thony B: Epidermal H(2)O(2) accumulation alters tetrahydrobiopterin (6BH4) recycling in vitiligo: identification of a general mechanism in regulation of all 6BH4-dependent processes? J Invest Dermatol. 2001 Jan;116(1):167-74. doi: 10.1046/j.1523-1747.2001.00220.x. [PubMed:11168813 ]
Walter R, Kaufmann PA, Buck A, Berthold T, Wyss C, von Schulthess GK, Schaffner A, Schoedon G: Tetrahydrobiopterin increases myocardial blood flow in healthy volunteers: a double-blind, placebo-controlled study. Swiss Med Wkly. 2001 Feb 24;131(7-8):91-4. doi: 2001/07/smw-06147. [PubMed:11416883 ]
Spaapen LJ, Bakker JA, Velter C, Loots W, Rubio-Gozalbo ME, Forget PP, Dorland L, De Koning TJ, Poll-The BT, Ploos van Amstel HK, Bekhof J, Blau N, Duran M: Tetrahydrobiopterin-responsive phenylalanine hydroxylase deficiency in Dutch neonates. J Inherit Metab Dis. 2001 Jun;24(3):352-8. doi: 10.1023/a:1010596317296. [PubMed:11486900 ]
Galley HF, Le Cras AE, Yassen K, Grant IS, Webster NR: Circulating tetrahydrobiopterin concentrations in patients with septic shock. Br J Anaesth. 2001 Apr;86(4):578-80. doi: 10.1093/bja/86.4.578. [PubMed:11573638 ]
Hyland K, Gunasekara RS, Munk-Martin TL, Arnold LA, Engle T: The hph-1 mouse: a model for dominantly inherited GTP-cyclohydrolase deficiency. Ann Neurol. 2003;54 Suppl 6:S46-8. doi: 10.1002/ana.10695. [PubMed:12891653 ]
Koch R, Moseley KD, Moats R, Yano S, Matalon R, Guttler F: Danger of high-protein dietary supplements to persons with hyperphenylalaninaemia. J Inherit Metab Dis. 2003;26(4):339-42. doi: 10.1023/a:1025103018278. [PubMed:12971421 ]
Kuhn DM, Geddes TJ: Tetrahydrobiopterin prevents nitration of tyrosine hydroxylase by peroxynitrite and nitrogen dioxide. Mol Pharmacol. 2003 Oct;64(4):946-53. doi: 10.1124/mol.64.4.946. [PubMed:14500751 ]
Matter H, Kumar HS, Fedorov R, Frey A, Kotsonis P, Hartmann E, Frohlich LG, Reif A, Pfleiderer W, Scheurer P, Ghosh DK, Schlichting I, Schmidt HH: Structural analysis of isoform-specific inhibitors targeting the tetrahydrobiopterin binding site of human nitric oxide synthases. J Med Chem. 2005 Jul 28;48(15):4783-92. doi: 10.1021/jm050007x. [PubMed:16033258 ]
Sawabe K, Suetake Y, Nakanishi N, Wakasugi KO, Hasegawa H: Cellular accumulation of tetrahydrobiopterin following its administration is mediated by two different processes; direct uptake and indirect uptake mediated by a methotrexate-sensitive process. Mol Genet Metab. 2005 Dec;86 Suppl 1:S133-8. doi: 10.1016/j.ymgme.2005.06.020. Epub 2005 Sep 13. [PubMed:16165391 ]
Toda Y, Mori K, Hashimoto T, Miyazaki M, Nozaki S, Watanabe Y, Kuroda Y, Kagami S: Administration of secretin for autism alters dopamine metabolism in the central nervous system. Brain Dev. 2006 Mar;28(2):99-103. doi: 10.1016/j.braindev.2005.05.005. Epub 2005 Sep 15. [PubMed:16168596 ]
Moens AL, Kass DA: Tetrahydrobiopterin and cardiovascular disease. Arterioscler Thromb Vasc Biol. 2006 Nov;26(11):2439-44. doi: 10.1161/01.ATV.0000243924.00970.cb. Epub 2006 Aug 31. [PubMed:16946131 ]
Sanford M, Keating GM: Spotlight on sapropterin in primary hyperphenylalaninemia. BioDrugs. 2009;23(3):201-2. doi: 10.2165/00063030-200923030-00007. [PubMed:19627172 ]
MacDonald A, Ahring K, Dokoupil K, Gokmen-Ozel H, Lammardo AM, Motzfeldt K, Robert M, Rocha JC, van Rijn M, Belanger-Quintana A: Adjusting diet with sapropterin in phenylketonuria: what factors should be considered? Br J Nutr. 2011 Jul;106(2):175-82. doi: 10.1017/S0007114511000298. [PubMed:21466737 ]
Burton BK, Nowacka M, Hennermann JB, Lipson M, Grange DK, Chakrapani A, Trefz F, Dorenbaum A, Imperiale M, Kim SS, Fernhoff PM: Safety of extended treatment with sapropterin dihydrochloride in patients with phenylketonuria: results of a phase 3b study. Mol Genet Metab. 2011 Aug;103(4):315-22. doi: 10.1016/j.ymgme.2011.03.020. Epub 2011 Mar 31. [PubMed:21646032 ]
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Leuret O, Barth M, Kuster A, Eyer D, de Parscau L, Odent S, Gilbert-Dussardier B, Feillet F, Labarthe F: Efficacy and safety of BH4 before the age of 4 years in patients with mild phenylketonuria. J Inherit Metab Dis. 2012 Nov;35(6):975-81. doi: 10.1007/s10545-012-9464-3. Epub 2012 Mar 3. [PubMed:22388642 ]
Ziesch B, Weigel J, Thiele A, Mutze U, Rohde C, Ceglarek U, Thiery J, Kiess W, Beblo S: Tetrahydrobiopterin (BH4) in PKU: effect on dietary treatment, metabolic control, and quality of life. J Inherit Metab Dis. 2012 Nov;35(6):983-92. doi: 10.1007/s10545-012-9458-1. Epub 2012 Mar 6. [PubMed:22391997 ]
Cunningham A, Bausell H, Brown M, Chapman M, DeFouw K, Ernst S, McClure J, McCune H, O'Steen D, Pender A, Skrabal J, Wessel A, Jurecki E, Shediac R, Prasad S, Gillis J, Cederbaum S: Recommendations for the use of sapropterin in phenylketonuria. Mol Genet Metab. 2012 Jul;106(3):269-76. doi: 10.1016/j.ymgme.2012.04.004. Epub 2012 Apr 13. [PubMed:22575621 ]
Shintaku H, Ohwada M: Long-term follow-up of tetrahydrobiopterin therapy in patients with tetrahydrobiopterin deficiency in Japan. Brain Dev. 2013 May;35(5):406-10. doi: 10.1016/j.braindev.2012.06.010. Epub 2012 Jul 24. [PubMed:22832064 ]
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Gokmen Ozel H, Lammardo AM, Motzfeldt K, Robert M, Rocha JC, van Rijn M, Ahring K, Belanger-Quintana A, MacDonald A, Dokoupil K: Use of sapropterin in the management of phenylketonuria: seven case reports. Mol Genet Metab. 2013 Feb;108(2):109-11. doi: 10.1016/j.ymgme.2012.11.012. Epub 2012 Nov 28. [PubMed:23266371 ]
Thiele AG, Weigel JF, Ziesch B, Rohde C, Mutze U, Ceglarek U, Thiery J, Muller AS, Kiess W, Beblo S: Nutritional Changes and Micronutrient Supply in Patients with Phenylketonuria Under Therapy with Tetrahydrobiopterin (BH(4)). JIMD Rep. 2013;9:31-40. doi: 10.1007/8904_2012_176. Epub 2012 Oct 17. [PubMed:23430545 ]
Cerone R, Andria G, Giovannini M, Leuzzi V, Riva E, Burlina A: Testing for tetrahydrobiopterin responsiveness in patients with hyperphenylalaninemia due to phenylalanine hydroxylase deficiency. Adv Ther. 2013 Mar;30(3):212-28. doi: 10.1007/s12325-013-0011-x. Epub 2013 Feb 20. [PubMed:23436109 ]
Keil S, Anjema K, van Spronsen FJ, Lambruschini N, Burlina A, Belanger-Quintana A, Couce ML, Feillet F, Cerone R, Lotz-Havla AS, Muntau AC, Bosch AM, Meli CA, Billette de Villemeur T, Kern I, Riva E, Giovannini M, Damaj L, Leuzzi V, Blau N: Long-term follow-up and outcome of phenylketonuria patients on sapropterin: a retrospective study. Pediatrics. 2013 Jun;131(6):e1881-8. doi: 10.1542/peds.2012-3291. Epub 2013 May 20. [PubMed:23690520 ]
Blau N: Sapropterin dihydrochloride for the treatment of hyperphenylalaninemias. Expert Opin Drug Metab Toxicol. 2013 Sep;9(9):1207-18. doi: 10.1517/17425255.2013.804064. Epub 2013 May 27. [PubMed:23705856 ]
Douglas TD, Jinnah HA, Bernhard D, Singh RH: The effects of sapropterin on urinary monoamine metabolites in phenylketonuria. Mol Genet Metab. 2013 Jul;109(3):243-50. doi: 10.1016/j.ymgme.2013.04.017. Epub 2013 May 1. [PubMed:23712020 ]
Stanhewicz AE, Alexander LM, Kenney WL: Oral sapropterin acutely augments reflex vasodilation in aged human skin through nitric oxide-dependent mechanisms. J Appl Physiol (1985). 2013 Oct 1;115(7):972-8. doi: 10.1152/japplphysiol.00481.2013. Epub 2013 Jun 6. [PubMed:23743404 ]

Showing metabocard for L-erythro-tetrahydrobiopterin (MMDBc0032102)

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