- Two Cases of Tetrahydrobiopterin Deficiency
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Choi JW, Yang SW, Moon HR, Cho SC
- KMID: 2335149
- J Korean Pediatr Soc.
- 1995 Mar;38(3):397-403.
Tetrahydrobiopterin(BH4) deficiency is a rare type of hyperphenylalaninemia and usually leads to a progressive neurologic deterioration despite early dietary control of blood phenylalanine concentration. We experienced two cases of BH4... -
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- Assessment for Hepatic Injuries Induced by CCl4 and 2,2'-azobis(2-amidinopropane) dihydrochloride(AAPH) in Sprague-Dawley Rats
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Lee YJ, Ha WS, Park ST
- KMID: 2083079
- Korean J Hepatobiliary Pancreat Surg.
- 1997 Aug;1(2):83-118.
INTRODUCTION: It is now generally accepted that free radicals play an important role in the development of various forms of tissue damage and pathological events, such as heart disease, cancer,... -
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- Effect of Dexamethasone on the Production of Nitric Oxide in Trabecular Meshwork Cells
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Lee SY, Kim JW
- KMID: 2212544
- J Korean Ophthalmol Soc.
- 2009 Aug;50(8):1254-1258.
- doi: 10.3341/jkos.2009.50.8.1254
PURPOSE: To investigate the effects of dexamethasone (DEX) on the production of nitric oxide (NO) and its enzymatic synthetic pathway in cultured human trabecular meshwork (HTM) cells. METHODS: Primarily cultured... -
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- A Case of Dihydropteridine Reductase Deficiency
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Oh SJ, Hong YH, Lee YW, Lee ST, Ki CS, Lee DH
- KMID: 1473822
- J Genet Med.
- 2009 Dec;6(2):170-174.
Tetrahydrobiopterin (BH4) deficiency is caused by mutations in genes encoding enzymes involved in the synthesis and regeneration of BH4. The condition is usually accompanied by hyperphenylalaninemia (HPA) and deficiency of... -
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- Pterin & DHPR measurement and DNA analysis in Korean PKU patients
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Chong CH, Lee HY, Lee DH, Lee SJ, Cha KW, Yim JB, Okano Y
- KMID: 2208554
- J Korean Pediatr Soc.
- 1993 Dec;36(12):1681-1690.
Phenylketonuria is metabolic disorder that results from a deficiency of the hepatic phenylalanine hydroxylase. But among patients with hyperphenylalaninemia, the defect resides in one of the enzymes necessary for production... -
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- Differential Effects of 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine on Motor Behavior and Dopamine Levels at Brain Regions in Three Different Mouse Strains
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Lee KS, Lee JK, Kim HG, Kim HR
- KMID: 1432768
- Korean J Physiol Pharmacol.
- 2013 Feb;17(1):89-97.
- doi: 10.4196/kjpp.2013.17.1.89
Developing an animal model for a specific disease is very important in the understanding of the underlying mechanism of the disease and allows testing of newly developed new drugs before... -
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- Inactivation of Aconitase by Tetrahydrobiopterin in DArgic Cells: Relevance to PD
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Yoon NS, Cho Y, Lee SY, Choi HJ, Hwang O
- KMID: 2165316
- Exp Neurobiol.
- 2010 Jun;19(1):23-29.
- doi: 10.5607/en.2010.19.1.23
Oxidative damage is thought to be a major cause of the progression of dopamine (DA)rgic neurodegeneration as in Parkinson's disease. We have previously reported that tetrahydrobiopterin (BH4), an endogenous molecule... -
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- Tetrahydrobiopterin Inhibits PDGF-stimulated Migration and Proliferation in Rat Aortic Smooth Muscle Cells via the Nitric Oxide Synthase-independent Pathway
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Jiang X, Kim B, Lin H, Lee CK, Kim J, Kang H, Lee P, Jung SH, Lee HM, Won KJ
- KMID: 2285400
- Korean J Physiol Pharmacol.
- 2010 Jun;14(3):177-183.
- doi: 10.4196/kjpp.2010.14.3.177
Tetrahydrobiopterin (BH4), an essential cofactor for nitric oxide synthase (NOS) activity, is known to play important roles in modulating both NO and superoxide production during vascular diseases such as atherosclerosis.... -
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- A Pilot Study of Fluorodeoxyglucose Positron Emission Tomography Findings in Patients with Phenylketonuria before and during Sapropterin Supplementation
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Ficicioglu C, Dubroff JG, Thomas N, Gallagher , Burfield J, Hussa C, Randall R, Zhuang H
- KMID: 2287553
- J Clin Neurol.
- 2013 Jul;9(3):151-156.
- doi: 10.3988/jcn.2013.9.3.151
BACKGROUND AND PURPOSE: PET scanning with fluorodeoxyglucose (FDG-PET) is a non-invasive method that measures regional glucose metabolic rate. Phenylalanine (Phe) and its metabolites appear to impair several aspects of brain... -
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- Relationship between Tetrahydrobiopterin and Portal Hypertension in Patients with Chronic Liver Disease
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Hong WK, Shim KY, Baik SK, Kim MY, Cho MY, Jang YO, Park YS, Han J, Kim G, Cho YZ, Hwang HW, Lee JH, Chae MH, Kwon SO
- KMID: 1734927
- J Korean Med Sci.
- 2014 Mar;29(3):392-399.
- doi: 10.3346/jkms.2014.29.3.392
Tetrahydrobiopterin (BH4) is an essential cofactor in NO synthesis by endothelial nitric oxide synthase (eNOS) enzymes. It has been previously suggested that reduced intrahepatic BH4 results in a decrease in... -
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- Insulin Enhances Nitric Oxide Production in Trabecular Meshwork Cells via De Novo Pathway for Tetrahydrobiopterin Synthesis
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Kim JW
- KMID: 1105029
- Korean J Ophthalmol.
- 2007 Mar;21(1):39-44.
- doi: 10.3341/kjo.2007.21.1.39
PURPOSE: To investigate the effect of insulin on the production of nitric oxide (NO) in the trabecular meshwork (TM) cells and the enzymatic synthetic pathway of tetrahydrobiopterin (BH4) synthesis.... -
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