- 関
- sapropterin
WordNet
- a complex consisting of an organic base in association with hydrogen chloride
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English Journal
- Propofol protects against high glucose-induced endothelial dysfunction in human umbilical vein endothelial cells.
- Zhu M, Chen J, Tan Z, Wang J.SourceDepartment of Anaesthesiology, Fudan University, People's Repubic of China.
- Anesthesia and analgesia.Anesth Analg.2012 Feb;114(2):303-9. doi: 10.1213/ANE.0b013e31823f0c42. Epub 2011 Dec 9.
- BACKGROUND: Hyperglycemia, via peroxynitrite-mediated endothelial nitric oxide synthase (eNOS) enzymatic uncoupling, induced endothelial dysfunction. Propofol has been reported to improve high glucose-induced endothelial dysfunction. However, its mechanisms of action remain unclear. We hypothesized
- PMID 22156331
- Sapropterin hydrochloride: enzyme enhancement therapy for phenylketonuria.
- Lachmann R.AbstractPhenylketonuria (PKU) is an inherited disorder of amino acid metabolism caused by deficiency of the enzyme phenylalanine hydroxylase (PAH). Historically PKU was a common genetic cause of severe learning difficulties and developmental delay, but with the introduction of newborn screening and early dietary management, it has become a treatable disease and people born with PKU should now have IQs and achievements similar to their peers. Dietary treatment, however, involves lifestyle changes that pervade most aspects of daily life for an individual and their family. A simple pharmacological treatment for PKU would have a great appeal. Sapropterin hydrochloride is a synthetic form of tetrahydrobiopterin, the cofactor for PAH. A proportion of mutant PAH enzymes show enhanced activity in the presence of pharmacological doses of sapropterin and, for some patients with milder forms of PKU, sapropterin can effectively lower plasma phenylalanine levels. This article discusses the potential place for sapropterin in the management of PKU and how this expensive orphan drug is being integrated into patient care in different healthcare systems.
- Therapeutic advances in endocrinology and metabolism.Ther Adv Endocrinol Metab.2011 Jun;2(3):127-33. doi: 10.1177/2042018811402248.
- Phenylketonuria (PKU) is an inherited disorder of amino acid metabolism caused by deficiency of the enzyme phenylalanine hydroxylase (PAH). Historically PKU was a common genetic cause of severe learning difficulties and developmental delay, but with the introduction of newborn screening and early di
- PMID 23148178
- New era in treatment for phenylketonuria: Pharmacologic therapy with sapropterin dihydrochloride.
- Harding CO.SourceDepartments of Molecular and Medical Genetics and Pediatrics, Oregon Health & Science University, Portland, Oregon, USA.
- Biologics : targets & therapy.Biologics.2010 Aug 9;4:231-6.
- Oral administration of sapropterin hydrochloride, recently approved for use by the US Food and Drug Administration and the European Commission, is a novel approach for the treatment of phenylketonuria (PKU), one of the most common inborn errors of metabolism. PKU is caused by an inherited deficiency
- PMID 20714359
Japanese Journal
- 塩酸サプロブテリン(SUNO588)のラットにおける体内動態 (Studies on metabolism and disposition of sapropterin hydrochloride (SUN 0588) L-erythro-tetrahydrobiopterin hydrochloride in rats)
Related Links
- Ther Adv Endocrinol Metab. 2011 Jun;2(3):127-33. doi: 10.1177/ 2042018811402248. Sapropterin hydrochloride: enzyme enhancement therapy for phenylketonuria. Lachmann R. Phenylketonuria (PKU) is an inherited disorder of amino acid ...
- Sapropterin hydrochloride is a synthetic form of tetrahydrobiopterin, the cofactor for PAH. A proportion of mutant PAH enzymes show enhanced activity in the presence of pharmacological doses of sapropterin and, for some patients with milder ...
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- sapropterin
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- 塩酸サプロプテリン sapropterin hydrochloride
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サプロプテリン
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- sapropterin hydrochloride
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塩酸塩、ハイドロクロライド