Results 261 to 270 of about 26,569 (299)

Tetrahydrobiopterin: biochemistry and pathophysiology

Biochemical Journal, 2011
BH4 (6R-L-erythro-5,6,7,8-tetrahydrobiopterin) is an essential cofactor of a set of enzymes that are of central metabolic importance, including four aromatic amino acid hydroxylases, alkylglycerol mono-oxygenase and three NOS (NO synthase) isoenzymes. Consequently, BH4 is present in probably every cell or tissue of higher organisms and plays a key role
Werner, E R, Blau, N, Thöny, B
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Tetrahydrobiopterin [PDF]

Current Hypertension Reports, 2008
The endothelium plays a pivotal role in vascular physiology through a variety of factors, foremost of which is nitric oxide (NO). However, the biochemical mechanisms leading to reduced NO availability and subsequent endothelial dysfunction are not clearly understood.
Muhiddin A. Ozkor, Arshed A. Quyyumi
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Tetrahydrobiopterin Radical Enzymology

Chemical Reviews, 2003
AbstractFor Abstract see ChemInform Abstract in Full Text.
Dennis J. Stuehr, Brian R. Crane, Chin Chuan Wei   +2 more
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Tetrahydrobiopterin and Parkinson's disease

Acta Neurologica Scandinavica, 1989
Two patients with Parkinson's disease were treated with 1 g tetrahydrobiopterin (BH4) for 5 days. Clinical improvement was not observed. In the cerebrospinal fluid (CSF) a 4-8 fold increase in the concentration of homovanillic acid (HVA), and a 3-fold increase in the concentration of 5-hydroxyindole acetic acid (5-HIAA) was measured.
Hans C. Lou, A.-M. Gerdes, I. C. Dissing, V. Rasmussen, H. Pakkenberg, F. Güttler, C. Lykkelund   +6 more
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Tetrahydrobiopterin and maternal PKU

Molecular Genetics and Metabolism, 2005
A 29-year-old woman with PKU is presented, who was successfully treated with phenylalanine restriction as well as oral BH4 during this pregnancy, with a normal outcome. Her PAH mutation was R408W/F39L. Remarkably, the blood phenylalanine control was easily accomplished during this pregnancy.
Richard Koch, Kathryn Moseley, Flemming Güttler   +2 more
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Neurological Involvement in Tetrahydrobiopterin Deficiency

Journal of Pediatric Biochemistry, 2016
Tetrahydrobiopterin (BH4) is a natural and essential cofactor for the enzymatic hydroxylation of phenylalanine (Phe) and tyrosine (Tyr), and for two tryptophan hydroxylases, three nitric oxide synthases, and glyceryl-ether monooxygenase. Five separate genetic conditions affecting BH4 synthesis or recycling have been identified so far, including ...
Francesca Falvo, Michele Grisolia, Ferdinando Ceravolo, Daniela Procopio, Vincenzo Salpietro, Vincenzo Salpietro, Italia Mascaro, Daniela Concolino, Agata Polizzi, Stefania Ferraro, Giuseppina Leone, Martino Ruggieri   +11 more
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Maternal Phenylketonuria and Tetrahydrobiopterin

Pediatrics, 2008
Untreated maternal phenylketonuria (PKU) results in a significant occurrence of microcephaly and congenital heart disease in the offspring. Before the documentation of this fact by Lenke and Levy1 in 1980, there was confusion as to the occurrence of these abnormalities.
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Disorders of Phenylalanine and Tetrahydrobiopterin Metabolism

Physician's Guide to the Diagnosis, Treatment, and Follow-Up of Inherited Metabolic Diseases, 2022
N. Blau, F. Spronsen
semanticscholar   +1 more source

Tetrahydrobiopterin induced neonatal tyrosinaemia

European Journal of Pediatrics, 1996
Sir: Transient neonatal tyrosinaemia is thought to be caused by late maturation of the hepatic enzymes 4-hydroxyphenylpyruvate dioxygenase and tyrosine aminotransferase [4]. In addition to prematurity, high protein intake and ascorbate deficiency are suggested as risk factors [2].
Nenad Blau, Dietrich Matern, Michael Beck   +2 more
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