Results 231 to 240 of about 23,011 (262)
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Tetrahydrobiopterin

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.
Chin-Chuan, Wei   +2 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   +2 more
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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].
N, Blau, M, Beck, D, Matern
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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.
I C, Dissing   +6 more
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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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Differential Diagnosis of Tetrahydrobiopterin Deficiency

Journal of Inherited Metabolic Disease, 1985
AbstractSix hundred and seventy‐three children (483 newborns and 190 older selected children) were screened for tetrahydrobiopterin (BH4) deficiency by HPLC of urine pterins and BH4 load test. One patient with GTP cyclohydrolase I deficiency, 36 patients with dihydrobiopterin synthetase (DHBS) deficiency (of which six were in the newborn and 30 in the ...
A, Niederwieser   +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 ...
Mascaro I   +10 more
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