Results 91 to 100 of about 10,182 (138)

The auto‐oxidation of tetrahydrobiopterin

European Journal of Biochemistry, 1988
The product of the aerobic oxidation of tetrahydrobiopterin, quinonoid dihydrobiopterin, is unstable and rapidly rearranges to form a 7,8‐dihydropteridine. Kaufman [Kaufman, S. (1967) J. Biol. Chem. 242, 3934–3943] identified the stable product produced in 0.1 M phosphate pH 6.8, as 7,8‐dihydrobiopterin. However, Armarego et al. [Armarego, W. L.
M D, Davis, S, Kaufman, S, Milstien
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Tetrahydrobiopterin Radical Enzymology

Chemical Reviews, 2003
AbstractFor Abstract see ChemInform Abstract in Full Text.
Chin-Chuan, Wei, Brian R, Crane, Dennis J, Stuehr   +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, Kathryn, Moseley, Flemming, Guttler   +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.
I C, Dissing, F, Güttler, H, Pakkenberg, H, Lou, A M, Gerdes, C, Lykkelund, V, Rasmussen   +6 more
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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, A, Ponzone, H C, Curtius   +2 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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International database of tetrahydrobiopterin deficiencies

Journal of Inherited Metabolic Disease, 1995
SummaryApproximately 2% of newborns with hyperphenylalaninaemia are deficient in tetrahydrobiopterin. Selective screening must be performed in all instances where hyperphenylalaninaemia is detected by neonatal screening. In the last 20 years, 308 patients with tetrahydrobiopterin deficiencies have been recognized as a result of screening carried out ...
Blau N, Barnes I, Dhondt JL
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Tetrahydrobiopterin metabolism in the Rett disease

Brain and Development, 1985
Tetrahydrobiopterin metabolism was studied in nine Swedish patients with the Rett disease. Normal values were found for the serum biopterin level, urine biopterins level and dihydropterine reductase activity. These findings suggest that a primary disturbance of tetrahydrobiopterin metabolism is unlikely.
A, Sahota, R, Leeming, J, Blair, B, Hagberg   +3 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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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, Ceravolo F, Ferraro S, Procopio D, Moricca MT, Leone G, Salpietro V, Polizzi A, RUGGIERI, MARTINO, Falvo F, Concolino D.   +10 more
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