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Hyperphenylalaninaemia caused by defects in biopterin metabolism
Journal of Inherited Metabolic Disease, 1985AbstractThe hepatic phenylalanine hydroxylating system consists of three essential components, phenylalanine hydroxylase, dihydropteridine reductase and the non‐protein coenzyme, tetrahydrobiopterin. The reductase and the pterin coenzyme are also essential components of the tyrosine and tryptophan hydroxylating systems.
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New aspects in biopterin biosyntheses
1981A human kidney preparation which catalyzes the conversion of D-erythro-dihydroneopterin triphosphate (NeH2P3) to dihydrobiopterin (BH2), sepiapterin, pyruvate and pterin in the presence of Mg++ and NADPH was studied. After incubation with NeH2P3, the formation of Tanaka’s compound X (probably 6-(1’,2’-dioxopropyl)-7,8-dihydropterin) could be proven as ...
H-Ch Curtius +4 more
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Reindarstellung von Biopterin und 7-Biopterin
Hoppe-Seyler´s Zeitschrift für physiologische Chemie, 1962H. Rembold, H. Metzger
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BIOPTERIN DERIVATIVES IN SENILE DEMENTIA
The Lancet, 1979R J, Leeming, J A, Blair, V, Melikian
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Biopterin and Folic Acid Deficiency
The American Journal of Clinical Nutrition, 1967A F, Fleming, H P, Broquist
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[Biopterin and child neurologic disease].
No to hattatsu = Brain and development, 2009Tetrahydrobiopterin (BH4) deficiencies are disorders affecting phenylalanine metabolism in the liver and neurotransmitter biosynthesis in the brain. BH4 is the essential cofactor in the enzymatic hydroxylation of 3 aromatic amino acids (phenylalanine, tyrosine, and tryptophan).
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