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Biosynthesis and metabolism of pterins in peripheral blood mononuclear cells and leukemia lines of man and mouse.
- G. Schoedon, J. Troppmair, A. Fontana, C. Huber, H. Curtius, A. Niederwieser
- BiologyEuropean journal of biochemistry
- 1 July 1987
Neopterin is an unspecific marker for the activation of the cellular immune system and is explained by the different regulation of the rate-limiting steps of tetrahydrobiopterin biosynthesis in man and in mouse.
Decreased vigilance and neurotransmitter synthesis after discontinuation of dietary treatment for phenylketonuria in adolescents
- H. Lou, F. Güttler, C. Lykkelund, P. Bruhn, A. Niederwieser
- MedicineEuropean Journal of Pediatrics
- 1 May 1985
Preliminary findings demonstrate that there may be hazards in the discontinuation of dietary treatment, even in adolescents or young adults, for neurotransmitter metabolism and mental function.
Differential diagnosis of tetrahydrobiopterin deficiency
- A. Niederwieser, A. Ponzone, H. Curtius
- Medicine, BiologyJournal of Inherited Metabolic Disease
- 1 March 1985
Since subvariants of patients with BH4 deficiency exist, homovanillic acid, 5-hydroxyindole acetic acid, pterins, phenylalanine, and tyrosine in cerebrospinal fluid should be measured for diagnosis and the control of therapy.
Atypical phenylketonuria due to tetrahydrobiopterin deficiency. Diagnosis and treatment with tetrahydrobiopterin, dihydrobiopterin and sepiapterin.
Tetrahydrobiopterin therapy of atypical phenylketonuria due to defective dihydrobiopterin biosynthesis.
A patient with atypical phenylketonuria (defective BH2 synthesis), detected at age 6 months because of severe muscle hypotonia and serum phenylalanine of 20 mg/100 ml, had normal activities of…
Serotonin and dopamine synthesis in phenylketonuria.
- H. Curtius, A. Niederwieser, H. Schmidt
- Biology, MedicineAdvances in experimental medicine and biology
In two patients with dihydrobiopterin synthetase deficiency, the dopamine, and even more pronounced the serotonin, excretions are considerably reduced and the dopamine excretion was reduced to about 50% and the serotonin excretion to only 10% compared to controls.
GTP cyclohydrolase I deficiency, a new enzyme defect causing hyperphenylalaninemia with neopterin, biopterin, dopamine, and serotonin deficiencies and muscular hypotonia
- A. Niederwieser, N. Blau, M. Wang, P. Joller, M. Atarés, J. Cardesa-García
- Medicine, BiologyEuropean Journal of Pediatrics
- 1 February 1984
This is the first case of a new variant of hyperphenylalaninemia in which the formation of dihydroneopterin triphosphate and its pterin metabolites in liver is markedly diminished, and it is concluded that the molybdenum cofactor of these enzymes may not be derived from dihydroneship in man.
GTP-cyclohydrolases: a review.
- N. Blau, A. Niederwieser
- Biology, Computer ScienceJournal of clinical chemistry and clinical…
- 1 April 1985
The occurrence, properties and functions of GTP-cyclohydrolases in mammalian and non-mammalian systems is reviewed and a variant form of tetrahydrobiopterin-deficient hyperphenylalaninaemia is described.
Interferon-gamma enhances biosynthesis of pterins in peripheral blood mononuclear cells by induction of GTP-cyclohydrolase I activity.
- G. Schoedon, J. Troppmair, G. Adolf, C. Huber, A. Niederwieser
- BiologyJournal of interferon research
- 1 December 1986
It appears that IFN-gamma selectively stimulates the early steps of pterin biosynthesis in macrophages, thereby leading to accumulation and excretion of dihydroneopterin and neopterin.
 Thin-layer chromatography of amino acids and derivatives.
- A. Niederwieser
- BiologyMethods in enzymology