Hans-Christoph Curtius

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Six 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 older(More)
6-Pyruvoyl-tetrahydropterin synthase (PTS), a key enzyme in the synthesis of tetrahydrobiopterin in man, is defective in the most frequent variant of tetrahydrobiopterin-deficient hyperphenylalaninaemia (atypical phenylketonuria). An assay for PTS activity in erythrocytes was developed. It is based on the PTS-catalysed formation of tetrahydrobiopterin from(More)
Administration of a single dose of tetrahydrobiopterin dihydrochloride, 10–20 mg/kg orally, to a patient with dihydrobiopterin deficiency led to disappearance of clinical symptoms for 4 days, normalization of urinary phenylalanine and serotonin and decrease of elevated neopterin for 2–3 days. A dose-dependent stimulation of serotonin production was(More)
The biosynthesis of tetrahydrobiopterin (BH4) from dihydroneopterin triphosphate (NH2P3) was studied in human liver extract. The phosphate-eliminating enzyme (PEE) was purified ~750-fold. The conversion of NH2P3 to BH4 was catalyzed by this enzyme in the presence of partially purified sepiapterin reductase, Mg2+ and NADPH. The PEE is heat stable when heated(More)
Primapterinuria, a recently discovered variant of hyperphenylalaninaemia, is characterized by the excretion of 7-substituted pterins in the patient's urine (Curtius et al 1988). Although 9 patients have already been diagnosed worldwide (Dhondt et al 1987, 1988; Blaskovics and Giudici 1988; Blau et al 1988), the exact metabolic defect is still hypothetical.(More)
From the $Division of Clinical Chemistry, Department of Pediatrics, University of Zurich, CH-8032 Zurich, Switzerland, the §Institute of Medical Genetics, Emst-Moritz-Arndt University, Fleischmannstrasse 42-44, D/O-2200 Greifswald, Germany, the llnstitute of Biochemistry, University of Zurich-Irchel, CH-8057 Zurich, Switzerland, and the 11 Department of(More)
A new form of atypical phenylketonuria coupled with a transient hyperphenylalaninaemia has recently been discovered. The new metabolic disorder is characterized by an accompanying excretion of 7-substituted pterins, i.e. 7-biopterin (primapterin), 6-oxo-7-biopterin and 7-neopterin (anapterin) in the patients' urine (Curtius et al 1988; Dhondt et al 1988).(More)
Fig. 3. Comparison of delta center frequency (0.98-2.93 Hz) between 16 treated schizophrenic patients and 15 normals. There are significant differences in the Mann-Whitney U-test for all electrodes except C4. The smaller columns show delta center frequency for the 6 untreated schizophrenic patients, which should not be compared statistically. They show the(More)
An enzyme which reduces 6-pyruvoyl-tetrahydropterin has been purified to apparent homogeneity from human liver. It consists of a single polypeptide chain with a molecular weight of 35 kDa, has an isoelectric point of 5.9 ± 0.1 and contains no glycosyl residues. The pure enzyme has a specific activity of 450 mU/mg protein at pH 7.0 in 10 mM potassium(More)
t o t h e e n o l i c d o u b l e b o n d a t C ( 2 ' ) C ( 3 ' ) a n d t h u s i n c o r p o r a t e d i n t o t h e f i n a l p r o d u c t , B H 4 . T h e b a s e i n v o l v e d c o u l d p a r t i a l l y e x c h a n g e w i t h s o l v e n t , o r i t m i g h t b e a b i o r t r i f u n c t i o n a l o n e w h i c h d o e s n o t e x c h a n g e . T h(More)