232 research outputs found
Ein einfaches Verfahren zur Herstellung anellierter Thiophene
A simple method for the synthesis of fused thiophenes by reaction of agr-carboxymethyl substituted cyclic ketones withLawesson-reagent is described. Considerations concerning the reaction mechanism are given
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Heavy ion injector for the CERN Linac 1
An injector system has been designed to provide a fully stripped oxygen beam for acceleration in the CERN PS complex. An ECR source will provide an O/sup 6/+ beam to a heavy ion RFQ accelerator. The beam from the RFQ will be further accelerated by the CERN Linac 1 (Old Linac) in the 2 ..beta.. lambda-mode to an energy of 12.5 MeV/u at which point it will be fully stripped for subsequent acceleration in the CERN synchrotrons. The specifications of the new equipment and modifications to the existing linear accelerator are described
Schädigung der Schleimhaut der oberen Luftwege durch Dauerinhalation kleinster Konzentrationen von Schwefelwasserstoff
The high current stripper, the charge separator, and their magnets for the beam transfer line to the heavy ion synchrotron SIS
Diagnosis, classification, and genetics of phenylketonuria and tetrahydrobiopterin (BH4) deficiencies
This article summarizes the present knowledge, recent developments, and common pitfalls in the diagnosis, classification, and genetics of hyperphenylalaninemia, including tetrahydrobiopterin (BH4) deficiency. It is a product of the recent workshop organized by the European Phenylketonuria Group in March 2011 in Lisbon, Portugal. Results of the workshop demonstrate that following newborn screening for phenylketonuria (PKU), using tandem mass-spectrometry, every newborn with even slightly elevated blood phenylalanine (Phe) levels needs to be screened for BH4 deficiency. Dried blood spots are the best sample for the simultaneous measurement of amino acids (phenylalanine and tyrosine), pterins (neopterin and biopterin), and dihydropteridine reductase activity from a single specimen. Following diagnosis, the patient's phenotype and individually tailored treatment should be established as soon as possible. Not only blood Phe levels, but also daily tolerance for dietary Phe and potential responsiveness to BH4 are part of the investigations. Efficiency testing with synthetic BH4 (sapropterin dihydrochloride) over several weeks should follow the initial 24-48-hour screening test with 20mg/kg/day BH4. The specific genotype, i.e. the combination of both PAH alleles of the patient, helps or facilitates to determine both the biochemical phenotype (severity of PKU) and the responsiveness to BH4. The rate of Phe metabolic disposal after Phe challenge may be an additional useful tool in the interpretation of phenotype-genotype correlation
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