"Viel Lärm um nichts?" : Fachsprachenlehrwerke im Spannungsfeld zwischen Theorie und Praxis ; eine vergleichende Analyse berufs- und fachbezogener DaF-Lehrwerke aus dem Bereich Wirtschaft

Through a comparative analysis of DaF-textbooks (DaF= Deutsch als Fremdsprache: German as a Foreign language) of "business German" and "professional German" I explore in this article as to what extent general language and special language textbooks differ from one another. This study not only highlights similarities and differences in the explored textbooks but also opens up a new perspective on the problems, which arise through the very particular requirements of special language textbooks. The outcome of my study implies that, although special language textbooks differ from general textbooks in their conceptualization, in fact their differences to general language textbooks tend to be marginal.Anhand einer vergleichenden Analyse von DaF-Lehrwerken aus dem Bereich "Wirtschaftsdeutsch" und "Berufsdeutsch" wird der Frage nachgegangen, inwiefern sich gemeinsprachliche und fachsprachliche Lehrwerke voneinander unterscheiden. Dabei werden nicht nur Unterschiede bzw. Gemeinsamkeiten sichtbar gemacht, sondern auch Probleme aufgezeigt, die sich aus den besonderen Anforderungen an Fachsprachenlehrwerke ergeben. Die Ergebnisse der Untersuchung weisen darauf hin, dass sich fachsprachliche Lehrwerke zumeist vor allem idealtypisch von gemeinsprachlichen unterscheiden, in der konkreten Umsetzung sind die Unterschiede bisweilen marginal.A partir da análise comparativa de obras didáticas de DaF (= Deutsch als Fremdsprache: Alemão como Língua Estrangeira) das áreas de "alemão para economistas" e de "alemão para profissionais", este trabalho investiga até que ponto obras didáticas para o ensino da linguagem comum se distinguem de obras didáticas para o ensino de linguagens técnico-científicas. Além das diferenças e das semelhanças das obras didáticas analisadas, esta pesquisa mostra também os problemas surgidos das necessidades específicas de obras didáticas para o ensino de linguagens técnico-científicas. Os resultados da pesquisa apontam que, embora existam diferenças entre a concepção das obras didáticas para o ensino da linguagem comum e aquelas para o ensino de linguagem técnico-científica, na prática, essas diferenças são insignificantes

NMR for food profiling - Long Drink, Short Experiment. An interdisciplinary approach to promoting judgement

Der vorliegende Beitrag stellt ein interdisziplinäres Projekt der Unterrichtsfächer Chemie und Ernährungs- und Verbraucher*innenbildung vor. Es zielt darauf ab, dass Schülerinnen und Schüler eine wissensbasierte Urteilskompetenz entwickeln, um in ihrem Alltag reflektierte Entscheidungen für eine nachhaltige Lebensführung zu treffen. Mit Hilfe dieser Urteilskompetenz können sie ein faktenbasiert begründetes Maß zwischen Konsum und Verzicht finden. (DIPF/Orig.)This article presents an interdisciplinary project of the subjects chemistry and nutrition and consumer education. It aims to develop a knowledge-based judgement competence for decisions for a sustainable lifestyle in everyday life. With this judgement competence, students can find a fact-based, well-founded measure between consumption and renunciation. (DIPF/Orig.

Impacts of water vapor on Saharan air layer radiative heating

Abstract Airborne lidar observations of long-range transported Saharan air layers in the western North Atlantic trades indicate increased amounts of water vapor within the dust layers compared to the surrounding dry free atmosphere. This study investigates the impact of such enhanced water vapor concentrations on radiative heating. Therefore, spatially high resolved airborne high spectral resolution and differential absorption lidar measurements are used for the parametrization of aerosol optical properties and water vapor concentrations in radiative transfer calculations. Heating rates that are calculated under consideration of the measured water vapor distribution strongly differ from heating rates that are derived under assumption of an atmospheric reference water vapor profile which is steadily decreasing with altitude. Results highlight that water vapor represents a major radiative driver for dust layer vertical mixing and the maintenance of bounding inversions at the top and bottom of the dust layer

Novel insights into PORCN mutations, associated phenotypes and pathophysiological aspects.

BACKGROUND: Goltz syndrome (GS) is a X-linked disorder defined by defects of mesodermal- and ectodermal-derived structures and caused by PORCN mutations. Features include striated skin-pigmentation, ocular and skeletal malformations and supernumerary or hypoplastic nipples. Generally, GS is associated with in utero lethality in males and most of the reported male patients show mosaicism (only three non-mosaic surviving males have been described so far). Also, precise descriptions of neurological deficits in GS are rare and less severe phenotypes might not only be caused by mosaicism but also by less pathogenic mutations suggesting the need of a molecular genetics and functional work-up of these rare variants. RESULTS: We report two cases: one girl suffering from typical skin and skeletal abnormalities, developmental delay, microcephaly, thin corpus callosum, periventricular gliosis and drug-resistant epilepsy caused by a PORCN nonsense-mutation (c.283C > T, p.Arg95Ter). Presence of these combined neurological features indicates that CNS-vulnerability might be a guiding symptom in the diagnosis of GS patients. The other patient is a boy with a supernumerary nipple and skeletal anomalies but also, developmental delay, microcephaly, cerebral atrophy with delayed myelination and drug-resistant epilepsy as predominant features. Skin abnormalities were not observed. Genotyping revealed a novel PORCN missense-mutation (c.847G > C, p.Asp283His) absent in the Genome Aggregation Database (gnomAD) but also identified in his asymptomatic mother. Given that non-random X-chromosome inactivation was excluded in the mother, fibroblasts of the index had been analyzed for PORCN protein-abundance and -distribution, vulnerability against additional ER-stress burden as well as for protein secretion revealing changes. CONCLUSIONS: Our combined findings may suggest incomplete penetrance for the p.Asp283His variant and provide novel insights into the molecular etiology of GS by adding impaired ER-function and altered protein secretion to the list of pathophysiological processes resulting in the clinical manifestation of GS

Novel insights into PORCN mutations, associated phenotypes and pathophysiological aspects.

BACKGROUND: Goltz syndrome (GS) is a X-linked disorder defined by defects of mesodermal- and ectodermal-derived structures and caused by PORCN mutations. Features include striated skin-pigmentation, ocular and skeletal malformations and supernumerary or hypoplastic nipples. Generally, GS is associated with in utero lethality in males and most of the reported male patients show mosaicism (only three non-mosaic surviving males have been described so far). Also, precise descriptions of neurological deficits in GS are rare and less severe phenotypes might not only be caused by mosaicism but also by less pathogenic mutations suggesting the need of a molecular genetics and functional work-up of these rare variants. RESULTS: We report two cases: one girl suffering from typical skin and skeletal abnormalities, developmental delay, microcephaly, thin corpus callosum, periventricular gliosis and drug-resistant epilepsy caused by a PORCN nonsense-mutation (c.283C > T, p.Arg95Ter). Presence of these combined neurological features indicates that CNS-vulnerability might be a guiding symptom in the diagnosis of GS patients. The other patient is a boy with a supernumerary nipple and skeletal anomalies but also, developmental delay, microcephaly, cerebral atrophy with delayed myelination and drug-resistant epilepsy as predominant features. Skin abnormalities were not observed. Genotyping revealed a novel PORCN missense-mutation (c.847G > C, p.Asp283His) absent in the Genome Aggregation Database (gnomAD) but also identified in his asymptomatic mother. Given that non-random X-chromosome inactivation was excluded in the mother, fibroblasts of the index had been analyzed for PORCN protein-abundance and -distribution, vulnerability against additional ER-stress burden as well as for protein secretion revealing changes. CONCLUSIONS: Our combined findings may suggest incomplete penetrance for the p.Asp283His variant and provide novel insights into the molecular etiology of GS by adding impaired ER-function and altered protein secretion to the list of pathophysiological processes resulting in the clinical manifestation of GS

A Homozygous PPP1R21 Splice Variant Associated with Severe Developmental Delay, Absence of Speech, and Muscle Weakness Leads to Activated Proteasome Function

PPP1R21 acts as a co-factor for protein phosphatase 1 (PP1), an important serine/threonine phosphatase known to be essential for cell division, control of glycogen metabolism, protein synthesis, and muscle contractility. Bi-allelic pathogenic variants in PPP1R21 were linked to a neurodevelopmental disorder with hypotonia, facial dysmorphism, and brain abnormalities (NEDHFBA) with pediatric onset. Functional studies unraveled impaired vesicular transport as being part of PPP1R21-related pathomechanism. To decipher further the pathophysiological processes leading to the clinical manifestation of NEDHFBA, we investigated the proteomic signature of fibroblasts derived from the first NEDHFBA patient harboring a splice-site mutation in PPP1R21 and presenting with a milder phenotype. Proteomic findings and further functional studies demonstrate a profound activation of the ubiquitin–proteasome system with presence of protein aggregates and impact on cellular fitness and moreover suggest a cross-link between activation of the proteolytic system and cytoskeletal architecture (including filopodia) as exemplified on paradigmatic proteins including actin, thus extending the pathophysiological spectrum of the disease. In addition, the proteomic signature of PPP1R21-mutant fibroblasts displayed a dysregulation of a variety of proteins of neurological relevance. This includes increase proteins which might act toward antagonization of cellular stress burden in terms of pro-survival, a molecular finding which might accord with the presentation of a milder phenotype of our NEDHFBA patient

Inhibitors of dihydroorotate dehydrogenase cooperate with molnupiravir and N4-hydroxycytidine to suppress SARS-CoV-2 replication

Funding Information: We thank Thorsten Wolff, Daniel Bourquain, Jessica Schulz, and Christian Mache from the Robert-Koch Institute and Martin Beer from the Friedrich Loeffler Institute (FLI) for providing isolates of SARS-CoV-2 variants. We thank Anna Kraft and Gabriele Czerwinski (both FLI) for support in the preparation of samples for pathology, and Catherine Hambly (University of Aberdeen) for help with daily energy expenditure measurements. We would like to thank Cathrin Bierwirth (University Medical Center Göttingen), Isabell Schulz, Anne-Kathrin Donner, and Frank-Thorben Peters for excellent technician assistance and Jasmin Fertey and Alexandra Rockstroh for providing the virus stocks for the mice experiment (Fraunhofer Institute IZI Leipzig). We acknowledge support by the Open Access Publication Funds of the Göttingen University. KMS was a member of the Göttingen Graduate School GGNB during this work. This work was funded by the COVID-19 Forschungsnetzwerk Niedersachsen (COFONI) to MD, by the Federal Ministry of Education and Research Germany ( Bundesministerium für Bildung und Forschung; BMBF ; OrganSARS , 01KI2058 ) to SP and TM, and by a grant of the Max Planck Foundation to DG. Declaration of interests AS, HK, EP, and DV are employees of Immunic AG and own shares and/or stock-options of the parent company of Immunic AG, Immunic Inc. Some of the Immunic AG employees also hold patents for the Immunic compounds described in this manuscript (WO2012/001,148, WO03006425). KMS, AD, and MD are employees of University Medical Center Göttingen, which has signed a License Agreement with Immunic AG covering the combination of DHODH inhibitors and nucleoside analogs to treat viral infections, including COVID-19 (inventors: MD, KMS, and AD). The other authors declare no conflict of interest.Peer reviewedPublisher PD