Untersuchung der Umsetzbarkeit eines aeroelastisch ähnlichen Halbmodells im European Transonic Windtunnel

Der Entwurf eines Flügel-Windkanalmodells in unterschiedlichen Materialvarianten für aeroelastische Untersuchungen im Europäischen transsonischen Windkanal (ETW) wird gezeigt. Das Windkanalmodell basiert auf der generischen Konfiguration DLR D150, einer Kurz- bzw. Mittelstreckenkonfiguration (150 PAX, single aisles, Ma_cruise=0.78). Insbesondere steht die Umsetzung der Ähnlichkeitsanforderungen, Großversion zum Modell, im Hinblick auf die Baubarkeit eines solchen Modells im Mittelpunkt

A multinuclear 1H, 13C and 11B solid-state MAS NMR study of 16- and 18-electron organometallic ruthenium and osmium carborane complexes

YesThe first 1H, 13C, 31P and 11B solid state MAS NMR studies of electron- deficient carborane-containing ruthenium and osmium complexes [Ru/Os(p-cym)(1,2-dicarba-closo-dodecaborane-1,2- dithiolate)] are reported. The MAS NMR data from these 16-electron complexes are compared to those of free carborane-ligand and an 18-electron triphenylphosphine ruthenium adduct, and reveal clear spectral differences between 16- and 18-electron organometallic carborane systems in the solid state.We thank the Swiss National Science Foundation (grant no. PA00P2-145308 to NPEB), the ERC (grant no. 247450 to PJS), EPSRC (grant no. EP/F034210/1) and EC COST Action CM1105 for support. JVH thanks EPSRC and the University of Warwick for partial funding of the solid state NMR infrastructure at Warwick, and acknowledges additional support obtained through Birmingham Science City: Innovative Uses for Advanced Materials in the Modern World (West Midlands Centre for Advanced Materials Project 2), with support from Advantage West Midlands (AWM) and partial funding by the European Regional Development Fund (ERDF)

Transition metal catalyzed element–element′ additions to alkynes

The efficient and stereoselective synthesis of, or precursors to, multi-substituted alkenes has attracted substantial interest due to their existence in various industrially and biologically important compounds. One of the most atom economical routes to such alkenes is the transition metal catalyzed hetero element–element′ π-insertion into alkynes. This article provides a thorough up-to-date review on this area of chemistry, including discussions on the mechanism, range of Esingle bondE′ bonds accessible and the stoichiometric/catalytic transition metal mediators employed

In vivo measures of cartilage deformation: patterns in healthy and osteoarthritic female knees using 3T MR imaging

ObjectiveTo explore and to compare the magnitude and spatial pattern of in vivo femorotibial cartilage deformation in healthy and in osteoarthritic (OA) knees.MethodsOne knee each in 30 women (age: 55 ± 6 years; BMI: 28 ± 2.4 kg/m(2); 11 healthy and 19 with radiographic femorotibial OA) was examined at 3Tesla using a coronal fat-suppressed gradient echo SPGR sequence. Regional and subregional femorotibial cartilage thickness was determined under unloaded and loaded conditions, with 50% body weight being applied to the knee in 20° knee flexion during imaging.ResultsCartilage became significantly (p < 0.05) thinner during loading in the medial tibia (-2.7%), the weight-bearing medial femur (-4.1%) and in the lateral tibia (-1.8%), but not in the lateral femur (+0.1%). The magnitude of deformation in the medial tibia and femur tended to be greater in osteoarthritic knees than in healthy knees. The subregional pattern of cartilage deformation was similar for the different stages of radiographic OA.ConclusionOsteoarthritic cartilage tended to display greater deformation upon loading than healthy cartilage, suggesting that knee OA affects the mechanical properties of cartilage. The pattern of in vivo deformation indicated that cartilage loss in OA progression is mechanically driven

Monogenic variants in dystonia: an exome-wide sequencing study

Background Dystonia is a clinically and genetically heterogeneous condition that occurs in isolation (isolated dystonia), in combination with other movement disorders (combined dystonia), or in the context of multisymptomatic phenotypes (isolated or combined dystonia with other neurological involvement). However, our understanding of its aetiology is still incomplete. We aimed to elucidate the monogenic causes for the major clinical categories of dystonia. Methods For this exome-wide sequencing study, study participants were identified at 33 movement-disorder and neuropaediatric specialty centres in Austria, Czech Republic, France, Germany, Poland, Slovakia, and Switzerland. Each individual with dystonia was diagnosed in accordance with the dystonia consensus definition. Index cases were eligible for this study if they had no previous genetic diagnosis and no indication of an acquired cause of their illness. The second criterion was not applied to a subset of participants with a working clinical diagnosis of dystonic cerebral palsy. Genomic DNA was extracted from blood of participants and whole-exome sequenced. To find causative variants in known disorder-associated genes, all variants were filtered, and unreported variants were classified according to American College of Medical Genetics and Genomics guidelines. All considered variants were reviewed in expert round-table sessions to validate their clinical significance. Variants that survived filtering and interpretation procedures were defined as diagnostic variants. In the cases that went undiagnosed, candidate dystonia-causing genes were prioritised in a stepwise workflow. Findings We sequenced the exomes of 764 individuals with dystonia and 346 healthy parents who were recruited between June 1, 2015, and July 31, 2019. We identified causative or probable causative variants in 135 (19%) of 728 families, involving 78 distinct monogenic disorders. We observed a larger proportion of individuals with diagnostic variants in those with dystonia (either isolated or combined) with coexisting non-movement disorder-related neurological symptoms (100 [45%] of 222;excepting cases with evidence of perinatal brain injury) than in those with combined (19 [19%] of 98) or isolated (16 [4%] of 388) dystonia. Across all categories of dystonia, 104 (65%) of the 160 detected variants affected genes which are associated with neurodevelopmental disorders. We found diagnostic variants in 11 genes not previously linked to dystonia, and propose a predictive clinical score that could guide the implementation of exome sequencing in routine diagnostics. In cases without perinatal sentinel events, genomic alterations contributed substantively to the diagnosis of dystonic cerebral palsy. In 15 families, we delineated 12 candidate genes. These include IMPDH2, encoding a key purine biosynthetic enzyme, for which robust evidence existed for its involvement in a neurodevelopmental disorder with dystonia. We identified six variants in IMPDH2, collected from four independent cohorts, that were predicted to be deleterious de-novo variants and expected to result in deregulation of purine metabolism. Interpretation In this study, we have determined the role of monogenic variants across the range of dystonic disorders, providing guidance for the introduction of personalised care strategies and fostering follow-up pathophysiological explorations

Pseudo electron-deficient organometallics: limited reactivity towards electron-donating ligands

YesHalf-sandwich metal complexes are of considerable interest in medicine, material, and nanomaterial chemistry. The design of libraries of such complexes with particular reactivity and properties is therefore a major quest. Here, we report the unique and peculiar reactivity of eight apparently 16-electron half-sandwich metal (ruthenium, osmium, rhodium, and iridium) complexes based on benzene-1,2-dithiolato and 3,6-dichlorobenzene-1,2-dithiolato chelating ligands. These electron-deficient complexes do not react with electron-donor pyridine derivatives, even with the strong σ-donor 4-dimethylaminopyridine (DMAP) ligand. The Ru, Rh, and Ir complexes accept electrons from the triphenylphosphine ligand (σ-donor, π-acceptor), whilst the Os complexes were found to be the first examples of non-electron-acceptor electron-deficient metal complexes. We rationalized these unique properties by a combination of experimental techniques and DFT/TDFT calculations. The synthetic versatility offered by this family of complexes, the low reactivity at the metal center, and the facile functionalization of the non-innocent benzene ligands is expected to allow the synthesis of libraries of pseudo electron-deficient half-sandwich complexes with unusual properties for a large range of applications