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Mitochondriopathies often present clinically as multisystemic disorders of primarily high-energy consuming organs. Assembly, turnover, and surveillance of mitochondrial proteins are essential for mitochondrial function and a key task of AAA family members of metalloproteases. We identified a homozygous mutation in the nuclear encoded mitochondrial escape 1-like 1 gene YME1L1, member of the AAA protease family, as a cause of a novel mitochondriopathy in a consanguineous pedigree of Saudi Arabian descent. The homozygous missense mutation, located in a highly conserved region in the mitochondrial pre-sequence, inhibits cleavage of YME1L1 by the mitochondrial processing peptidase, which culminates in the rapid degradation of YME1L1 precursor protein. Impaired YME1L1 function causes a proliferation defect and mitochondrial network fragmentation due to abnormal processing of OPA1. Our results identify mutations in YME1L1 as a cause of a mitochondriopathy with optic nerve atrophy highlighting the importance of YME1L1 for mitochondrial functionality in humans.
Due to their multifunctionality, tablets offer tremendous advantages for research on handwriting dynamics or for interactive use of learning apps in schools. Further, the widespread use of tablet computers has had a great impact on handwriting in the current generation. But, is it advisable to teach how to write and to assess handwriting in pre- and primary schoolchildren on tablets rather than on paper? Since handwriting is not automatized before the age of 10 years, children's handwriting movements require graphomotor and visual feedback as well as permanent control of movement execution during handwriting. Modifications in writing conditions, for instance the smoother writing surface of a tablet, might influence handwriting performance in general and in particular those of non-automatized beginning writers. In order to investigate how handwriting performance is affected by a difference in friction of the writing surface, we recruited three groups with varying levels of handwriting automaticity: 25 preschoolers, 27 second graders, and 25 adults. We administered three tasks measuring graphomotor abilities, visuomotor abilities, and handwriting performance (only second graders and adults). We evaluated two aspects of handwriting performance: the handwriting quality with a visual score and the handwriting dynamics using online handwriting measures [e.g., writing duration, writing velocity, strokes and number of inversions in velocity (NIV)]. In particular, NIVs which describe the number of velocity peaks during handwriting are directly related to the level of handwriting automaticity. In general, we found differences between writing on paper compared to the tablet. These differences were partly task-dependent. The comparison between tablet and paper revealed a faster writing velocity for all groups and all tasks on the tablet which indicates that all participants—even the experienced writers—were influenced by the lower friction of the tablet surface. Our results for the group-comparison show advancing levels in handwriting automaticity from preschoolers to second graders to adults, which confirms that our method depicts handwriting performance in groups with varying degrees of handwriting automaticity. We conclude that the smoother tablet surface requires additional control of handwriting movements and therefore might present an additional challenge for learners of handwriting.
Due to their multifunctionality, tablets offer tremendous advantages for research on handwriting dynamics or for interactive use of learning apps in schools. Further, the widespread use of tablet computers has had a great impact on handwriting in the current generation. But, is it advisable to teach how to write and to assess handwriting in pre- and primary schoolchildren on tablets rather than on paper? Since handwriting is not automatized before the age of 10 years, children's handwriting movements require graphomotor and visual feedback as well as permanent control of movement execution during handwriting. Modifications in writing conditions, for instance the smoother writing surface of a tablet, might influence handwriting performance in general and in particular those of non-automatized beginning writers. In order to investigate how handwriting performance is affected by a difference in friction of the writing surface, we recruited three groups with varying levels of handwriting automaticity: 25 preschoolers, 27 second graders, and 25 adults. We administered three tasks measuring graphomotor abilities, visuomotor abilities, and handwriting performance (only second graders and adults). We evaluated two aspects of handwriting performance: the handwriting quality with a visual score and the handwriting dynamics using online handwriting measures [e.g., writing duration, writing velocity, strokes and number of inversions in velocity (NIV)]. In particular, NIVs which describe the number of velocity peaks during handwriting are directly related to the level of handwriting automaticity. In general, we found differences between writing on paper compared to the tablet. These differences were partly task-dependent. The comparison between tablet and paper revealed a faster writing velocity for all groups and all tasks on the tablet which indicates that all participants—even the experienced writers—were influenced by the lower friction of the tablet surface. Our results for the group-comparison show advancing levels in handwriting automaticity from preschoolers to second graders to adults, which confirms that our method depicts handwriting performance in groups with varying degrees of handwriting automaticity. We conclude that the smoother tablet surface requires additional control of handwriting movements and therefore might present an additional challenge for learners of handwriting.
Land-use intensification is a major driver of biodiversity loss(1,2). Alongside reductions in local species diversity, biotic homogenization at larger spatial scales is of great concern for conservation. Biotic homogenization means a decrease in beta-diversity (the compositional dissimilarity between sites). Most studies have investigated losses in local (alpha)-diversity(1,3) and neglected biodiversity loss at larger spatial scales. Studies addressing beta-diversity have focused on single or a few organism groups (for example, ref. 4), and it is thus unknown whether land-use intensification homogenizes communities at different trophic levels, above-and belowground. Here we show that even moderate increases in local land-use intensity (LUI) cause biotic homogenization across microbial, plant and animal groups, both above- and belowground, and that this is largely independent of changes in alpha-diversity. We analysed a unique grassland biodiversity dataset, with abundances of more than 4,000 species belonging to 12 trophic groups. LUI, and, in particular, high mowing intensity, had consistent effects on beta-diversity across groups, causing a homogenization of soil microbial, fungal pathogen, plant and arthropod communities. These effects were nonlinear and the strongest declines in beta-diversity occurred in the transition from extensively managed to intermediate intensity grassland. LUI tended to reduce local alpha-diversity in aboveground groups, whereas the alpha-diversity increased in belowground groups. Correlations between the alpha-diversity of different groups, particularly between plants and their consumers, became weaker at high LUI. This suggests a loss of specialist species and is further evidence for biotic homogenization. The consistently negative effects of LUI on landscape-scale biodiversity underscore the high value of extensively managed grasslands for conserving multitrophic biodiversity and ecosystem service provision. Indeed, biotic homogenization rather than local diversity loss could prove to be the most substantial consequence of land-use intensification.
Our study addresses the following research questions: Are there differences between handwriting movements on paper and on a tablet computer? Can experienced writers, such as most adults, adapt their graphomotor execution during writing to a rather unfamiliar surface for instance a tablet computer? We examined the handwriting performance of adults in three tasks with different complexity: (a) graphomotor abilities, (b) visuomotor abilities and (c) handwriting. Each participant performed each task twice, once on paper and once on a tablet computer with a pen. We tested 25 participants by measuring their writing duration, in air time, number of pen lifts, writing velocity and number of inversions in velocity. The data were analyzed using linear mixed-effects modeling with repeated measures. Our results reveal differences between writing on paper and on a tablet computer which were partly task-dependent. Our findings also show that participants were able to adapt their graphomotor execution to the smoother surface of the tablet computer during the tasks. (C) 2016 Elsevier B.V. All rights reserved.
Im Graduiertenkolleg NatRiskChange der Universität Potsdam und anderen Forschungseinrichtungen werden beobachtete sowie zukünftig mögliche Veränderungen von Naturgefahren untersucht. Teil des strukturierten Doktorandenprogramms sind sogenannte Task-Force-Einsätze, bei denen die Promovierende zeitlich begrenzt ein aktuelles Ereignis auswerten. Im Zuge dieser Aktivität wurde die Sturzflut vom 29.05.2016 in Braunsbach (Baden-Württemberg) untersucht.
In diesem Bericht werden erste Auswertungen zur Einordnung der Niederschläge, zu den hydrologischen und geomorphologischen Prozessen im Einzugsgebiet des Orlacher Bachs sowie zu den verursachten Schäden beleuchtet.
Die Region war Zentrum extremer Regenfälle in der Größenordnung von 100 mm innerhalb von 2 Stunden. Das 6 km² kleine Einzugsgebiet hat eine sehr schnelle Reaktionszeit, zumal bei vorgesättigtem Boden. Im steilen Bachtal haben mehrere kleinere und größere Hangrutschungen über 8000 m³ Geröll, Schutt und Schwemmholz in das Gewässer eingetragen und möglicherweise kurzzeitige Aufstauungen und Durchbrüche verursacht. Neben den großen Wassermengen mit einer Abflussspitze in einer Größenordnung von 100 m³/s hat gerade die Geschiebefracht zu großen Schäden an den Gebäuden entlang des Bachlaufs in Braunsbach geführt.