Identification of eight novel coagulation factor XIII subunit A mutations: implied consequences for structure and function

Severe hereditary coagulation factor XIII deficiency is a rare homozygous bleeding disorder affecting one person in every two million individuals. In contrast, heterozygous factor XIII deficiency is more common, but usually not associated with severe hemorrhage such as intracranial bleeding or hemarthrosis. In most cases, the disease is caused by F13A gene mutations. Causative mutations associated with the F13B gene are rarer.

Something to smile about: The interrelationship between attractiveness and emotional expression

Previous studies have suggested a link between the processing of the emotional expression of a face and how attractive it appears. In two experiments we investigated the interrelationship between attractiveness and happiness. In Experiment 1 we presented morphed faces varying in attractiveness and happiness and asked participants to choose the more attractive of two simultaneously presented faces. In the second experiment we used the same stimuli as in Experiment 1 and asked participants to choose the happier face. The results of Experiment 1 revealed that the evaluation of attractiveness is strongly influenced by the intensity of a smile expressed on a face: A happy facial expression could even compensate for relative unattractiveness. Conversely, the findings of Experiment 2 showed that facial attractiveness also influences the evaluation of happiness: It was easier to choose the happier of two faces if the happier face was also more attractive. We discuss the interrelationship of happiness and attractiveness with regard to evolutionary relevance of positive affective status and rewarding effects.

Quantifying differences in land use emission estimates implied by definition discrepancies

The quantification of CO2 emissions from anthropogenic land use and land use change (eLUC) is essential to understand the drivers of the atmospheric CO2 increase and to inform climate change mitigation policy. Reported values in synthesis reports are commonly derived from different approaches (observation-driven bookkeeping and process-modelling) but recent work has emphasized that inconsistencies between methods may imply substantial differences in eLUC estimates. However, a consistent quantification is lacking and no concise modelling protocol for the separation of primary and secondary components of eLUC has been established. Here, we review differences of eLUC quantification methods and apply an Earth System Model (ESM) of Intermediate Complexity to quantify them. We find that the magnitude of effects due to merely conceptual differences between ESM and offline vegetation model-based quantifications is ~ 20 % for today. Under a future business-as-usual scenario, differences tend to increase further due to slowing land conversion rates and an increasing impact of altered environmental conditions on land-atmosphere fluxes. We establish how coupled Earth System Models may be applied to separate secondary component fluxes of eLUC arising from the replacement of potential C sinks/sources and the land use feedback and show that secondary fluxes derived from offline vegetation models are conceptually and quantitatively not identical to either, nor their sum. Therefore, we argue that synthesis studies should resort to the "least common denominator" of different methods, following the bookkeeping approach where only primary land use emissions are quantified under the assumption of constant environmental boundary conditions.