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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.
GrassPlot is a collaborative vegetation-plot database organised by the Eurasian Dry Grassland Group (EDGG) and listed in the Global Index of Vegetation-Plot Databases (GIVD ID EU-00-003). GrassPlot collects plot records (releves) from grasslands and other open habitats of the Palaearctic biogeographic realm. It focuses on precisely delimited plots of eight standard grain sizes (0.0001; 0.001;... 1,000 m(2)) and on nested-plot series with at least four different grain sizes. The usage of GrassPlot is regulated through Bylaws that intend to balance the interests of data contributors and data users. The current version (v. 1.00) contains data for approximately 170,000 plots of different sizes and 2,800 nested-plot series. The key components are richness data and metadata. However, most included datasets also encompass compositional data. About 14,000 plots have near-complete records of terricolous bryophytes and lichens in addition to vascular plants. At present, GrassPlot contains data from 36 countries throughout the Palaearctic, spread across elevational gradients and major grassland types. GrassPlot with its multi-scale and multi-taxon focus complements the larger international vegetationplot databases, such as the European Vegetation Archive (EVA) and the global database " sPlot". Its main aim is to facilitate studies on the scale-and taxon-dependency of biodiversity patterns and drivers along macroecological gradients. GrassPlot is a dynamic database and will expand through new data collection coordinated by the elected Governing Board. We invite researchers with suitable data to join GrassPlot. Researchers with project ideas addressable with GrassPlot data are welcome to submit proposals to the Governing Board.
We present an evolutionary sequence of a low mass star from the Asymtotic Giant Branch (AGB) through its post- AGB stage, during which its surface chemical composition changes from hydrogen-rich to strongly hydrogen-deficient as consequence of a very late thermal pulse, following the so-called born-again scenario. The internal structure and abundance changes during this pulse are computed with a %newly developed numerical method which allows the physically consistent calculation of stellar layers where thermonuclear and mixing time scale are comparable --- a situation which occurs when the helium flash driven convection zone extends to the hydrogen-rich surface layers during the pulse peak. The final surface mass fractions are [He/C/O]=[0.38/0.36/0.22], where the high oxygen abundance is due to diffusive overshoot employed during the AGB evolution. These models are the first to achieve general agreement with the surface abundance pattern observed in hydrogen-deficient post-AGB stars --- e.g. the PG 1159 stars or the WR-type central stars of planetary nebulae ---, confirming the born-again scenario with a physically consistent calculation and supporting the occurrence of convective overshooting in thermally pulsing AGB stars.
Injection of fluids into deep saline aquifers causes a pore pressure increase in the storage formation, and thus displacement of resident brine. Via hydraulically conductive faults, brine may migrate upwards into shallower aquifers and lead to unwanted salinisation of potable groundwater resources. In the present study, we investigated different scenarios for a potential storage site in the Northeast German Basin using a three-dimensional (3-D) regional-scale model that includes four major fault zones. The focus was on assessing the impact of fault length and the effect of a secondary reservoir above the storage formation, as well as model boundary conditions and initial salinity distribution on the potential salinisation of shallow groundwater resources. We employed numerical simulations of brine injection as a representative fluid. Our simulation results demonstrate that the lateral model boundary settings and the effective fault damage zone volume have the greatest influence on pressure build-up and development within the reservoir, and thus intensity and duration of fluid flow through the faults. Higher vertical pressure gradients for short fault segments or a small effective fault damage zone volume result in the highest salinisation potential due to a larger vertical fault height affected by fluid displacement. Consequently, it has a strong impact on the degree of shallow aquifer salinisation, whether a gradient in salinity exists or the saltwater-freshwater interface lies below the fluid displacement depth in the faults. A small effective fault damage zone volume or low fault permeability further extend the duration of fluid flow, which can persist for several tens to hundreds of years, if the reservoir is laterally confined. Laterally open reservoir boundaries, large effective fault damage zone volumes and intermediate reservoirs significantly reduce vertical brine migration and the potential of freshwater salinisation because the origin depth of displaced brine is located only a few decametres below the shallow aquifer in maximum. The present study demonstrates that the existence of hydraulically conductive faults is not necessarily an exclusion criterion for potential injection sites, because salinisation of shallower aquifers strongly depends on initial salinity distribution, location of hydraulically conductive faults and their effective damage zone volumes as well as geological boundary conditions.
ArcticBeach v1.0
(2022)
In the Arctic, air temperatures are increasing and sea ice is declining, resulting in larger waves and a longer open water season, all of which intensify the thaw and erosion of ice-rich coasts. Climate change has been shown to increase the rate of Arctic coastal erosion, causing problems for Arctic cultural heritage, existing industrial, military, and civil infrastructure, as well as changes in nearshore biogeochemistry. Numerical models that reproduce historical and project future Arctic erosion rates are necessary to understand how further climate change will affect these problems, and no such model yet exists to simulate the physics of erosion on a pan-Arctic scale. We have coupled a bathystrophic storm surge model to a simplified physical erosion model of a permafrost coastline. This Arctic erosion model, called ArcticBeach v1.0, is a first step toward a physical parameterization of Arctic shoreline erosion for larger-scale models. It is forced by wind speed and direction, wave period and height, sea surface temperature, all of which are masked during times of sea ice cover near the coastline. Model tuning requires observed historical retreat rates (at least one value), as well as rough nearshore bathymetry. These parameters are already available on a pan-Arctic scale. The model is validated at three study sites at 1) Drew Point (DP), Alaska, 2) Mamontovy Khayata (MK), Siberia, and 3) Veslebogen Cliffs, Svalbard. Simulated cumulative retreat rates for DP and MK respectively (169 and 170 m) over the time periods studied at each site (2007-2016, and 1995-2018) are found to the same order of magnitude as observed cumulative retreat (172 and 120 m). The rocky Veslebogen cliffs have small observed cumulative retreat rates (0.05 m over 2014-2016), and our model was also able to reproduce this same order of magnitude of retreat (0.08 m). Given the large differences in geomorphology between the study sites, this study provides a proof-of-concept that ArcticBeach v1.0 can be applied on very different permafrost coastlines. ArcticBeach v1.0 provides a promising starting point to project retreat of Arctic shorelines, or to evaluate historical retreat in places that have had few observations.
Injection of fluids into deep saline aquifers causes a pore pressure increase in the storage formation, and thus displacement of resident brine. Via hydraulically conductive faults, brine may migrate upwards into shallower aquifers and lead to unwanted salinisation of potable groundwater resources. In the present study, we investigated different scenarios for a potential storage site in the Northeast German Basin using a three-dimensional (3-D) regional-scale model that includes four major fault zones. The focus was on assessing the impact of fault length and the effect of a secondary reservoir above the storage formation, as well as model boundary conditions and initial salinity distribution on the potential salinisation of shallow groundwater resources. We employed numerical simulations of brine injection as a representative fluid.
Our simulation results demonstrate that the lateral model boundary settings and the effective fault damage zone volume have the greatest influence on pressure build-up and development within the reservoir, and thus intensity and duration of fluid flow through the faults. Higher vertical pressure gradients for short fault segments or a small effective fault damage zone volume result in the highest salinisation potential due to a larger vertical fault height affected by fluid displacement. Consequently, it has a strong impact on the degree of shallow aquifer salinisation, whether a gradient in salinity exists or the saltwater-freshwater interface lies below the fluid displacement depth in the faults. A small effective fault damage zone volume or low fault permeability further extend the duration of fluid flow, which can persist for several tens to hundreds of years, if the reservoir is laterally confined. Laterally open reservoir boundaries, large effective fault damage zone volumes and intermediate reservoirs significantly reduce vertical brine migration and the potential of freshwater salinisation because the origin depth of displaced brine is located only a few decametres below the shallow aquifer in maximum.
The present study demonstrates that the existence of hydraulically conductive faults is not necessarily an exclusion criterion for potential injection sites, because salinisation of shallower aquifers strongly depends on initial salinity distribution, location of hydraulically conductive faults and their effective damage zone volumes as well as geological boundary conditions.
Militär und Gesellschaft in der Frühen Neuzeit = Themenheft: Universitäten im Dreißigjährigen Krieg
(2011)
Die Geschichte der deutschen Universitäten in der Zeit des Dreißigjährigen Krieges gehört traditionell nicht zu den bevorzugten Themen der historischen Forschung – weder der universitäts-, noch der militärgeschichtlichen. Dieses ausgesprochene Desiderat hat spezifisch historiographiegeschichtliche Gründe und ist noch heute für viele Verzerrungen und Einseitigkeiten bei der Darstellung dieser für das deutsche Universitätswesen durchaus zäsurhaften Epoche verantwortlich. Die in diesem Heft präsentierten exemplarischen Studien gehen auf Vorträge eines Workshops des Arbeitskreises Militär und Gesellschaft in der Frühen Neuzeit auf Schloss Friedenstein in Gotha im Mai 2010 zurück und verstehen sich als eine erste Annäherung an ein noch weithin offenes, aber keineswegs ausgeschöpftes Forschungsfeld.