University of Graz

About: University of Graz is a education organization based out in Graz, Steiermark, Austria. It is known for research contribution in the topics: Population & Context (language use). The organization has 17934 authors who have published 37489 publications receiving 1110980 citations. The organization is also known as: Carolo Franciscea Graecensis & Karl Franzens Universität.

The extended phenotype of LPS-responsive beige-like anchor protein (LRBA) deficiency

Abstract: Background LPS-responsive beige-like anchor protein (LRBA) deficiency is a primary immunodeficiency caused by biallelic mutations in LRBA that abolish LRBA protein expression. Objective We sought to report the extended phenotype of LRBA deficiency in a cohort of 22 LRBA-deficient patients. Methods Clinical criteria, protein detection, and genetic sequencing were applied to diagnose LRBA deficiency. Results Ninety-three patients met the inclusion criteria and were considered to have possible LRBA deficiency. Twenty-four patients did not express LRBA protein and were labeled as having probable LRBA deficiency, whereas 22 were genetically confirmed as having definitive LRBA deficiency, with biallelic mutations in LRBA . Seventeen of these were novel and included homozygous or compound heterozygous mutations. Immune dysregulation (95%), organomegaly (86%), recurrent infections (71%), and hypogammaglobulinemia (57%) were the main clinical complications observed in LRBA-deficient patients. Although 81% of LRBA-deficient patients had normal T-cell counts, 73% had reduced regulatory T (Treg) cell numbers. Most LRBA-deficient patients had low B-cell subset counts, mainly in switched memory B cells (80%) and plasmablasts (92%), with a defective specific antibody response in 67%. Of the 22 patients, 3 are deceased, 2 were treated successfully with hematopoietic stem cell transplantation, 7 are receiving immunoglobulin replacement, and 15 are receiving immunosuppressive treatment with systemic corticosteroids alone or in combination with steroid-sparing agents. Conclusion This report describes the largest cohort of patients with LRBA deficiency and offers guidelines for physicians to identify LRBA deficiency, supporting appropriate clinical management.

Tropospheric Ozone Assessment Report:Assessment of global-scale model performance for global and regional ozone distributions, variability, and trends

Abstract: The goal of the Tropospheric Ozone Assessment Report (TOAR) is to provide the research community with an up-to-date scientific assessment of tropospheric ozone, from the surface to the tropopause. While a suite of observations provides significant information on the spatial and temporal distribution of tropospheric ozone, observational gaps make it necessary to use global atmospheric chemistry models to synthesize our understanding of the processes and variables that control tropospheric ozone abundance and its variability. Models facilitate the interpretation of the observations and allow us to make projections of future tropospheric ozone and trace gas distributions for different anthropogenic or natural perturbations. This paper assesses the skill of current-generation global atmospheric chemistry models in simulating the observed present-day tropospheric ozone distribution, variability, and trends. Drawing upon the results of recent international multi-model intercomparisons and using a range of model evaluation techniques, we demonstrate that global chemistry models are broadly skillful in capturing the spatio-temporal variations of tropospheric ozone over the seasonal cycle, for extreme pollution episodes, and changes over interannual to decadal periods. However, models are consistently biased high in the northern hemisphere and biased low in the southern hemisphere, throughout the depth of the troposphere, and are unable to replicate particular metrics that define the longer term trends in tropospheric ozone as derived from some background sites. When the models compare unfavorably against observations, we discuss the potential causes of model biases and propose directions for future developments, including improved evaluations that may be able to better diagnose the root cause of the model-observation disparity. Overall, model results should be approached critically, including determining whether the model performance is acceptable for the problem being addressed, whether biases can be tolerated or corrected, whether the model is appropriately constituted, and whether there is a way to satisfactorily quantify the uncertainty.

An Algorithmic Framework for the Exact Solution of the Prize-Collecting Steiner Tree Problem

Abstract: The Prize-Collecting Steiner Tree Problem (PCST) on a graph with edge costs and vertex profits asks for a subtree minimizing the sum of the total cost of all edges in the subtree plus the total profit of all vertices not contained in the subtree. PCST appears frequently in the design of utility networks where profit generating customers and the network connecting them have to be chosen in the most profitable way. Our main contribution is the formulation and implementation of a branch-and-cut algorithm based on a directed graph model where we combine several state-of-the-art methods previously used for the Steiner tree problem. Our method outperforms the previously published results on the standard benchmark set of problems. We can solve all benchmark instances from the literature to optimality, including some of them for which the optimum was not known. Compared to a recent algorithm by Lucena and Resende, our new method is faster by more than two orders of magnitude. We also introduce a new class of more challenging instances and present computational results for them. Finally, for a set of large-scale real-world instances arising in the design of fiber optic networks, we also obtain optimal solution values.

De‐racemization of Enantiomers versus De‐epimerization of Diastereomers—Classification of Dynamic Kinetic Asymmetric Transformations (DYKAT)

Abstract: The isolation of single stereoisomers from a racemic (or diastereomeric) mixture by enzymatic or chemical resolution techniques goes in hand with the disposal of 50% (racemate) or more (diastereomeric mixtures) of the "undesired" substrate isomer(s). In order to circumvent this drawback, dynamic systems have been developed for the de-racemization of enantiomers and the de-epimerizations of diastereomers. Key strategies within this area are discussed and are classified according to their underlying kinetics, that is, dynamic kinetic resolution (DKR), dynamic kinetic asymmetric transformations (DYKAT), and hybrids between both of them. Finally, two novel types of DYKAT are defined.