ABCC6 deficiency is caused by variants in the ABCC6 gene, leading to dysfunction of the ABCC6 protein. This can result in the development of the infantile phenotype, generalized arterial calcification of infancy type 2 (GACI2), or the adolescent-adult phenotype, pseudoxanthoma elasticum (PXE). To date, the impact of ABCC6 deficiency in a pediatric population has not been comprehensively studied. This analysis aimed to collectively characterize the genotypic and phenotypic presentation of ABCC6 deficiency in the pediatric population. A literature review and analysis identified 95 individuals with ABCC6 variant(s) and documented clinical manifestations occurring from ages 0 to < 18 years. Of the 133 ABCC6 variants found, 57.1% were pathogenic, 26.3% were likely pathogenic, and 10.5% were of uncertain significance. A high prevalence of ectopic calcification with cardiovascular, dermatologic, neurologic, and ocular complications was observed across this pediatric ABCC6 deficiency cohort. While 56% were diagnosed with GACI and 44% with PXE, many individuals exhibited overlapping features of both conditions. There was a relatively high frequency of clinical manifestations through 6 years of age with lower frequency from ages 7 to 18 years. There was significant phenotypic variability observed across patients harboring the same ABCC6 variant(s). These findings demonstrate the wide spectrum and early emergence of cardiovascular, neurologic, and ocular complications in pediatric patients with ABCC6 deficiency. Given the variability of clinical presentations and absence of systematic phenotype characterization, pediatric ABCC6 deficiency is likely underdiagnosed. Establishing guidelines for assessment, genetic diagnosis, monitoring, and prognostic counseling would assist in the timely diagnosis and multidisciplinary management of pediatric patients with ABCC6 deficiency.

Urban green spaces are essential for enhancing ecosystem services, biodiversity, and human well-being, yet their sustainability relies on the long-term vitality and aesthetic quality of trees. This study examined changes in tree vitality and decorativeness in Akademski Park, Belgrade, Serbia, over a ten-year period (2015–2025) to assess species-specific responses to urban environmental stress. Data were collected using the basic Visual Tree Assessment (VTA) method, evaluating 132 trees in 2015 and 121 in 2025 across 22 species. Both vitality and aesthetic values were rated on a five-point scale, and differences were tested using the Wilcoxon signed-rank test for paired samples. Results showed a slight overall decline in vitality (from 3.9 to 3.7) and a stable decorativeness score (3.6–3.7). Significant reductions in vitality were observed for Cedrus atlantica (p=0.002), while Tilia cordata showed improvement (p=0.037). Platanus × acerifolia exhibited a significant increase in decorativeness (p=0.040), confirming its adaptability to urban conditions. Other species displayed minor or non-significant changes, indicating general stability in tree health and visual quality. The findings suggest that Akademski Park has maintained its ecological and aesthetic function despite environmental pressures. Long-term monitoring and species-specific management are essential for sustaining urban tree vitality and optimising the resilience of green infrastructure under changing climatic conditions.

Hybridization and polyploidization are the main drivers of diversification in Sorbus, and have generated numerous evolutionary lineages across Europe. Newly derived polyploid lineages usually reproduce via apomixis and represent novel genetic and morphological entities, usually circumscribed as distinct species. Whereas Sorbus has been thoroughly studied in Central and Western Europe, its diversity in the Balkans remains less explored. This is especially the case for Sorbus subgen. Tormaria that includes derivates of crosses between S. subgen. Aria and S. torminalis. Here, we report the discovery of a triploid population of S. subgen. Tormaria in south-western Bosnia and Herzegovina. We performed amplified fragment length polymorphism fingerprinting, evaluation of nuclear microsatellites, plastid DNA sequencing, flow cytometric ploidy level estimation, reproduction mode screening, and morphological characterization to disentangle its relationship to the previously reported diploid populations from Bosnia and Herzegovina and congeners from Central Europe, as well as to both parents. The data revealed that the aforementioned population includes mostly triploid facultative apomicts that are genetically divergent from other taxa. We therefore describe it as a new species, Sorbus hercegovinae, and provide its description, distribution data, and conservation status. Our study highlights the Balkan Peninsula as one of the hotspots of whitebeam diversity.

Vehicular communication via V2X networks significantly improves road safety, but is vulnerable to data manipulation, which can lead to serious incidents. To address this threat, misbehavior detection systems (MBDs) have been developed to detect such misbehavior. In order to enhance the detection of data manipulation, trust assessment in V2X networks has recently gained increasing attention. Trust assessment takes into account the output of various security mechanisms such as MBDs or Intrusion Detection Systems (IDSs) to detect misbehavior. One particular challenge in trust assessment is the appropriate quantification of the output of these security mechanisms into trust opinions. In this paper, we propose a trust quantification methodology that transforms the output of an MBD into a subjective logic opinion. Furthermore, we apply a hyperparameter optimization approach to determine the optimal parameter set for an MBD. Our evaluation using three MBD variants shows that the optimization approach significantly increased the detectionperformance of all MBDs. The MBD variant that used the optimization approach and our proposed trust quantification methodology achieved the best performance, increasing the F1 score by over 13 % compared to other state-of-the-art MBD variants analyzed in this work.

Understanding the mechanical behavior of lunar regolith simulants is crucial for progressing engineering analysis in surface infrastructure design and testbed evaluation. This study provides a detailed geotechnical characterization of the Colorado School of Mines Lunar Highlands Type-Testbed simulants, including a new highland-type lunar simulant integrated into the Mines Lunar Surface Simulator testbed. It includes the determination of key geotechnical properties such as particle size distribution, density, compressibility, cohesion, friction angle, and particle shape, using standardized ASTM testing procedures. Key findings show that while particle size distribution similarities exist among the simulants and actual lunar regolith, there are significant differences in density-dependent mechanical behaviors such as shear strength, compressibility, and deformation responses. These results emphasize the complexity of simulant fidelity and its impact on testbed experiments when simulating mid-Technology Readiness Level environments. Additionally, we emphasize the importance of density-specific testing for evaluating both geotechnical properties and simulant performance. This work presents the first comprehensive publication of the full geotechnical profile of Colorado School of Mines highland simulants and highlights the importance of using multiple parameters beyond just particle size distribution to evaluate simulant fidelity. Future research will concentrate on characterizing testbed variability and assessing properties across different density states to improve the application of lunar simulants in engineering analysis and testbed experimentation.

In response to the 2020 Update of the European Strategy for Particle Physics, the Future Circular Collider (FCC) Feasibility Study was launched as an international collaboration hosted by CERN. This report describes the FCC integrated programme, which consists of two stages: an electron-positron collider (FCC-ee) in the first phase, serving as a high-luminosity Higgs, top, and electroweak factory; followed by a proton-proton collider (FCC-hh) at the energy frontier in the second phase. The FCC-ee is designed to operate at four key centre-of-mass energies: the Z pole, the WW pair production threshold, the ZH production peak, and the top/anti-top production threshold—each delivering the highest possible luminosities to four experiments. Over 15 years of operation, FCC-ee will produce more than 6 trillion Z bosons, 200 million WW pairs, nearly 3 million Higgs bosons, and 2 million top anti-top pairs. Precise energy calibration at the Z pole and WW threshold will be achieved through frequent resonant depolarisation of pilot bunches. The sequence of operation modes between the Z, WW, and ZH substages remains flexible. The FCC-hh will operate at a centre-of-mass energy of approximately 85 TeV—nearly an order of magnitude higher than the LHC—and is designed to deliver 5 to 10 times the integrated luminosity of the upcoming High-Luminosity LHC. Its mass reach for direct discovery extends to several tens of TeV. In addition to proton-proton collisions, the FCC-hh is capable of supporting ion-ion, ion-proton, and lepton-hadron collision modes. This second volume of the Feasibility Study Report presents the complete design of the FCC-ee collider, its operation and staging strategy, the full-energy booster and injector complex, required accelerator technologies, safety concepts, and technical infrastructure. It also includes the design of the FCC-hh hadron collider, development of high-field magnets, hadron injector options, and key technical systems for FCC-hh.

USB4 Version 2.0 is the first generation to adopt Pulse Amplitude Modulation-3 (PAM-3) signaling while increasing the data rate to 40 Gb/s, thus needing PAM-3 transceivers that can satisfy its various requirements. This paper presents the architectural choices and trade-offs in high-speed PAM-3 wireline transmitters. A quarter-rate clocking architecture is adopted to investigate the impact of different driver and Feed-Forward Equalizer (FFE) implementations on complexity, area, and power consumption across varying data rates. Three transmitter architectures are designed and simulated in 22 nm CMOS technology, following the standard as a guideline. Analog and digital FFE implementations are combined with voltage-mode, conventional and DAC driver implementations. All three designs employ a 4-tap FFE as required by the specification. Among them, the DAC-based design achieves the highest energy efficiency and smallest area, whereas the all-analog solution ranks second. The analog driver with digital FFE results in the most power and area consumption.

Geodetic survey is a comprehensive process for collecting both graphical and descriptive data related to real estate, primarily for the purpose of establishing cadastral records and land registration. The graphical data serve as the fundamental basis for creating various small-scale cartographic representations. Geodetic surveys in Bosnia and Herzegovina have been conducted twice. The first comprehensive systematic survey was carried out by the administration of the Austro-Hungarian Monarchy, covering the entire territory of Bosnia and Herzegovina at that time. The second comprehensive survey began after the end of World War II and lasted several decades, though not all of Bosnia and Herzegovina's territory was surveyed during this period. The result of the geodetic survey of Bosnia and Herzegovina during the rule of the Austro-Hungarian Monarchy was the creation of cadastral maps that did not include representations of relief features. Cadastral plans were produced at scales of 1:6250, 1:3125, 1:1562.5, and 1:781.25. The scale of the cadastral plan depended on the built-up density of the surveyed territory. The real estate data collected during this survey are referred to in Bosnian-Herzegovinian geodetic terminology by a unique name – the “old survey”. Conversely, the geodetic surveys conducted during socialist Bosnia and Herzegovina included, among other features, representations of the territory on cadastral plans created at scales ranging from 1:5000 to 1:500. These plans incorporated a separate layer depicting terrain elevation, which allowed for the creation of topographic-cadastral plans by overlaying these elevation layers onto cadastral maps. In Bosnian geodetic terminology, the data collected during this period are referred to as the “new survey”. Cadastral plans from both the old and new surveys are collectively referred to as large-scale map representations. From these representations, a multitude of small-scale map representations can be generated. Despite the availability of these data, it's notable that throughout the history of Bosnia and Herzegovina, the graphical depiction of the region has been portrayed on numerous small-scale cartographic representations created across various historical periods. After the completion of the geodetic survey of the settlement of Prusac, situated within the present-day municipality of Donji Vakuf, by the administration of the Austro-Hungarian Monarchy, a cadastral register and land register were established. The cadastral record from the land cadastre of the old survey served as the official cadastral record for several decades. However, the destruction of this register during the hostilities of the Second World War left the area of Prusac without a cadastral register. To maintain a record of real estate ownership, an evidentiary land cadastre was instituted for the Prusac area, utilizing archival lithographic copies of cadastral plans from the old survey as graphical bases. In the 1970s, an aerial photogrammetric survey of the municipality of Donji Vakuf, including the area of the settlement of Prusac, was carried out. Utilizing this data, the cadastral register of the new survey was established. Following the establishment of the cadastral register of the new survey, the use of the cadastral register from the evidentiary cadastre of the settlement of Prusac was discontinued. Evidentiary cadastre was established partially on the basis of the data of the old survey. It has been repealed, resulting in the discontinuation of the evidentiary cadastral register's use as an official cadastral record. However, to maintain the land register, “cadastral plans” from the evidentiary land cadastre were occasionally employed. After the establishment of the cadastral register of the new survey, the cadastral plans from this survey became the official graphic base for maintaining the cadastral register. Prusac, a city with a history spanning through several centuries, held significant importance following the Ottoman Empire's occupation of Bosnia and Herzegovina. During the Ottoman rule, Prusac emerged as a prominent location, notably recognized for the religious and cultural event known as Ajvatovica. Various localities within Prusac and its environs hold associations with this event. The focus of this paper is to examine the depiction of the Ajvatovica locality on both analog cadastral plans from the old and new surveys. Additionally, the research delves into locating and presenting Prusac on small-scale cartographic representations dating back to the Ottoman Empire era.

Efficient and consistent string processing is critical in the exponentially growing genomic data era. Locally Consistent Parsing (LCP) addresses this need by partitioning an input genome string into short, exactly matching substrings (e.g.,"cores"), ensuring consistency across partitions. Labeling the cores of an input string consistently not only provides a compact representation of the input but also enables the reapplication of LCP to refine the cores over multiple iterations, providing a progressively longer and more informative set of substrings for downstream analyses. We present the first iterative implementation of LCP with Lcptools and demonstrate its effectiveness in identifying cores with minimal collisions. Experimental results show that the number of cores at the i^th iteration is O(n/c^i) for c ~ 2.34, while the average length and the average distance between consecutive cores are O(c^i). Compared to the popular sketching techniques, LCP produces significantly fewer cores, enabling a more compact representation and faster analyses. To demonstrate the advantages of LCP in genomic string processing in terms of computation and memory efficiency, we also introduce LCPan, an efficient variation graph constructor. We show that LCPan generates variation graphs>10x faster than vg, while using>13x less memory.

This paper explores both theoretical and practical dimensions of peacebuilding in post-conflict societies, with a specific focus on Bosnia and Herzegovina and the role of the High Representative, established by Annex 10 of the General Framework Agreement for Peace (Dayton Peace Agreement). By analysing the functioning of the Office of the High Representative (OHR), the study investigates the scope and limitations of the liberal institutionalist approach to peacebuilding, which dominated international intervention strategies in Bosnia and Herzegovina after 1995. The paper argues that classical institutional models failed to adequately respond to the fragmented political landscape and deep ethno-political divisions in Bosnia and Herzegovina, leading to a gradual transformation of the High Representative’s mandate from a coordinating body to a politically active institution with significant executive powers. The aim of this study is to examine the institutional evolution of the OHR and assess its contribution to state-building and peace preservation in Bosnia and Herzegovina, while also critically engaging with broader debates on the effectiveness of international peacebuilding in complex post-conflict environments. The research employs a qualitative methodology, including the analysis of primary documents (Annex 10, decisions of the OHR, legal acts), secondary sources (academic literature, international reports), and a case study approach based on a chronological review of the actions taken by High Representatives from 1995 to 2021. The analysis focuses on three core areas where the intervention of the High Representative had a decisive impact: (1) the imposition of binding decisions and laws in cases where domestic political actors failed to reach consensus; (2) judicial reforms and the annulment of rulings by domestic courts that were seen as contrary to the peace agreement; and (3) the dismissal of political officials deemed to be violating the Dayton Agreement or undermining the constitutional order. Findings indicate that this form of international tutelage contributed to short-term stabilization and institutional functionality but also raised concerns over democratic legitimacy and the limited ownership of domestic actors in the reform process. The theoretical framework links traditional liberal peacebuilding approaches with more recent critiques that emphasize the need for deeper local participation, long-term capacity-building, and context-sensitive governance. In this regard, the Bosnia and Herzegovina case illustrates the ambivalence of international interventions—balancing between ensuring stability and respecting sovereignty, and between delivering quick institutional fixes and fostering sustainable domestic governance. Scholarly and political critiques of the OHR’s role vary widely—from accusations of autocratic governance to acknowledgments of its necessity—thus exposing the ongoing tension between efficiency and legitimacy in international administration. The paper concludes that while the institution of the High Representative has played a critical role in maintaining peace and facilitating institutional development in Bosnia and Herzegovina, long-term sustainability requires a shift from external supervision to internal political consensus. The contribution of this research lies in its systematic analysis of the institutional trajectory of the OHR and its theoretical interpretation within broader debates on post-conflict international governance. The findings offer valuable insights for future peacebuilding missions in similarly complex environments.

Efficient and consistent string processing is critical in the exponentially growing genomic data era. Locally Consistent Parsing (LCP) addresses this need by partitioning an input genome string into short, exactly matching substrings (“cores”), ensuring consistency across partitions. Compared to the popular sketching techniques, LCP produces fewer cores, enabling a more compact representation and faster analyses. Here, we present the first iterative implementation of LCP with Lcptools and introduce LCPan, an efficient variation graph constructor, which we show generates variation graphs >12×\documentclass[12pt]{minimal} \usepackage{amsmath} \usepackage{wasysym} \usepackage{amsfonts} \usepackage{amssymb} \usepackage{amsbsy} \usepackage{mathrsfs} \usepackage{upgreek} \setlength{\oddsidemargin}{-69pt} \begin{document}$$\times$$\end{document} faster than vg, while using >13×\documentclass[12pt]{minimal} \usepackage{amsmath} \usepackage{wasysym} \usepackage{amsfonts} \usepackage{amssymb} \usepackage{amsbsy} \usepackage{mathrsfs} \usepackage{upgreek} \setlength{\oddsidemargin}{-69pt} \begin{document}$$\times$$\end{document} less memory.