bluuebunny/crossref_metadata_2025_split huggingface dataset:
Familial Mediterranean fever gene variations could trigger VPS16-associated early-onset dystonia and diabetes mellitus: clinical identification of a family with MEFV and VPS16 genetic variation association
Authors: Inalkac Gemici Yagmur , Ekici Cemal , Batum Melike , Akbostanci Cenk , Koc Ahmet , Mavioglu Hatice 
Published: July 2024

Abstract

Objectives
We describe the clinical pictures of an index case with dystonia and his family resulting from VPS16 and MEFV genetic variations based on previously published data and discuss the mechanisms that may have brought out the clinical findings.

Methods
A 17-year-old male had generalized dystonia that started at age 6 years, non-febrile abdominal pain attacks and was diagnosed with type 1 diabetes at age 14 years. Meanwhile, his 13-year-old sister had the same clinical presentation. His father was diabetic and his mother was asymptomatic. There was no consanguinity between the parents. Genetic variations were detected with whole exome sequencing.

Results
VPS16 c.1513C&gt;T/p.Arg505* (likely pathogenic), MEFV c.2080A&gt;G p.Met694val (pathogenic) and MEFV c.1772T&gt;C p.Ile591Thr (unknown significance) heterozygous variants were detected in his siblings. The father had VPS16 c.1513C&gt;T/p.Arg505* and MEFV c.2080A&gt;G p Met694val variations and the mother had MEFV c.1772T&gt;C p.Ile591Thr variations.

Conclusions
The occurrence of these diseases in siblings but their absence in the parents suggests the idea that the coexistence of two separate variations in the VPS16 and MEFV genes determines the phenotype. In addition, the increase in MEFV variation load in this family and the fact that DM occurs at an earlier age suggest that inflammation may cause an early diabetic clinical presentation.
Friendship and the Common Good in Julius Caesar
Authors: Cavanagh Dermot 
Published: July 2024

Abstract
This essay proposes a new reading of Julius Caesar’s account of friendship that also illuminates Shakespeare’s broader communitarian political attitudes. It argues that the ‘quarrel’ (or reconciliation) scene between Brutus and Cassius presents a crucial turn in the play’s closely meditated account of amity. This sheds new light on Shakespeare’s response to two classical sources: Cicero’s De Amicitia and Euripides’ Iphigenia at Aulius, whose opening provides the model for this sequence. The significance of how Brutus and Cassius settle their differences, as well as their refusal to believe the enemy is always occulted within the friend, has been under-estimated. The high-minded ideals of Ciceronian amicitia seem wildly remote from the play’s exposure of how friendship works in political life. We witness it being made serviceable to ambitions and held in bad faith: Cicero acknowledges these possibilities too. Yet the neglected later sequences of Julius Caesar tell a different story about friendship that emphasizes its role in promoting commonalty and reconciliation. The tragedy lies in the loss of this potential.
Metaphors and Telescopes: The Reception of Emanuele Tesauro’s Il Cannocchiale Aristotelico in Marvell’s Poetry
Authors: Holberton Edward 
Published: July 2024

Abstract
This article argues that Emanuele Tesauro’s treatise on wit, Il Cannocchiale Aristotelico (1654) influenced Andrew Marvell’s Restoration satire ‘The Last Instructions to a Painter’. It highlights a number of close correspondences between the engraved allegorical frontispiece of Tesauro’s treatise and Marvell’s final envoi ‘To the King’, which ironize the poem’s claim that it only satirizes the king’s courtiers. There are also affinities between Tesauro’s and Marvell’s development of imagery drawn from a Martial epigram at another pivotal moment in the ‘The Last Instructions to a Painter’, which reveal Marvell’s keen sense of the shifting associations between poetic style, religion and Restoration politics. Marvell found in Il Cannocchiale Aristotelico, the essay argues, a discussion of the acuity of metaphor which meshed excitingly with his own previously developed practice, especially his interest in reflexive and optical imagery. The article canvasses some of the ways in which Il Cannocchiale Aristotelico may have influenced Marvell’s later poetry, particularly in the use of ‘laconic’ wit, as he adapted to the poetic and political coteries of the Restoration. But it argues that ‘The Last Instructions to a Painter’’s notoriously baroque tribute to the Scottish Catholic soldier Archibald Douglas identifies Tesauro’s theory of wit with counter-reformation aesthetics, and uses parody to negotiate an uncomfortable recognition of how Marvell’s lyric poetry might be interpreted by Restoration readers.
Human Capital Portability and Careers in Finance
Authors: Gao Janet , Wang Wenyu , Wu Yufeng 
Published: July 2024

Abstract
How does firm-specific human capital shape careers in the finance industry? We build a dynamic model where workers accumulate portable and nonportable (firm-specific) human capital and learn about their match quality with employers. Estimating the model using granular data on M&A advisory bankers, we show that a large fraction of bankers’ human capital is nonportable, ranging from 12% to 46% across different firm types. Bankers make a dynamic trade-off between portability and returns on human capital, leading to time-varying job preferences over their life cycle. Our results have broad implications for careers in finance and the provision of financial services.
A Policy Analysis of the Temporary Protection Statute for Venezuelans in Colombia: Normalising Exceptionality using Humanitarian Discourses
Authors: Poveda-Clavijo Juliana , Mena Rodrigo 
Published: July 2024

Abstract
The Venezuelan crisis has led to over three million people crossing into Colombia. One strategy to address this situation is the Temporary Protection Statute (TPS), aimed at granting migrants legal status and responsibilities without providing them refugee status. Critics argue that portraying this solution as humanitarian might undermine its role as a protective measure. Additionally, there is a lack of understanding of the TPS as a political tool and the problems it aims to tackle. Using a Post-structural Policy Analysis (PPA), this article unpacks the TPS as a political device and the driving forces behind its development. Through text analysis and qualitative semi-structured interviews, the PPA reveals how the TPS in Colombia can be seen as a multifaceted legal and political tool bridging short-term humanitarian needs (primarily protection, healthcare, and labour market access) and long-term development practices (such as integration through ensuring economic, social, and environmental rights), within a complex political landscape hindering long-term solutions. Its legal text epitomises political drivers and discourses shaping migration governance and international protection. This study also shows PPA’s effectiveness in revealing these political factors and the policy-problem-making justifying the TPS as a solution, and offers case-based insights to enhance comparative migration studies.
Crucial points about international private foundations that founders sometimes wish they had paid more attention to
Authors: Niegel Johanna 
Published: July 2024

Abstract
In this 20th anniversary issue of ‘Private Foundations: A World Review’, the Editor under the heading ‘Crucial points about international private foundations that founders sometimes wish they had paid more attention to’ highlights and provides an assessment of the most important issues that founders of international foundations should take into consideration before taking action: Purposes—Legal Personality—Retention of Founders Rights and International Recognition. She concludes that a bespoke founder’s role is of essence, as soon as a foundation has international connections, as an influential role of the founder may well jeopardize the integrity of a foundation.
Utility of the WHO dengue guidelines in pediatric immunological studies
Authors: Sánchez Jorge L , Salgado Doris M , Vega Martha Rocío , Castro-Trujillo Sebastián , Narváez Carlos F 
Published: July 2024

Abstract
Dengue is a significant health problem due to the high burden of critical infections during outbreaks. In 1997, the World Health Organization (WHO) classified dengue as dengue fever (DF), dengue hemorrhagic fever (DHF), and dengue shock syndrome (DSS). It was revised in 2009 (updated in 2015), and the new guidelines recommended classifying patients as dengue without warning signs (DNS), dengue with warning signs (DWS), and severe dengue (SD). Although the utility of the revised 2009 classification for clinical studies is accepted, for immunological studies it needs to be clarified. We determined the usefulness of the 2009 classification for pediatric studies that analyze the circulating interleukin (IL)-6 and IL-8, two inflammatory cytokines. Plasma levels of IL-6 and IL-8 were evaluated in the acute and convalescent phases by flow cytometry in children with dengue classified using the 1997 and 2009 WHO guidelines. The plasma levels of IL-6 and IL-8 were elevated during the acute and decreased during convalescence, and both cytokines served as a good marker of acute dengue illness compared to convalescence. There were no differences in the plasma level of the evaluated cytokines among children with different clinical severity with any classification, except for the IL-8, which was higher in DWS than DNS. Based on the levels of IL-8, the 2009 classification identified DWS plus SD (hospital-treated children) compared to the DNS group [area under the curve (AUC): 0.7, p = 0.028]. These results support the utility of the revised 2009 (updated in 2015) classification in studies of immune markers in pediatric dengue.
Thalattosuchian crocodylomorphs from the Sinemurian (Early Jurassic) of the UK
Authors: Young Mark T , Dufeau David , Bowman Charlotte , Cowgill Thomas , Schwab Julia A , Witmer Lawrence M , Herrera Yanina , Katsamenis Orestis L , Steel Lorna , Rigby Martin , Brusatte Stephen L 
Published: July 2024

Abstract
Thalattosuchian crocodylomorphs were a ubiquitous component of shallow marine ecosystems during the Jurassic and Early Cretaceous. Alas, their origins remain a mystery. Here we describe three specimens from the Sinemurian (and possibly Early Pliensbachian) of the UK: a partial cranial rostrum, a series of cervical vertebrae, and two dorsal vertebrae adhered with matrix. These specimens are amongst the oldest known thalattosuchian fossils, with the partial cranial rostrum being the oldest known non-neothalattosuchian thalattosuchian. This partial cranial rostrum has a unique combination of rostral characters never seen before in any crocodylomorph, and helps to elucidate early thalattosuchian internal rostrum evolution, suggesting that the reduction in thalattosuchian paranasal sinuses was not related to either the reorganization of rostral neurovasculature seen in later diverging taxa or the increased cancellous bone microstructure. Based on our CT sample, a shift in cranial bone microstructure occurred in the Eoneustes + Metriorhynchidae subclade, one that coincided with the enlargement of the salt glands and decoupling of the external antorbital fenestra from the paranasal sinuses. Without extensive histological sampling we cannot determine whether the shift to an obligate aquatic lifestyle occurred prior to the evolution of Metriorhynchidae.
Solvability analysis for the Boussinesq model of heat transfer under the nonlinear Robin boundary condition for the temperature
Authors: Alekseev Gennady V. , Soboleva Olga V. 
Published: July 2024

We consider the new boundary value problem for the generalized Boussinesq model of heat transfer under the inhomogeneous Dirichlet boundary condition for the velocity and under mixed boundary conditions for the temperature. It is assumed that the viscosity, thermal conductivity and buoyancy force in the model equations, as well as the heat exchange boundary coefficient, depend on the temperature. The mathematical apparatus for studying the inhomogeneous boundary value problem under study based on the variational method is being developed. Using this apparatus, we prove the main theorem on the global existence of a weak solution of the mentioned boundary value problem and establish sufficient conditions for the problem data ensuring the local uniqueness of the weak solution that has the additional property of smoothness with respect to temperature.
This article is part of the theme issue ‘Non-smooth variational problems with applications in mechanics’.
On hyperelastic solid with thin rigid inclusion and crack subjected to global injectivity condition
Authors: Furtsev A. I. , Rudoy E. M. , Sazhenkov S. A. 
Published: July 2024

The paper investigates a problem concerning the equilibrium of a solid body containing a thin rigid inclusion and a crack. It is assumed that the body is hyperelastic, therefore, it is described within the framework of finite strain theory. One of the peculiarities of this problem is a global injectivity constraint, which prevents the body, the crack faces and the inclusion from both mutual and self penetration. First, the paper deals with the differential formulation of the problem. Next, we consider energy minimization, showing that the latter provides the weak formulation of the former. Finally, the existence of the weak solution is demonstrated through the use of the variational technique.
This article is part of the theme issue ‘Non-smooth variational problems with applications in mechanics’.
Exploring For Gloss: Active Exploration in Visual Material Perception
Authors: Lin Lisa P.Y. , Drewing Knut , Doerschner Katja 
Published: July 2024

AbstractImage motion contributes to the perception of visual material properties, and motion signals are generated during active exploration. However, little is known about how specific perceptual tasks influence the actions that generate these cues. In an experiment using virtual reality and real-time hand tracking, we investigated how the demands of perceptual tasks (e.g., judging gloss or lightness) shape exploratory behaviours. Participants either observed or actively explored objects varying in gloss and lightness while performing a matching task. We analysed how their exploration patterns varied based on the tasks. Using the same stimuli in both tasks, we found that participants explored objects more extensively when judging gloss than when judging lightness. These findings suggest a strategic prioritisation of relevant cues for gloss judgments, with participants using larger movements and object rotation to enhance viewing perspectives and highlight detection. Our findings show that exploration behaviours are task-dependent, with actions adapted to the demands of the perceptual task at hand.
Temperature-dependent polar lignification of a seed coat suberin layer promoting dormancy in Arabidopsis thaliana
Authors: Hyvärinen Lena , Fuchs Christelle , Utz-Pugin Anne , Gully Kay , Megies Christian , Holbein Julia , Iwasaki Mayumi , Demonsais Lara , Capitão Maria Beatriz , Barberon Marie , Franke Rochus , Nawrath Christiane , Loubéry Sylvain , Lopez-Molina Luis 
Published: July 2024

AbstractThe seed is a landmark plant adaptation where the embryo is sheltered by a protective seed coat to facilitate dispersion. In Arabidopsis, the seed coat, derived from ovular integuments, plays a critical role in maintaining dormancy, ensuring germination occurs during a favorable season. Dormancy is enhanced by cold temperatures during seed development by affecting seed coat permeability through changes in apoplastic barriers. However, the localization and composition of these apoplastic barriers are poorly understood. This study identifies and investigates a polar barrier in the seed coat’s outer integument (oi1) cells. We present histological, biochemical, and genetic evidence showing that cold promotes polar seed coat lignification of the outer integument 1 (oi1) cells and suberization throughout the entire oi1 cell boundary. The polar oi1 barrier is regulated by the transcription factors MYB107 and MYB9. MYB107, in particular, is crucial for the lignified polar oi1 barrier formation under cold temperatures. The absence of the oi1 barrier in mutant seeds correlates with increased permeability and reduced dormancy. Our findings elucidate how temperature-induced modifications in seed coat composition regulate dormancy, highlighting the roles of suberin and lignin in this process.Significance statementOur study uncovers how cold temperatures during seed development in the mother plant influence seed dormancy through apoplastic modifications in theArabidopsis thalianaseed coat. We identified a polar lignin barrier in the outer integument 1 (oi1) cells, which are also suberized. Lignification and suberization are regulated by transcription factors MYB107 and MYB9. Cold promotes lignification and suberization of oi1 cells through MYB107, thus creating a “memory” that reduces seed permeability and strengthens dormancy. Mutants defective in the oi1 barrier exhibit lower dormancy, highlighting the adaptive importance of this barrier. These findings advance our understanding of temperature-induced seed coat adaptations and their agricultural implications, particularly in the context of climate change, offering valuable insights for improving crop resilience and yield.
Predicting recoverability of collapsed food webs through perturbation and dimension reduction
Authors: Patnaik Swastik , Baruah Gaurav 
Published: July 2024

AbstractBiodiversity collapse, driven by escalating environmental changes, poses significant threats to ecosystem stability and the provision of essential ecosystem services. Understanding the recoverability of collapsed food webs thus is crucial for devising effective conservation strategies. This study delves into the theoretical exploration of the recoverability of food webs from a collapsed state. Through simple tools like dimension reduction, propagation of species-specific perturbation, and dynamical simulations, we explore whether simple tri-trophic food webs can be recovered from a collapsed state. Our study examines in detail the topological features of predator-prey food webs that could either facilitate or impede their recovery. We demonstrate that the recoverability of complex food webs can be predicted by using a simple dimension-reduced model, with certain structural factors that could constrain the full recovery of collapsed food webs. Furthermore, dynamic simulations also highlighted the significance of topological features such as connectance and the number of predator links in determining recoverability. Our dimension-reduced modeling framework offers insights into the feasibility of restoring entire complex predator-prey networks through species-specific interventions. This study contributes to a deeper understanding of ecosystem resilience and aids in the development of targeted conservation strategies.
Colony demographics shape nest construction in ants
Authors: Rajendran Harikrishnan , Weinberger Roi , Fonio Ehud , Feinerman Ofer 
Published: July 2024

AbstractThe ant nest serves as the skeleton of the ant superorganism. Similar to a skeleton, the nest expands as the colony grows and requires repair after catastrophic events. We experimentally compared nest excavation by colonies seeded from a single mated queen and then allowed to grow for six months, to excavation triggered by a catastrophic event in colonies with fixed demographics. The areas excavated by equal group sizes differed significantly between these conditions: heterogeneous populations in naturally growing colonies as well as cohorts of young ants dig larger areas than old ant cohorts. Moreover, we find that younger ants tend to dig slanted tunnels while older ants dig straight down. This is a novel form of age polyethism, where an ant’s age dictates not only her likelihood to engage in a task but also the way she performs the task. We further present a quantitative model that predicts that under normal growth, digging is predominantly performed by the younger ants while after a catastrophe all ants dig to restore lost nest volume. The fact that the nests of naturally growing colonies exhibit slanted tunnels strengthens this prediction. Finally, our results indicate how a colony’s demographic and physical history are sketched into the current structure of its nest.
Identification of a Monovalent Pseudo-Natural Product Degrader Class Supercharging Degradation of IDO1 by its native E3 KLHDC3
Authors: Hennes Elisabeth , Lucas Belén , Scholes Natalie S. , Cheng Xiu-Fen , Scott Daniel C. , Bischoff Matthias , Reich Katharina , Gasper Raphael , Lucas María , Xu Teng Teng , Pulvermacher Lisa-Marie , Dötsch Lara , Imrichova Hana , Brause Alexandra , Naredla Kesava Reddy , Sievers Sonja , Kumar Kamal , Janning Petra , Gersch Malte , Murray Peter J. , Schulman Brenda A. , Winter Georg E. , Ziegler Slava , Waldmann Herbert 
Published: July 2024

AbstractTargeted protein degradation (TPD) modulates protein function beyond inhibition of enzyme activity or protein-protein interactions. Most degrader drugs function by directly mediating proximity between a neosubstrate and hijacked E3 ligase. Here, we identified pseudo-natural products derived from (-)-myrtanol, termed iDegs that inhibit and induce degradation of the immunomodulatory enzyme indoleamine-2,3-dioxygenase 1 (IDO1) by a distinct mechanism. iDegs boost IDO1 ubiquitination and degradation by the cullin-RING E3 ligase CRL2KLHDC3, which we identified to natively mediate ubiquitin-mediated degradation of IDO1. Therefore, iDegs increase IDO1 turnover using the native proteolytic pathway. In contrast to clinically explored IDO1 inhibitors, iDegs reduce formation of kynurenine by both inhibition and induced degradation of the enzyme and, thus, would also modulate non-enzymatic functions of IDO1. This unique mechanism of action may open up new therapeutic opportunities for the treatment of cancer beyond classical inhibition of IDO1.
A dynamic redox switch turns TRC40 into a chaperone protecting human cells against ATP-depleting, oxidative stress
Authors: Dempsey Bianca , Dubrall Risai , Chan Olivia , Lapacz Kim Jasmin , Riemer Jan , Jakob Ursula , Ulrich Kathrin 
Published: July 2024

ABSTRACTOxidative stress represents a major challenge for cellular proteostasis. The accumulation of reactive oxygen species, such as hydrogen peroxide, impairs the fidelity of protein biosynthesis and causes non-specific oxidative protein modifications and aggregation. This situation is further aggravated by the oxidative stress-mediated drop in cellular ATP levels, which reduces the activity of ATP-dependent chaperones and proteases. We now demonstrate that to cope with oxidative unfolding stress, human cells rely on the moonlighting function of TRC40, which turns from an ATP-dependent targeting factor into an ATP-independent chaperone upon oxidation. Controlled by a highly conserved redox switch, oxidized TRC40 forms chaperone-active tetramers and high-molecular complexes which prevent the aggregation of unfolding proteins. Acute oxidative stress leads to the reversible formation of distinct TRC40 foci, associated with the canonical chaperones Hsp70 and Hsp110, suggesting a role of these stress-induced structures in recovering and sorting aberrant proteins. Consistently, we discovered that TRC40 is essential upon ATP-depleting, oxidative stress conditions to counteract the accumulation of mis- and unfolded proteins, which are rapidly cleared in an TRC40-dependent manner. Our data reveal that TRC40 is an integral part of protein quality control and support its role as a triaging factor in cellular proteostasis.