bluuebunny/crossref_metadata_2025_split huggingface dataset:
Defect in degradation of glycogenin‐exposed residual glycogen in lysosomes is the fundamental pathomechanism of Pompe disease
Authors: Zhang Na , Liu Fuchen , Zhao Yuying , Sun Xiaohan , Wen Bing , Lu Jian‐qiang , Yan Chuanzhu , Li Duoling 
Published: February 2024

AbstractPompe disease is a lysosomal storage disorder that preferentially affects muscles, and it is caused by GAA mutation coding acid alpha‐glucosidase in lysosome and glycophagy deficiency. While the initial pathology of Pompe disease is glycogen accumulation in lysosomes, the special role of the lysosomal pathway in glycogen degradation is not fully understood. Hence, we investigated the characteristics of accumulated glycogen and the mechanism underlying glycophagy disturbance in Pompe disease. Skeletal muscle specimens were obtained from the affected sites of patients and mouse models with Pompe disease. Histological analysis, immunoblot analysis, immunofluorescence assay, and lysosome isolation were utilized to analyze the characteristics of accumulated glycogen. Cell culture, lentiviral infection, and the CRISPR/Cas9 approach were utilized to investigate the regulation of glycophagy accumulation. We demonstrated residual glycogen, which was distinguishable from mature glycogen by exposed glycogenin and more α‐amylase resistance, accumulated in the skeletal muscle of Pompe disease. Lysosome isolation revealed glycogen‐free glycogenin in wild type mouse lysosomes and variously sized glycogenin in Gaa−/− mouse lysosomes. Our study identified that a defect in the degradation of glycogenin‐exposed residual glycogen in lysosomes was the fundamental pathological mechanism of Pompe disease. Meanwhile, glycogenin‐exposed residual glycogen was absent in other glycogen storage diseases caused by cytoplasmic glycogenolysis deficiencies. In vitro, the generation of residual glycogen resulted from cytoplasmic glycogenolysis. Notably, the inhibition of glycogen phosphorylase led to a reduction in glycogenin‐exposed residual glycogen and glycophagy accumulations in cellular models of Pompe disease. Therefore, the lysosomal hydrolysis pathway played a crucial role in the degradation of residual glycogen into glycogenin, which took place in tandem with cytoplasmic glycogenolysis. These findings may offer a novel substrate reduction therapeutic strategy for Pompe disease. © 2024 The Pathological Society of Great Britain and Ireland.
Advancing mechanical performance in sustainable engineering: Synergistic effects of graphene oxide/nano‐silica hybrid nanofiller via latex coagulation in natural rubber composites
Authors: Prajitha Velayudhan , Jibin Keloth Paduvilan , Abitha V. K. , Sisanth Krishnageham Sidharthan , Huskic Miroslav , Meera A. P. , George Jesiya Susan , Thomas Sabu 
Published: February 2024

AbstractThis research investigates the utilization of a hybrid nanofiller comprising graphene oxide (GO) and amine‐modified nano silica in natural rubber matrix via the latex stage coagulation method. The resulting novel composite materials were characterized comprehensively for their static and dynamic mechanical properties. The hybrid nanofiller exhibited excellent dispersion within the rubber latex, facilitating the formation of a well‐interconnected network structure. The formed network resulted in remarkable improvements in tensile strength, elongation at break, and modulus of the composites. Dynamic mechanical analysis revealed that the hybrid filler (GO/nano‐silica hybrid (GO/NS)) could effectively reinforce the rubber matrix by constraining the mobility of rubber chains, leading to increased stiffness and mechanical strength. In fact, the confinement effect of the nanofillers has been carefully evaluated from dynamic mechanical spectroscopy. The increased glass transition temperature of the rubber nanocomposites specifies the restricted segmental mobility of the rubber chains. Moreover, the hybrid composites displayed significantly low rolling resistance, particularly GO/NS 2 (31% reduction), indicating their potential application as tire treads for fuel‐efficient and green tire manufacturing. The mechanical and rolling resistance properties achieved in these composites can be attributed to the efficient dispersion of the GO/nano‐silica hybrid(GO/NS) nanofiller and the resulting network formation within the rubber matrix. Findings emphasized the composites' viability as sustainable and eco‐friendly tire materials, potential for enhanced fuel efficiency, and reduced environmental footprint in tire manufacturing technology.Highlights
The hybrid nanofiller (GO/nano silica hybrid) exhibited excellent dispersion within the rubber latex, leading to the formation of a well‐interconnected network structure.
The resulting network structure significantly improved the tensile strength, elongation at break, and modulus of the composite materials.
Dynamic mechanical analysis demonstrated that the hybrid filler effectively reinforced the rubber matrix, increasing stiffness and mechanical strength by constraining the mobility of rubber chains.
Our hybrid composites displayed a remarkable reduction in rolling resistance, particularly GO/NS2, with a 31% reduction, indicating their potential application as tire treads for fuel‐efficient and green tire manufacturing.
The study emphasized the composites viability as sustainable and eco‐friendly tire materials, offering potential benefits for enhanced fuel efficiency and reduced environmental footprint in tire manufacturing technology.
3D printing polypropylene composites reinforced with functionalized halloysite: Balance between stiffness and impact resistance
Authors: Patiño‐Almanza Rodrigo , García‐Méndez Rey Fernando , Rivera‐Armenta José Luis , Strachota Adam , Almendarez‐Camarillo Armando 
Published: February 2024

AbstractIn this work, a new nano‐reinforced polypropylene filament was produced and fabricated by 3D printing to create a nanocomposite with a good balance between stiffness and impact resistance properties. Nanocomposite filament with 0.5, 1.0, and 1.5 wt% of functionalized halloysite nanotubes was prepared. The chemical modification was carried out to obtain a reinforcement that could act as an improved β‐nucleating agent and characterized by infrared spectroscopy. The spectrum showed the appearance of two new bands at 1571 and 1410 cm−1, which could indicate an interaction between the pimelic acid molecules and the oxygenated surface of the halloysite nanotubes. Energy dispersive X‐ray spectroscopy analysis confirmed a good dispersion of the modified reinforcement along the surface of the nanocomposite filament. Wide‐angle X‐ray scattering and differential scanning calorimetry analyses showed the improvement of the β‐nucleating ability of the halloysite nanotubes through new chemically functionalized, obtaining percentages of β‐crystal of 80% for the nanocomposite reinforced with 0.5 wt%. The dynamic mechanical analysis showed that the 3D‐printed functionalized nanocomposites presented higher storage and loss modulus (an increase of 148% and 122%, respectively). Finally, the impact strength properties increased by 90%, 108%, and 21% for the functionalized nanocomposites with 0.5, 1.0, and 1.5 wt%, respectively.Highlights
Chemical functionalization of a natural reinforcement as a ‐nucleating agent.
3D printing polypropylene nanocomposites with enhanced properties.
Good balance between impact and stiffness properties of nanocomposites.
Reduction of mesoporous poly(ether ether ketone) materials, preserving pore size
Authors: Weintraub Sagiv , Gellerman Gary , Bazylevich Andrii , Bikson Ben 
Published: February 2024

AbstractPoly(ether ether ketone) (PEEK) is an important high‐performance engineering polymer that is used extensively in numerous engineering applications that require chemical, solvent and thermal resistance. These attributes make PEEK attractive for the preparation of porous materials and membranes. Chemical modifications of preformed porous PEEK materials can expand the utilization of PEEK in a range of applications. It is desirable to carry out chemical functionalization without affecting the preformed porous structure or the degree of polymer crystallinity responsible for the sought‐after properties. We report here on the heterogeneous functionalization of preformed mesoporous PEEK materials with hydroxyl groups using sodium borohydride as a reducing agent in a poly(ethylene glycol)/tetrahydrofuran solvent mixture. The pore structure of functionalized materials was characterized, and the degree of modification with functional groups was measured as a function of reaction protocol. The methodology was applied to the preparation of porous PEEK materials in the form of beads and hollow fibers that differed in semicrystalline morphology and pore structure. The degree of surface modification by –OH groups in reduced PEEK (PEEK‐OH) porous materials was determined quantitatively by measuring the carbocation adduct concentration formed upon the dissolution of PEEK‐OH in sulfuric acid using UV–visible spectroscopy. Bis(4‐(4‐methoxyphenoxy)phenyl)methanone was used to optimize the reaction condition protocol. The degree of crystallinity and pore structure of the functionalized articles were largely preserved following modification. © 2024 Society of Industrial Chemistry.
Population pharmacokinetic modeling of oral brepocitinib in healthy volunteers and patients with immuno‐inflammatory diseases
Authors: Maleki Farzaneh , Clark Elias , Banfield Christopher , Byon Wonkyung , Nicholas Timothy 
Published: February 2024

AbstractThe objective of this population pharmacokinetic (PK) analysis was to characterize the concentration–time profile of brepocitinib plasma concentration after single‐ and multiple‐oral administration in healthy volunteers (HVs) and patients with immuno‐inflammatory diseases. Blood samples from phase I HV and phase II clinical studies of patients with alopecia areata, psoriasis, psoriatic arthritis, ulcerative colitis (UC), vitiligo, and hidradenitis suppurativa were analyzed using a nonlinear mixed‐effects modeling approach. Effects of patients' characteristics on brepocitinib exposure were investigated. Overall, 8552 brepocitinib plasma concentrations from 775 individuals were included in the analysis. The PKs of brepocitinib were adequately described by a two‐compartment model with first‐order absorption and a lag time for tablet formulation, dose‐dependent bioavailability, and Box–Cox transformed interindividual variabilities on apparent clearance (CL/F) and apparent central volume of distribution (Vc/F). For a typical 70‐kg non‐Asian female patient with baseline aspartate aminotransferase of 22 unit/liter, CL/F and Vc/F estimates were 17.5 L/h and 88.5 L, respectively. Asians had a higher exposure (independent of body weight), caused by a 10% lower CL/F when compared to other individuals. Independent of baseline body weight, the male population showed 13% higher Vc/F compared to the female population. Patients with UC were predicted to have 46% slower absorption rate compared to other individuals. The PKs of brepocitinib were well‐characterized by a two‐compartment model with first‐order absorption and dose‐dependent bioavailability. Several covariates, such as race and sex, were identified to have statistically significant, but not clinically meaningful, effects on the estimated PK parameters.
Stability Enhancement in All‐Inorganic Perovskite Light Emitting Diodes via Dual Encapsulation
Authors: Shi Jindou , Wang Zeyu , Gaponenko Nikolai V. , Da Zheyuan , Zhang Chen , Wang Junnan , Ji Yongqiang , Ding Yusong , Yao Qing , Xu Youlong , Wang Minqiang 
Published: February 2024

AbstractAddressing the challenge of lighting stability in perovskite white light emitting diodes (WLEDs) is crucial for their commercial viability. CsPbX3 (X = Cl, Br, I, or mixed) nanocrystals (NCs) are promising for next‐generation lighting due to their superior optical and electronic properties. However, the inherent soft material structure of CsPbX3 NCs is particularly susceptible to the elevated temperatures associated with prolonged WLED operation. Additionally, these NCs face stability challenges in high humidity environments, leading to reduced lighting performance. This study introduces a two‐step dual encapsulation method, resulting in CsPbBr3@SiO2/Al2SiO5 composite fibers (CFs) with enhanced optical stability under extreme conditions. In testing, WLEDs incorporating these CFs, even under prolonged operation at high power (100 mA for 9 h), maintain consistent electroluminescence (EL) intensity and optoelectronic parameters, with surface temperatures reaching 84.2 °C. Crucially, when subjected to 85 °C and 85% relative humidity for 200 h, the WLEDs preserve 97% of their initial fluorescence efficiency. These findings underscore the efficacy of the dual encapsulation strategy in significantly improving perovskite material stability, marking a significant step toward their commercial application in optoelectronic lighting.
Intestinal Colonized Silkworm Chrysalis‐Like Probiotic Composites for Multi‐Crossed Comprehensive Synergistic Therapy of Inflammatory Bowel Disease
Authors: Wang Ya , Zhang Yujie , Wang Pengqian , Han Jing , Zhang Xiaojiang , Shi Feiyu , Zhang Zhe , Guo Gang , Wang Ruochen , Shao Dan , Wu Daocheng , She Junjun 
Published: February 2024

AbstractInspired by the timely emergence of silkworm pupae from their cocoons, silkworm chrysalis‐like probiotic composites (SCPCs) are developed for the comprehensive therapy of inflammatory bowel disease (IBD), in which probiotics are enveloped as the “pupa” in a sequential layering of silk sericin (SS), tannic acid (TA), and polydopamine, akin to the protective “cocoon”. Compared to unwrapped probiotics, these composites not only demonstrate exceptional resistance to the harsh gastrointestinal environment and exhibit over 200 times greater intestinal colonization but also safeguard probiotics from the damage of IBD environment while enabling probiotics sustained release. The probiotics, in synergy with SS and TA, provide a multi‐crossed comprehensive therapy for IBD that simultaneously addresses various pathological features of IBD, including intestinal barrier disruption, elevated pro‐inflammatory cytokines, heightened oxidative stress, and disturbances in the intestinal microbiota. SCPCs exhibit remarkable outcomes, including a 9.7‐fold reduction in intestinal permeability, an 8.9‐fold decrease in IL‐6 levels, and a 2.9‐fold reduction in TNF‐α levels compared to uncoated probiotics. Furthermore, SCPCs demonstrate an impressive 92.25% reactive oxygen species clearance rate, significantly enhance the richness of beneficial intestinal probiotics, and effectively diminish the abundance of pathogenic bacteria, indicating a substantial improvement in the overall therapeutic effect of IBD.
Discovering T-dualities of little string theories
Authors: Bhardwaj Lakshya 
Published: February 2024

Abstract
We describe a general method for deducing T-dualities of little string theories, which are dualities between these theories that arise when they are compactified on circle. The method works for both untwisted and twisted circle compactifications of little string theories and is based on surface geometries associated to these circle compactifications. The surface geometries describe information about Calabi-Yau threefolds on which M-theory can be compactified to construct the corresponding circle compactified little string theories. Using this method, we deduce at least one T-dual, and in some cases multiple T-duals, for untwisted and twisted circle compactifications of most of the little string theories that can be described on their tensor branches in terms of a 6d supersymmetric gauge theory with a simple non-abelian gauge group, which are also known as rank-0 little string theories. This includes little string theories carrying $$ \mathcal{N} $$
N
= (1, 1) and $$ \mathcal{N} $$
N
= (1, 0) supersymmetries. For many, but not all, circle compactifications of $$ \mathcal{N} $$
N
= (1, 1) little string theories, we have T-dualities that realize Langlands dualities between affine Lie algebras. Along the way, we find another discrete theta angle distinct from 0 and π for an E-string node.
Sozialpädiatrische Versorgungssituation und -bedarfe in Zeiten der COVID-19-Pandemie 2020 bis 2022. Was wird jetzt gebraucht?
Authors: Peters Elke , Schmidt Hannah , Baltus Hannah , Schnoor Maike , Hartmann Nina , Katalinic Alexander 
Published: February 2024

Zusammenfassung
Hintergrund
Kinder und Jugendliche mit sozialpädiatrischen Versorgungsbedarfen stellen eine Gruppe mit besonderen Herausforderungen dar. Ziel der qualitativen Studie war, die sozialpädiatrische Versorgung in der Pandemie aus Expert*innensicht zu beschreiben. Hieraus sollten Rückschlüsse für möglicherweise entstandene sozialpädiatrische Versorgungsbedarfe abgeleitet werden.

Methoden
Es wurden 25 Expert*innen aus dem sozialpädiatrischen Bereich von Mai bis November 2022 mit leitfadengestützten Interviews zu folgenden Themen befragt: Abweichungen der Versorgung, Inanspruchnahmeverhalten von Familien, individuelle Belastungen und Ressourcen sowie nachhaltige Bedarfe. Die Interviews wurden von 2 wissenschaftlichen Mitarbeitenden inhaltsanalytisch ausgewertet.

Ergebnisse
Temporär zeigte sich ein deutlich eingeschränktes sozialpädiatrisches Versorgungsangebot. Während bereits vor der Pandemie gut eingebundene Familien ausreichend mithilfe von Telefon‑/Videokontakten betreut werden konnten, wurde eine Dunkelziffer von Risikogruppen z. B. mit geringer Handlungskompetenz beschrieben, welche Leistungen nicht oder verzögert in Anspruch nahmen. Beobachtet wurden Versorgungsbedarfe für neu entwickelte psychische Auffälligkeiten und Therapierückschritte aufgrund eingeschränkter Fördermöglichkeiten sowie ein Nachholbedarf verpasster Möglichkeiten der Frühförderung bei Entwicklungsstörungen.

Diskussion
Um entstandene Versorgungsbedarfe zu decken, sollten zielgerichtet unterversorgte Familien identifiziert und zeitnah unter Berücksichtigung individueller Merkmale versorgt werden. Hierzu könnten z. B. vermehrt aufsuchende Versorgungsangebote etabliert werden, die unbürokratisch bei betroffenen Familien ankommen.
Thermo-physical Characterisation of Plasters Containing Phase Change Materials (PCMs)
Authors: Baccega Eleonora 
Published: February 2024

AbstractThe integration of phase change materials (PCMs) within building materials is an interesting strategy to improve the thermal performance of buildings, thus reducing the energy demand for heating and/or cooling. To do so, the thermo-physical characterisation of the new enhanced materials is of outmost importance which, however, is difficult to carry out due to several limitations related to the most used techniques. To overcome these, a new alternative set up was realized, which allowed the thermo-physical characterization of different plaster samples enhanced with granular organic PCM. A steady-state test was conducted maintaining constant thermal gradients through which the thermal conductivity of the materials used was estimated. Then, a two-step ramp unsteady-state test was conducted through which the specific heat and the latent heat were estimated, showing a good agreement with values provided by the PCM suppliers. The estimated properties were then validated against experimental data acquired during the monitoring activity under real outdoor conditions of different wall samples on which the PCM-enhanced plasters were applied. With the estimated properties, RMSE values were lower than 1 °C for temperatures and lower than 2.50 W·m−2 for heat fluxes.
Should Decisions in QCDCL Follow Prefix Order?
Authors: Böhm Benjamin , Peitl Tomáš , Beyersdorff Olaf 
Published: February 2024

AbstractQuantified conflict-driven clause learning (QCDCL) is one of the main solving approaches for quantified Boolean formulas (QBF). One of the differences between QCDCL and propositional CDCL is that QCDCL typically follows the prefix order of the QBF for making decisions. We investigate an alternative model for QCDCL solving where decisions can be made in arbitrary order. The resulting system$$\textsf{QCDCL}^\textsf {{A\tiny {\MakeUppercase {ny}}}}$$QCDCLANYis still sound and terminating, but does not necessarily allow to always learn asserting clauses or cubes. To address this potential drawback, we additionally introduce two subsystems that guarantee to always learn asserting clauses ($$\textsf{QCDCL}^\textsf {{U\tiny {\MakeUppercase {ni}}-A\tiny {\MakeUppercase {ny}}}}$$QCDCLUNI-ANY) and asserting cubes ($$\textsf{QCDCL}^\textsf {{E\tiny {\MakeUppercase {xi}}-A\tiny {\MakeUppercase {ny}}}}$$QCDCLEXI-ANY), respectively. We model all four approaches by formal proof systems and show that$$\textsf{QCDCL}^\textsf {{U\tiny {\MakeUppercase {ni}}-A\tiny {\MakeUppercase {ny}}}}$$QCDCLUNI-ANYis exponentially better than$$\mathsf{{QCDCL}} $$QCDCLon false formulas, whereas$$\textsf{QCDCL}^\textsf {{E\tiny {\MakeUppercase {xi}}-A\tiny {\MakeUppercase {ny}}}}$$QCDCLEXI-ANYis exponentially better than$$\mathsf{{QCDCL}} $$QCDCLon true QBFs. Technically, this involves constructing specific QBF families and showing lower and upper bounds in the respective proof systems. We complement our theoretical study with some initial experiments that confirm our theoretical findings.
Differences in Antenatal Care Policies in England, Finland, and the Netherlands: A Framing analysis
Authors: Wierenga Hanna , Målqvist Mats 
Published: February 2024

Abstract
Background and Purpose
The World Health Organization (WHO) states that good quality antenatal care should strive for both mother and child achieving their best possible health. On a policy level, in Europe these goals are reached with varying approaches. This research offers a fresh look on the underlying assumptions embedded in the ANC policies in three European countries.

Methods
A framing analysis was conducted to publicly available ANC policies on uncomplicated pregnancies in Finland, England, and the Netherlands. Analysis was guided by van Hulst and Yanowa and included the following phases: a) Sense-making, b) Selecting, naming, and categorizing and c) Storytelling.

Main Findings
Findings of this study demonstrate how ANC is organized with distinct frames. The Finnish ANC policies emphasized equity in care and instead of focusing on women, the ANC focused on the family. In England the pregnant woman was central, and it is seen as her responsibility to understand the ANC protocols. The ANC in the Netherlands focused on the pregnant woman’s pregnancy experience and freedom.

Conclusion
The three studied countries had individual priorities and values guiding ANC provision. Despite each country being in line with the WHO ANC recommendations, areas requiring improvement should not be overlooked.