bluuebunny/crossref_metadata_2025_split huggingface dataset:
Structure and properties of La0.6Ce0.3Pr0.1B6‐ZrB2, a directionally solidified eutectic with different growth rates
Authors: Shi Feng , Liu Danmin , Wang Yan , Liu Linlin , Xiao Weiqiang , Liu Cuixiu , Yang Xinyu , Zhang Jiuxing 
Published: July 2021

AbstractLa0.6Ce0.3Pr0.1B6‐ZrB2, a directionally solidified eutectic (DSE), is a material with a high‐performance thermionic emission with great application prospects. The influence of the growth rates of this DSE on the microstructure, growth direction, two phases orientation relationship, mechanical properties, and thermionic emission properties was investigated. The experimental results show that the microstructure and growth direction of the two phases varied with the growth rate of the eutectic: the faster the growth rate, the lower the uniformity of the ZrB2 phase became, and the growth directions of the La0.6Ce0.3Pr0.1B6 matrix and the ZrB2 phase were also altered accordingly. In the La0.6Ce0.3Pr0.1B6‐ZrB2 DSE with a growth rate of 100 mm/h, the two phases have a unique growth direction and a crystallographic orientation relationship. The fracture toughness of the La0.6Ce0.3Pr0.1B6‐ZrB2 DSE is at least 50% higher than that of the single crystal La0.6Ce0.3Pr0.1B6, and the fracture toughness of the DSEs formed at different growth rates also differed.
Fermi level pinning in Co‐doped BaTiO3: Part I. DC and AC electrical conductivities and degradation behavior
Authors: Ryu Gyung Hyun , Bowes Preston C. , McGarrahan John R. , Irving Douglas L. , Dickey Elizabeth C. 
Published: July 2021

AbstractWe explore the synergistic effects of co‐doping BaTiO3 with a judicious combination of acceptors and donors to control the point defect chemistry and electrical properties, with the goal of simultaneously limiting the electronic and ionic conductivities over broad temperature and oxygen partial pressure (pO2) ranges. Specifically, we compare the temperature‐ and pO2‐dependent electrical properties of BaTiO3 ceramics acceptor‐doped with either Mn or Mg and co‐doped with a Y donor. This study, which is the first of a two‐part series, presents the electrical properties as a function of pO2, temperature, and time, focusing on the grain‐interior electrical response. The DC and AC electrical conductivity measurements reveal that co‐doping with Mn and Y can result in (1) increased electrical resistivity over a broad temperature range, (2) pO2‐independent electrical conductivity in oxidizing conditions, and (3) improved time‐dependent dielectric degradation resistance. These behaviors are attributed to a Fermi level pinning effect, as is explained in the companion paper, which presents complementary density functional theory (DFT)‐based grand‐canonical defect chemistry models. The collective experimental and computational studies demonstrate that the pO2‐independent electrical conductivity in the Mn and Y co‐doped BaTiO3 is attributed to a Fermi level pinning mechanism arising from the multivalent Mn dopant, and the background reservoir of positive charge provided by the predominant substitution of Y on the Ba sites. The enhanced degradation resistance is attributed to a reduced oxygen vacancy concentration relative to the other doping chemistries.
Deaths of people with intellectual disabilities: Analysis of deaths in England fromCOVID‐19 and other causes
Authors: Heslop Pauline , Byrne Victoria , Calkin Rachel , Huxor Avon , Sadoo Annie , Sullivan Brian 
Published: July 2021

AbstractBackgroundPeople with intellectual disabilities experience significant health inequities. The aim of this report is to understand the circumstances leading to death from COVID‐19 in people with intellectual disabilities.MethodLocal areas in England prioritised reviewing 200 deaths of adults with intellectual disabilities. Of these, approximately 80% were required to be deaths from suspected or confirmed COVID‐19 as this was the focus of the study; the remainder from other causes. All deaths occurred between 2 March2020 and 9 June 2020.ResultsPeople with intellectual disabilities differed from the general population in their symptoms of COVID‐19 and age at death. The overall quality of care was rated similar to other deaths of people with intellectual disabilities. Concerns were raised relating to recognising acute deterioration and do not attempt cardio‐pulmonary resuscitation decisions.ConclusionsService improvements are indicated in the ways in which people with intellectual disabilities encounter COVID‐19 and experience the disease.
Efficacy and safety of mogamulizumab by patient baseline blood tumour burden: a post hoc analysis of the MAVORIC trial
Authors: Cowan R.A. , Scarisbrick J.J. , Zinzani P.L. , Nicolay J.P. , Sokol L. , Pinter‐Brown L. , Quaglino P. , Iversen L. , Dummer R. , Musiek A. , Foss F. , Ito T. , Rosen J‐P. , Medley M.C. 
Published: July 2021

AbstractBackgroundMogamulizumab was compared with vorinostat in the phase 3 MAVORIC trial (NCT01728805) in 372 patients with relapsed/refractory mycosis fungoides (MF) or Sézary syndrome (SS) who had failed ≥1 prior systemic therapy. Mogamulizumab significantly prolonged progression‐free survival (PFS), with a superior objective response rate (ORR) vs. vorinostat.ObjectivesThis post hoc analysis was performed to evaluate the effect of baseline blood tumour burden on patient response to mogamulizumab.MethodsPFS, ORR, time to next treatment (TTNT), skin response (modified Severity‐Weighted Assessment Tool [mSWAT]) and safety were assessed in patients stratified by blood classification (B0 [n = 126], B1 [n = 62], or B2 [n = 184], indicating increasing blood involvement).ResultsInvestigator‐assessed PFS was longer for mogamulizumab versus vorinostat across all blood classes, significantly so for B1 and B2 patients. ORR was higher with mogamulizumab than with vorinostat in all blood classification groups and more markedly so with escalating B class (B0: 15.6% vs. 6.5%, P = 0.0549; B1: 25.8% vs. 6.5%, P = 0.2758; B2: 37.4% vs. 3.2%, P < 0.0001). TTNT was significantly longer for patients treated with mogamulizumab versus vorinostat with B1 (12.63 vs. 3.07 months; HR 0.32 [95% CI 0.16–0.67]; P = 0.0018) and B2 (13.07 vs. 3.53 months; HR 0.30 [95% CI 0.21–0.43]; P < 0.0001) blood involvement. In the mogamulizumab arm, 81 patients (43.5%) had ≥50% change in the mSWAT vs. 41 patients (22.0%) with vorinostat; mSWAT improvements with mogamulizumab occurred most often in B1 and B2 patients. Rapid, sustained reductions were seen in CD4+CD26‐ cell counts and CD4:CD8 ratios in mogamulizumab patients for all B classes. Treatment‐emergent adverse events were less frequent overall with mogamulizumab and similar in frequency regardless of B class.ConclusionsThis post hoc analysis indicates greater clinical benefit with mogamulizumab vs. vorinostat in patients with MF and SS classified as having B1 and B2 blood involvement.
High rate of self‐improving phenotypes in children with non‐syndromic congenital ichthyosis: case series from south‐western Germany
Authors: Frommherz L. , Krause A. , Kopp J. , Hotz A. , Hübner S. , Reimer‐Taschenbrecker A. , Casetti F. , Zirn B. , Fischer J. , Has C. 
Published: July 2021

AbstractBackgroundNon‐syndromic congenital ichthyosis describes a heterogeneous group of hereditary skin disorders associated with erythroderma and scaling at birth. Although both severe and mild courses are known, the prediction of the natural history in clinical practice may be challenging.ObjectivesTo determine clinical course and genotype‐phenotype correlations in children affected by non‐syndromic congenital ichthyosis in a case series from south‐western Germany.MethodsWe performed a retrospective observational study of 32 children affected by non‐syndromic congenital ichthyoses seen in our genodermatosis clinic between 2011 and 2020. Follow‐ups included assessment of weight and severity of skin involvement utilizing a modified Ichthyosis Area Severity Index (mIASI). mIASI was calculated as a sum comprising the previously published IASI score and an additional novel score to evaluate palmoplantar involvement. Linear regression was assessed using Pearson correlation, and statistical analysis was performed using the Wilcoxon–Mann–Whitney test.ResultsThis study included 23 patients with autosomal recessive congenital ichthyosis, seven with keratinopathic ichthyosis and two with harlequin ichthyosis. Cutaneous manifestations improved in more than 70% of the children during the follow‐up. Especially in patients with mutations in ALOXE3 and ALOX12B, mIASI scores dropped significantly. The most common phenotype observed in this study was designated ′mild fine scaling ichthyosis′. Severe palmoplantar involvement occurred in patients with KRT1 and ABCA12 mutations; most patients demonstrated hyperlinearity as a sign of dryness and scaling. Weight was mainly in the normal range and negatively correlated with the severity of skin involvement.ConclusionsCongenital ichthyosis that self‐improves and evolves with mild fine scaling ichthyosis was the most common phenotype observed in our patients. This type might be underdiagnosed if the genetic diagnosis is not performed in the first year of life. mIASI is an easy and fast instrument for scoring disease severity and adding additional points for palmoplantar involvement might be valuable.
Multi‐stage metamorphic history of the Oki gneisses in Japan: Implications for Paleoproterozoic metamorphism and tectonic correlations in northeastern Asia
Authors: Kawabata Ryoichi , Imayama Takeshi , Kato Takenori , Oh Chang‐Whan , Horie Kenji , Takehara Mami 
Published: July 2021

AbstractMetamorphicP–Tconditions, monazite chemical ages and zircon U–Pb ages from the gneisses exposed in the Oki belt, Japan, were integrated to unravel the multi‐stage metamorphic history of the belt. Microstructural observations combined with obtainedP–Tconditions and metamorphic ages reveal three distinct stages of metamorphism: M1, M2 and M3. The M1 stage occurredc. 1.85 Ga with high‐Tgranulite‐facies metamorphism (793–803°C and 9.8–12.3 kbar and 738–755°C and 9.1–12.0 kbar in the southwestern and southeastern Oki gneisses, respectively). The age of the M1 stage is well recorded in monazites included in large garnet porphyroblasts and low Th/U metamorphic rims in zircons from the Oki gneisses. The M1 metamorphism was overprinted byc. 230 Ma metamorphism (M2), which occurred at granulite‐facies conditions (817–829°C and 9.0–10.3 kbar) in the southwestern Oki gneisses and at upper amphibolite‐facies conditions (693°C and 5.3 kbar) in the southeastern Oki gneisses. Monazites in small garnets, euhedral zircons and outermost rims of zircons crystallized during this stage. The final metamorphism occurred as retrograde amphibolite‐facies recrystallization (M3) at conditions of 558–638°C and 3.7–4.8 kbar. The inherited cores in zircons yield ages from Paleoarchean to Paleoproterozoic but lack Palaeozoic ages. The detrital zircon distribution and the Paleoproterozoic metamorphic event in the Oki belt support the idea that the Oki gneisses are fragments of a Precambrian terrane rather than Palaeozoic sediments derived from the terrane. Combined with previous studies, we concluded that thec. 1.85 Ga M1 high‐Tgranulite‐facies metamorphism in the Oki belt could be related to that of the Jiao‐Liao‐Ji belt in the eastern North China block via the northern Gyeonggi and Nangrim Massifs on the Korean Peninsula, whereas thec. 250–230 Ma M2 stage could be associated with collision between the North and South China blocks. The Oki belt geologically corresponds to the northern Gyeonggi Massif in South Korea due to their similar Paleoproterozoic and Triassic tectonothermal events.
Basal forebrain cholinergic system in the dementias: Vulnerability, resilience, and resistance
Authors: Geula Changiz , Dunlop Sara R. , Ayala Ivan , Kawles Allegra S. , Flanagan Margaret E. , Gefen Tamar , Mesulam Marek‐Marsel 
Published: July 2021

AbstractThe basal forebrain cholinergic neurons (BFCN) provide the primary source of cholinergic innervation of the human cerebral cortex. They are involved in the cognitive processes of learning, memory, and attention. These neurons are differentially vulnerable in various neuropathologic entities that cause dementia. This review summarizes the relevance to BFCN of neuropathologic markers associated with dementias, including the plaques and tangles of Alzheimer's disease (AD), the Lewy bodies of diffuse Lewy body disease, the tauopathy of frontotemporal lobar degeneration (FTLD‐TAU) and the TDP‐43 proteinopathy of FTLD‐TDP. Each of these proteinopathies has a different relationship to BFCN and their corticofugal axons. Available evidence points to early and substantial degeneration of the BFCN in AD and diffuse Lewy body disease. In AD, the major neurodegenerative correlate is accumulation of phosphotau in neurofibrillary tangles. However, these neurons are less vulnerable to the tauopathy of FTLD. An intriguing finding is that the intracellular tau of AD causes destruction of the BFCN, whereas that of FTLD does not. This observation has profound implications for exploring the impact of different species of tauopathy on neuronal survival. The proteinopathy of FTLD‐TDP shows virtually no abnormal inclusions within the BFCN. Thus, the BFCN are highly vulnerable to the neurodegenerative effects of tauopathy in AD, resilient to the neurodegenerative effect of tauopathy in FTLD and apparently resistant to the emergence of proteinopathy in FTLD‐TDP and perhaps also in Pick's disease. Investigations are beginning to shed light on the potential mechanisms of this differential vulnerability and their implications for therapeutic intervention.
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Need of support perceived by patients primarily curatively treated for breast, colorectal, or prostate cancer and close to discharge from hospital—A qualitative study
Authors: Hajdarevic Senada , Fallbjörk Ulrika , Fransson Per , Åström Sture 
Published: July 2021

AbstractAimTo describe perceived needs of support among patients close to discharge from the hospital and at the end of primary curative radiotherapy for breast, colorectal or prostate cancer.BackgroundFew studies have specifically explored patients’ early support needs when ending primary curative treatment.DesignQualitative interview study design.MethodsA purposive sample of 27 participants with breast, colorectal or prostate cancer aged 33–88 years. The interviews were analysed by qualitative content analysis. Reporting followed the COREQ guidelines.ResultsPersonal support to reach a sense of control and Social support for personal growth were two main themes, highlighting that people required adapted support from health care since needs of support could change over time. This support from health care was also relying on that trust‐based relationships were developed. Through mutuality with others and engagement in meaningful activities people became enabled and felt further supported. Personal support from health care seems specifically important for the patients’ feelings of control and could be a facilitator for patients to identify further support for personal growth in how to manage, on the one hand, illness and insecurity, and on the other, their well‐being and everyday life with cancer.ConclusionTo empower patients who are ending primary treatment and being close to discharge from hospital, healthcare professionals should recognise patients’ shifting needs and adapt the support. Adapted support is significant for patients’ sense of safety. Biomedical information is not sufficient to fully support patients.Relevance to clinical practiceOffering easy access to supportive care when primary treatment is finished could diminish people's stress, insecurity and avoidable use of healthcare services. Even after discharge, nurses preferably should adapt and offer support tailored to patients’ needs. Such support may improve patients’ sense of control and safety, trust in health care, feelings of community and encourage personal growth.
Organizational Demographic Faultlines: Their Impact on Collective Organizational Identification, Firm Performance, and Firm Innovation
Authors: Leicht‐Deobald Ulrich , Huettermann Hendrik , Bruch Heike , Lawrence Barbara S. 
Published: July 2021

AbstractIn this study, we seek to understand the consequences of demographic faultlines at the organizational level. Drawing from the faultline and cross‐categorization literature, we suggest that organizational demographic faultlines (based on age and gender) have the potential to either reduce or enhance employees’ collective organizational identification and, thereby, indirectly influence firm performance and innovation. Whether organizational demographic faultlines have detrimental or beneficial effects depends on the functional heterogeneity within faultline‐based demographic subgroups, where heterogeneity is defined as the extent to which subgroup members belong to different functional departments. We theorize that this functional heterogeneity alters the degree of social integration between demographic subgroups. Results from a multisource field study of demographic faultlines among 5,495 employees in 82 small and medium‐sized firms (<250 employees) support our model. We demonstrate that organizational demographic faultlines have important consequences, and we show that functional heterogeneity changes whether these consequences are negative or positive.
The work‐habits intervention model: A 12‐month study to change work‐email habits
Authors: Russell Emma , Daniels Kevin , Jackson Thomas W. , Fullman Marc 
Published: July 2021

We present a Work‐habit Intervention Model (WhIM) to explain and predict how to change work‐habits to be more effective. Habit change has primarily been researched within the health domain. The WhIM contributes a unique theoretical perspective by: (i) suggesting that work‐habit change requires a two‐stage process of exposure to regular rationalized plans and a stated intention to use these plans; and, (ii) defining effective work‐habit change in terms of improvements to both goal attainment and well‐being over time. Self‐regulatory resources are included as potential moderators of habit change. This approach implies that work‐habits (unlike health‐habits) are seldom constitutionally ‘good’ or ‘bad’, which means that change requires a clear rationale in terms of improving goal attainment and well‐being. The WhIM was evaluated in a 12‐month wait‐list intervention study designed to improve work‐email habits for workers in a UK organization (N = 127 T1; N = 58 T3; N = 46 all data). Findings were that the two‐stage process changed work‐email habits for those with higher levels of self‐efficacy, which predicted well‐being in terms of reduced negative affect (via perceived goal attainment). We outline theoretical and practical implications and encourage future research to refine the WhIM across a range of other work contexts.Practitioner points

Workers need to regularly engage with rationalized plans of action and state their intention to use these, in order to change work‐email habits.

Organizations should consider training workers to enhance their self‐efficacy prior to implementing a work‐email habit change intervention.

Providing regular feedback about the impact of work‐email habit change on well‐being and goal attainment is likely to make the change sustainable in the long‐term.
Identification of the calpain‐generated toxic fragment of ataxin‐3 protein provides new avenues for therapy of Machado–Joseph disease| Spinocerebellar ataxia type 3
Authors: Simões Ana Teresa , Carmona Vítor , Duarte‐Neves Joana , Cunha‐Santos Janete , Pereira de Almeida Luís 
Published: July 2021

AbstractAimsMachado–Joseph disease (MJD) is the most frequent dominantly inherited cerebellar ataxia worldwide. Expansion of a CAG trinucleotide in theMJD1gene translates into a polyglutamine tract within ataxin‐3, which upon proteolysis may lead to MJD. The aim of this work was to understand the in vivo contribution of calpain proteases to the pathogenesis of MJD. Therefore, we investigated (a) the calpain cleavage sites in ataxin‐3 protein, (b) the most toxic ataxin‐3 fragment generated by calpain cleavage and (c) whether targeting calpain cleavage sites of mutant ataxin‐3 could be a therapeutic strategy for MJD.MethodsWe generated truncated and calpain‐resistant constructs at the predicted cleavage sites of ataxin‐3 using inverse PCR mutagenesis. Lentiviral vectors encoding these constructs were transduced in the adult mouse brain prior to western blot and immunohistochemical analysis 5 and 8 weeks later.ResultsWe identified the putative calpain cleavage sites for both wild‐type and mutant ataxin‐3 proteins. The mutation of these sites eliminated the formation of the toxic fragments, namely, the 26‐kDa fragment, the major contributor for striatal degeneration. Nonetheless, reducing the formation of both the 26‐ and 34‐kDa fragments was required to preclude the intranuclear localisation of ataxin‐3. A neuroprotective effect was observed upon mutagenesis of calpain cleavage sites within mutant ataxin‐3 protein.ConclusionsThese findings suggest that the calpain system should be considered a target for MJD therapy. The identified calpain cleavage sites will contribute to the design of targeted drugs and genome editing systems for those specific locations.
SIMP1 modulates salt tolerance by elevating ERAD efficiency through UMP1A‐mediated proteasome maturation in plants
Authors: He Jiaxian , Zhuang Yufen , Li Chuan , Sun Xia , Zhao Shuangshuang , Ma Changle , Lin Honghui , Zhou Huapeng 
Published: July 2021

Summary

Salt stress significantly induces accumulation of misfolded or unfolded proteins in plants. Endoplasmic reticulum (ER)‐associated protein degradation (ERAD) and other degradative machineries function in the degradation of these abnormal proteins, leading to enhanced salt tolerance in plants.

Here we characterise that a novel receptor‐like kinase, Salt‐Induced Malectin‐like domain‐containing Protein1 (SIMP1), elevates ERAD efficiency during salt stress through UMP1A, a putative proteasome maturation factor in Arabidopsis.

SIMP1 loss‐of‐function caused a salt‐hypersensitive phenotype. SIMP1 interacts and phosphorylates UMP1A, and the protein stability of UMP1A is positively regulated by SIMP1. SIMP1 modulates the 26S proteasome maturation possibly through enhancing the recruitment of specific β subunits of the core catalytic particle to UMP1A. Functionally, the SIMP1–UMP1A module plays a positive role in ERAD efficiency in Arabidopsis. The degradation of misfolded/unfolded proteins was impaired in both simp1 and ump1a mutants during salt stress. Consistently, both simp1 and ump1a plants exhibited reduced ER stress tolerance. Phenotypic analysis revealed that SIMP1 regulates salt tolerance through UMP1A at least in part.

Taken together, our work demonstrated that SIMP1 modulates plant salt tolerance by promoting proteasome maturation via UMP1A, therefore mitigating ER stress through enhanced ERAD efficiency under saline conditions.