bluuebunny/crossref_metadata_2025_split huggingface dataset:
Frataxin deficiency in Friedreich’s ataxia is associated with reduced levels of HAX-1, a regulator of cardiomyocyte death and survival
Authors: Tiano Francesca , Amati Francesca , Cherubini Fabio , Morini Elena , Vancheri Chiara , Maletta Sara , Fortuni Silvia , Serio Dario , Quatrana Andrea , Luffarelli Riccardo , Benini Monica , Alfedi Giulia , Panarello Luca , Rufini Alessandra , Toschi Nicola , Frontali Marina , Romano Silvia , Marcotulli Christian , Casali Carlo , Gioiosa Silvia , Mariotti Caterina , Mongelli Alessia , Fichera Mario , Condò Ivano , Novelli Giuseppe , Testi Roberto , Malisan Florence 
Published: January 2020

AbstractFrataxin deficiency, responsible for Friedreich’s ataxia (FRDA), is crucial for cell survival since it critically affects viability of neurons, pancreatic beta cells and cardiomyocytes. In FRDA, the heart is frequently affected with typical manifestation of hypertrophic cardiomyopathy, which can progress to heart failure and cause premature death. A microarray analysis performed on FRDA patient’s lymphoblastoid cells stably reconstituted with frataxin, indicated HS-1-associated protein X-1 (HAX-1) as the most significantly upregulated transcript (FC = +2, P &lt; 0.0006). quantitative Reverse Transcription-Polymerase Chain Reaction (qRT-PCR) and western blot analysis performed on (I) HEK293 stably transfected with empty vector compared to wild-type frataxin and (II) lymphoblasts from FRDA patients show that low frataxin mRNA and protein expression correspond to reduced levels of HAX-1. Frataxin overexpression and silencing were also performed in the AC16 human cardiomyocyte cell line. HAX-1 protein levels are indeed regulated through frataxin modulation. Moreover, correlation between frataxin and HAX-1 was further evaluated in peripheral blood mononuclear cells (PBMCs) from FRDA patients and from non-related healthy controls. A regression model for frataxin which included HAX-1, group membership and group* HAX-1 interaction revealed that frataxin and HAX-1 are associated both at mRNA and protein levels. Additionally, a linked expression of FXN, HAX-1 and antioxidant defence proteins MnSOD and Nrf2 was observed both in PBMCs and AC16 cardiomyocytes. Our results suggest that HAX-1 could be considered as a potential biomarker of cardiac disease in FRDA and the evaluation of its expression might provide insights into its pathogenesis as well as improving risk stratification strategies.
Muscle weakness and selective muscle atrophy in osteoprotegerin-deficient mice
Authors: Hamoudi Dounia , Bouredji Zineb , Marcadet Laetitia , Yagita Hideo , Landry Louis-Bénédict , Argaw Anteneh , Frenette Jérôme 
Published: January 2020

Abstract
Bone and muscle are tightly coupled and form a functional unit under normal conditions. The receptor-activator of nuclear factor κB/receptor-activator of nuclear factor κB ligand/osteoprotegerin (RANK/RANKL/OPG) triad plays a crucial role in bone remodeling. RANKL inhibition by OPG prevents osteoporosis. In contrast, the absence of OPG results in elevated serum RANKL and early onset osteoporosis. However, the impacts of OPG deletion on muscle structure and function are unknown. Our results showed that 1-, 3- and 5-month-old Opg−/− mice have reduced tibial and femoral bone biomechanical properties and higher levels of circulating RANKL. OPG-deficient mice displayed reduced locomotor activity and signs of muscle weakness at 5 months of age. Furthermore, OPG deficiency did not affect the skeletal muscles in 1- and 3-month-old mice. However, it impaired fast-twitch EDL but not slow-twitch Sol muscles in 5-month-old Opg−/− mice. Moreover, 5-month-old Opg−/− mice exhibited selective atrophy of fast-twitch-type IIb myofibers, with increased expression of atrophic proteins such as NF-kB, atrogin-1 and MuRF-1. We used an in vitro model to show that RANKL-stimulated C2C12 myotubes significantly increased the expression of NF-kB, atrogin-1 and MuRF-1. A 2-month anti-RANKL treatment starting at 3 months of age in Opg−/− mice improved voluntary activity, the ex vivo maximum specific force (sP0) of EDL muscles, and whole limb grip force performance and rescued the biomechanical properties of bone. In conclusion, the deletion of OPG and the disruption of the RANKL/OPG balance induced osteoporosis as well as the selective weakness and atrophy of the powerful fast-twitch IIb myofibers, which was partly alleviated by an anti-RANKL treatment.
What’s good for the goose ain’t good for the gander: heterogeneous innovation capabilities and the performance effects of R&D
Authors: Coad Alex , Mathew Nanditha , Pugliese Emanuele 
Published: January 2020

Abstract
We investigate the effects of R&D investment on performance outcomes (sales growth and relative profitability) for Indian manufacturing firms. Previous research shows contradictory results—while some studies find a positive effect of R&D on firm performance, some find that firms investing in R&D do not perform significantly better, in some cases, even perform worse than their noninvesting counterparts. We claim that the effects of R&D on performance are often misspecified. Indeed, innovation capabilities will probably simultaneously influence the decision to invest in R&D and also R&D’s expected benefits. We apply endogenous switching regression to tackle the issue of selection and censored data, and the results we observe are sharp: Firms investing in R&D would have had less growth and less relative profitability if they had not done so. Interestingly, firms that did not invest in R&D would not have benefited had they done so. We interpret this as evidence that firms need to have sufficiently developed management capabilities to be able to convert R&D investments into tangible results, and that not all firms are well positioned to benefit from R&D investment.
Persisting Antibody Response 9 Years After Bivalent Human Papillomavirus (HPV) Vaccination in a Cohort of Dutch Women: Immune Response and the Relation to Genital HPV Infections
Authors: Hoes Joske , Pasmans Hella , Knol Mirjam J , Donken Robine , van Marm-Wattimena Naomi , Schepp Rutger M , King Audrey J , van der Klis Fiona R M , de Melker Hester E 
Published: January 2020

Abstract
The bivalent human papillomavirus (HPV) vaccine is highly effective and induces robust serological responses. Using a Dutch prospective cohort initiated in 2009, including 744 vaccinated and 294 unvaccinated girls (1993–1994) who provide a vaginal self-swab sample, serum sample, and questionnaire yearly, we report a high, persisting antibody response up to 9 years after vaccination for vaccine types HPV-16 or HPV-18. Antibodies against nonvaccine HPV types 31, 33, 45, 52, and 58 were lower but still significantly higher than in unvaccinated individuals. This was also reflected in the seroprevalence. We compared participant characteristics and antibody levels between vaccinated women with and those without HPV infections 1 year before infection (204 incident and 64 persistent infections), but we observed no consistent difference in type-specific antibody levels. Having a high-risk HPV infection was associated with sexual risk behavior and smoking 1 year before infection. Although high antibody levels are necessary for protection, our study suggests that on the individual level other factors such as HPV exposure or antibody avidity could be important.
Insecticide-treated bed net access and use among preschool children in Nouna District, Burkina Faso
Authors: Sié Ali , Bountogo Mamadou , Ouattara Mamadou , Zabre Pascal , Bagagnan Cheik , Zakane Alphonse , Brogdon Jessica , Lebas Elodie , Lin Ying , Godwin William W , Bärnighausen Till , Lietman Thomas M , Oldenburg Catherine E ,   the Étude CHAT Study Group
Published: January 2020

Abstract

Background
We evaluated universal insecticide-treated bed net access and use in children &lt;5 y of age in a rural area of Burkina Faso.

Methods
A door-to-door enumerative census was conducted in Nouna District, Burkina Faso in December 2018 through April 2019. The most recent mass bed net distribution campaign occurred in June 2016. Heads of households were interviewed about household bed net ownership and use by children &lt;5 y of age. We evaluated the relationship between demographic and socio-economic factors and household universal bed net access and use by children.

Results
In 23 610 households with at least one child &lt;5 y of age, 71 329 bed nets were reported (94.5% insecticide-treated). One-third (35.2%) of households had universal access and two-thirds (67.0%) of children slept under an insecticide-treated net the previous night. Children in households with universal access more often slept under a net the previous night (adjusted odds ratio 4.81 [95% confidence interval 4.39–5.26]).

Conclusions
Bed net coverage was substantially less than the 80% World Health Organization target for universal coverage in Nouna District. Insecticide-treated nets were used preferentially for children, but important gaps remain in consistent bed net use in this population. Structural and behavioural interventions are needed to close these gaps.
Mimicking cotranslational folding of prosubtilisin E in vitro
Authors: Kim Sung-Gun , Chen Yu-Jen , Falzon Liliana , Baum Jean , Inouye Masayori 
Published: January 2020

AbstractNascent polypeptides are synthesized on ribosomes starting at the N-terminus and simultaneously begin to fold during translation. We constructed N-terminal fragments of prosubtilisin E containing an intramolecular chaperone (IMC) at N-terminus to mimic cotranslational folding intermediates of prosubtilisin. The IMC-fragments of prosubtilisin exhibited progressive enhancement of their secondary structures and thermostabilities with increasing polypeptide length. However, even the largest IMC-fragment with 72 residues truncated from the C-terminus behaved as a molten globule, indicating the requirement of the C-terminal region to have a stable tertiary structure. Furthermore, truncation of the IMC in the IMC-fragments resulted in aggregation, suggesting that the IMC plays a crucial role to prevent misfolding and aggregation of cotranslational folding intermediates during translation of prosubtilisin polypeptide.
Predicting the Potential Invasive Range of Klambothrips myopori (Thysanopetra: Phlaeothripidae)
Authors: Shogren Christopher , Paine Timothy 
Published: January 2020

Abstract
Temperature-driven development of myoporum thrips, Klambothrips myopori (Thysanopetra: Phlaeothripidae), was examined at seven constant temperatures (15, 17, 20, 25, 30, 34, and 35.5°C) on Myoporum laetum Forst (Lamiales: Scrophulariaceae). Thrips successfully completed development to adult stage between 15 and 35.5°C. One linear and three nonlinear models were fitted to describe developmental rates of K. myopori as a function of temperature, and for estimating thermal constants and bioclimatic thresholds (Tmin, Topt, and Tmax). The Briere-1 model performed best in describing the developmental rate of cumulative life stages. Two ecological niche models, CLIMEX and Maxent, were used to predict the geographic distribution of K. myopori in its native range and globally. Overall predictions of environmental suitability differed greatly across models. The CLIMEX model accurately predicted known invasive and native localities, while the Maxent model failed to predict the native localities and parts of the invasive range. Based on the CLIMEX model, K. myopori has the potential to establish in many regions of the globe.
Effect of Rice Winter Cultural Management Practices on the Size of the Hatching Population of Triops longicaudatus (Notostraca: Triopsidae) in California Rice Fields
Authors: Bloese Joanna B , Goding Kevin M , Godfrey Larry D 
Published: January 2020

Abstract
The tadpole shrimp [Triops longicaudatus (Leconte)] has emerged as a significant pest of rice grown in California in recent decades. The change in T. longicaudatus’ pest status has coincided with changes in cultural management of residual rice straw postharvest. Policy changes have reduced the postharvest burning of fields from nearly 95% to less than 10%, promoting increased use of winter flooding as a means of accelerating straw decomposition. Field and laboratory trials were conducted from 2015 to 2017 at the Rice Experiment Station in Biggs, CA and in greenhouses at the University of California (UC) Davis to evaluate the effects of burning, flooding, and a fallow control on T. longicaudatus population dynamics. Experiments demonstrated that burning of rice straw failed to suppress densities of hatching T. longicaudatus and actually had the reverse effect, causing a 51% increase in numbers hatching, perhaps as a result of burning triggering termination of multiyear T. longicaudatus egg dormancy. Winter flooding had no measurable effect on T. longicaudatus hatch. Thus, these changes in winter cultural practices do not appear to be responsible for the emergence of T. longicaudatus as a major rice pest.
Incidence of Citrus leprosis virus C and Orchid fleck dichorhavirus Citrus Strain in Mites of the Genus Brevipalpus in Mexico
Authors: Beltran-Beltran Ana Karen , Santillán-Galicia Ma Teresa , Guzmán-Franco Ariel W , Teliz-Ortiz Daniel , Gutiérrez-Espinoza María Alejandra , Romero-Rosales Felipe , Robles-García Pedro L 
Published: January 2020

Abstract
The incidences of Citrus leprosis virus C (CiLV-C) and Orchid fleck dichorhavirus Citrus strain (OFV-citrus) were determined in field populations of Brevipalpus mites from 15 citrus-producing states in Mexico. Mites were collected from orange, grapefruit, mandarin, lime, and sweet lime orchards. Brevipalpus yothersi (Baker) (Trombidiformes: Tenuipalpidae) was the most abundant species followed by Brevipalpus californicus (Banks) (Trombidiformes: Tenuipalpidae), which confirmed previous reports. The viruses CiLV-C and OFV-citrus were found in both mite species. The incidence of CiLV-C, OFV-citrus and both viruses simultaneously (CiLV-C and OFV-citrus) was 17.2, 10.3, and 3.4% (n = 116) for B. yothersi, and 12.5, 20.8, and 4.1% (n = 24) for B. californicus, respectively. No significant difference was found when the incidence of these viruses was compared between both mite species. The importance of our results in relation to the epidemiology of leprosis is discussed.
Mating Attributes Relating to Parasitization and Productivity in Habrobracon hebetor (Hymenoptera: Braconidae) Rearing on Host Indian Meal Moth (Lepidoptera: Pyralidae)
Authors: Hasan Mahbub , Hasan Mehedi , Khatun Raosonara , Hossain Akhtar , Athanassiou Christos G , Bari Abdul 
Published: January 2020

Abstract
The mating attributes in relating to parasitism and progeny production capacity of the parasitoid Habrobracon hebetor Say, using Plodia interpunctella (Hübner) as a host, were investigated in laboratory studies. The results indicated that 100% of the host larvae were parasitized by different individuals of H. hebetor females that were mated by the same male. Mating duration did not differ significantly among different successive matings with different females and had no effect on the rate of parasitism. In general, pairs that had their copulation early after emergence exhibited the maximum rate of parasitism as compared to delayed intervals. Moreover, the age of the H. hebetor females had no effect on the percentage of parasitism. The maximum parasitism percentage was recorded for the H. hebetor individuals that had been developed from the age group of the 6-d-old adults. The male: female ratios did not differ significantly among the female age groups. The percentage of parasitism clearly indicated a decrease with the increase of the male: female ratio. Overall, the results of the present work can be further utilized in mass rearing and release of H. hebetor in biological control programs in stored product protection.
Continued growth of locally aggressive fibrous dysplasia of 22 years duration after reaching adulthood: a case report
Authors: Yamagishi Yusuke , Okamoto Masanori , Yoshimura Yasuo , Kito Munehisa , Aoki Kaoru , Takahashi Jun 
Published: January 2020

Abstract
Fibrous dysplasia generally stops growing when patients reach adulthood. Locally aggressive fibrous dysplasia is an extremely rare subtype of fibrous dysplasia that is characterized by progressive enlargement after bone maturation, cortical bone destruction and soft tissue invasion but without malignant transformation. At 50 years of age, a tumor was found in the rib of a patient. The tumor gradually enlarged over time and imaging findings suggested a malignant tumor. The case was further complicated by restrictive lung disorder. Biopsies from multiple sites showed no malignant findings, and marginal resection with partial curettage was performed. The final diagnosis was locally aggressive fibrous dysplasia, and the restrictive lung disorder improved postoperatively. The natural history of the disease is also unknown. This is the first report in the literature to describe a case in which a lesion exhibited long-term growth over a period of 22 years after reaching adulthood.
Slave Trade and Slavery in Asia—New Perspectives
Authors: Chakraborty Titas , van Rossum Matthias 
Published: January 2020

Abstract
Recent years have witnessed an expanding body of scholarship indicating the importance of slave trade and slavery in different parts of the Indian Ocean and Indonesian archipelago worlds. This work has not only challenged the dominant focus of slavery scholarship on the Atlantic context but has also encouraged scholars to reassess wider perspectives on Asian and global social histories. This special issue brings together contributions that explore these new horizons. Together, they take up the issue of slavery and mobility in different parts of the Indian Ocean and Indonesian archipelago worlds from a comparative perspective, dealing not only with the existence and patterns of slave trade itself but also with its social and sociopolitical implications. These articles require us to rethink some of the dominant perspectives in a historiography that for a long time has emphasized the unique and local character of “Asian” slaveries, positing dichotomies between slavery in the Atlantic and elsewhere, as well as between Western and non-Western slaveries. The contributions to this special issue challenge several of these existing dichotomies and provide new contributions to the understanding of the role and importance of slavery from a global perspective, as well as to the history of the Indian Ocean and Indonesian archipelago worlds. This introduction reflects on this collective contribution and aims to provide an outline for a relevant research agenda.
Trust in the invisible hand? The roles of the State and the markets in EU energy law
Authors: Huhta Kaisa 
Published: January 2020

Abstract
The article analyses the European Union (EU) legal approaches that govern security of electricity supply. This legal analysis demonstrates how EU law allocates power and responsibility between the State and the markets in the electricity sector to ensure the uninterrupted availability of affordable electricity. The article argues that the allocation of roles and responsibilities has been similarly structured in respect of different timescales of security of supply in the Clean Energy for All Europeans package. This legal structure relies heavily on the invisible hand of the market, in which context the role of the State is limited to providing a fallback in the event that the market is unable to achieve the objective of security of electricity supply.
Can Robots, Apps, and Other Technologies Meet the Future Global Demands of Dementia?
Authors: Astell Arlene , Semple James 
Published: January 2020

Abstract
Lower- and low- to middle-income countries are predicted to experience the fastest growth in numbers of people living with dementia by 2050, although they have the least developed infrastructure for diagnosis and support. As there are currently no disease-modifying therapies for dementia and only modest symptomatic relief, all that is available for people who receive a diagnosis is care and support to live as well as possible. The potential of digital technologies, including robots, smart environments, and mobile devices, to address the rising challenges posed by dementia is increasingly being explored. This chapter summarizes the major developments in technology for supporting people to live with dementia and examines their relevance and potential for addressing the challenges faced by some of the world’s poorest countries. All technologies are hampered by a lack of research investment, but even so, mobile platforms offer the most potential for deployment at scale.
Ethical Concerns About the Use of Assistive Technologies
Authors: Elger Bernice S. 
Published: January 2020

Abstract
Uptake of possible beneficial assistive technology is presently hampered by ethical concerns. It is important to examine the ethical issues raised by distinct types of existing technologies in more detail than has been done previously to obtain a nuanced judgment as to whether public health authorities and healthcare personnel should inform patients and interested stakeholders about them, offer patients and their informal caregivers more choice concerning the use of available assistive technologies, or even actively recommend some of them. The present chapter will focus on four examples of assistive technologies that, despite having been available for some time and being relatively simple to use, are not widely implemented: memory aid technology, “smart dresser” devices designed to help dementia patients with getting dressed, GPS tracking devices, and sensors to monitor patients in their private homes such as an intelligent wireless sensor system for the rapid detection of health issues. The first two technologies are chosen as examples for “aid” technology where the risk of harm is very low compared to significant benefits for patients, caregivers, and society. The second two are chosen because of their ethically relevant characteristics of control: their aim is patient surveillance and monitoring. The ethical issues related to these technologies will be discussed and conclusions drawn on how to appropriately balance beneficence and respect for patient autonomy.
Issues of Informed Consent from Persons with Dementia When Employing Assistive Technologies
Authors: Novitzky Peter , Chen Cynthia , Smeaton Alan F. , Verbruggen Renaat , Gordijn Bert 
Published: January 2020

Abstract
The use of assistive technologies (ATs) in the provision of healthcare promises to provide novel opportunities to protect, empower, and extend the autonomy of persons with dementia (PwDs). However, it also poses autonomy-related challenges, especially regarding informed consent. Traditional informed consent procedures, aimed at the protection of the autonomy of research participants and patients undergoing treatment, are not readily applicable to PwDs when it comes to deciding about ATs. This chapter analyzes the ethical challenges of obtaining informed consent from PwDs for research and development, and the use of ATs. The analysis reviews both traditional informed consent procedures and more innovative ones. The chapter will conclude with a list of recommendations, based on the findings of the ethical analysis.