bluuebunny/crossref_metadata_2025_split huggingface dataset:
Atxn2-CAG100-KnockIn mouse spinal cord shows progressive TDP43 pathology associated with cholesterol biosynthesis suppression
Authors: Canet-Pons Júlia , Sen Nesli-Ece , Arsovic Aleksandar , Almaguer-Mederos Luis-Enrique , Halbach Melanie V. , Key Jana , Döring Claudia , Kerksiek Anja , Picchiarelli Gina , Cassel Raphaelle , René Frédérique , Dieterlé Stéphane , Hein-Fuchs Nina , König Renate , Dupuis Luc , Lütjohann Dieter , Gispert Suzana , Auburger Georg 
Published: November 2019

AbstractLarge polyglutamine expansions in Ataxin-2 (ATXN2) cause multi-system nervous atrophy in Spinocerebellar Ataxia type 2 (SCA2). Intermediate size expansions carry a risk for selective motor neuron degeneration, known as Amyotrophic Lateral Sclerosis (ALS). Conversely, the depletion of ATXN2 prevents disease progression in ALS. Although ATXN2 interacts directly with RNA, and in ALS pathogenesis there is a crucial role of RNA toxicity, the affected functional pathways remain ill defined. Here, we examined an authentic SCA2 mouse model withAtxn2-CAG100-KnockIn for a first definition of molecular mechanisms in spinal cord pathology. Neurophysiology of lower limbs detected sensory neuropathy rather than motor denervation. Triple immunofluorescence demonstrated cytosolic ATXN2 aggregates sequestrating TDP43 and TIA1 from the nucleus. In immunoblots, this was accompanied by elevated CASP3, RIPK1 and PQBP1 abundance. RT-qPCR showed increase ofGrn,Tlr7andRnaset2mRNA versusEif5a2,Dcp2, Uhmk1andKif5adecrease. These SCA2 findings overlap well with known ALS features. Similar to other ataxias and dystonias, decreased mRNA levels forUnc80,Tacr1,Gnal,Ano3,Kcna2,Elovl5andCdr1contrasted withGpnmbincrease. Preterminal stage tissue showed strongly activated microglia containing ATXN2 aggregates, with parallel astrogliosis. Global transcriptome profiles from stages of incipient motor deficit versus preterminal age identified molecules with progressive downregulation, where a cluster of cholesterol biosynthesis enzymes includingDhcr24,Msmo1,Idi1andHmgcs1was prominent. Gas chromatography demonstrated a massive loss of crucial cholesterol precursor metabolites. Overall, the ATXN2 protein aggregation process affects diverse subcellular compartments, in particular stress granules, endoplasmic reticulum and receptor tyrosine kinase signaling. These findings identify new targets and potential biomarkers for neuroprotective therapies.
piRNA clusters need a minimum size to control transposable element invasions
Authors: Kofler Robert 
Published: November 2019

AbstractpiRNA clusters are thought to repress transposable element activity in mammals and invertebrates. Here we show that a simple population genetics model reveals a constraint on the size of piRNA clusters: the total size of the piRNA clusters of an organism ought to exceed 0.2% of a genome. Larger piRNA clusters accounting for up to 3% of the genome may be necessary when populations are small, transposition rates are high and TE insertions recessive. If piRNA clusters are too small the load of deleterious TE insertions accumulating during a TE invasion may drive populations extinct before an effective piRNA based defence against the TE can be established. Our finding is solely based on three well supported assumptions: i) TEs multiply withing genomes, ii) TEs are mostly deleterious and iii) piRNA clusters act as transposons traps, where a single insertion in a cluster silences all TE copies in trans. Interestingly, piRNA clusters of some species meet our minimum size requirements while clusters of other species don’t. Species with small piRNA clusters, such as humans and mice, may experience severe fitness reductions during invasions of novel TEs, possibly even threatening the persistence of some populations. This work also raises the important question of how piRNA clusters evolve. We propose that the size of piRNA clusters may be at an equilibrium between evolutionary forces that act to expand and contract piRNA clusters.
Drug use and criminality among unaccompanied refugee minors: a review of the literature
Authors: Ivert Anna-Karin , Magnusson Mia-Maria 
Published: November 2019

Purpose
Organisations working with children have acknowledged that unaccompanied refugee minors (URM) across Europe are exposed to environments and situations that put them at risk for becoming addicted to drugs or becoming involved in crime. The purpose of this paper is to study an examination of existing international research concerning URM and of whether, and if so how, issues relating to drug use and criminality among these children are discussed in the international literature.

Design/methodology/approach
A literature review was conducted using PsycINFO, PubMed, Sociological abstracts and ERIC databases, which together cover the social and behavioural science and also medicine.

Findings
Findings from the present review show that the issues of drug abuse and criminality among URM are rarely acknowledged in the international research literature. When the occurrence of substance abuse and/or criminality is discussed, it is often in relation to mental health problems and in terms of self-medication, i.e. that alcohol or drugs are used by the URM to cope with painful experiences or mental health problems, and also with the challenges of integrating into a new society, difficulties finding work, unsuitable living conditions and a lack of social support.

Originality/value
This review shows that several researchers have emphasised that untreated mental health problems, stressful living conditions and a lack of support and control might put these children at risk for substance abuse and criminality, and this suggests a need for further research in this area.
The sharing economy, regulations, and the role of local government
Authors: Park Minkyung 
Published: November 2019

PurposeThe purpose of this paper is to explore regulatory issues of short-term rentals (STR) and evaluate what constitutes effective regulation of STR by analyzing the STR ordinance in the City of Charleston, South Carolina, as well as reviewing the regulation process the City took.Design/methodology/approachIn this case study, the study employed a documentary research method (Ahmed, 2010) using multiple sources, including government documents, historical statistics and local publications. Prior to analyzing documents, face-to-face interviews with a couple of key government officials were conducted to understand the overall context of the regulation processes as well as to obtain primary information and data pertaining to Charleston’s STR regulation processes.FindingsThe study identified three noteworthy points. First, the legalization process was transparent and democratic; opportunities for stakeholder participation in crafting the Charleston’s ordinance included the establishment of the STR Task Force and public listening sessions. Second, the Charleston’s STR regulation is designed to protect non-participating residents and the historic characters of its neighborhoods; several measures (e.g. strict eligibility, operational restrictions, licensing and permitting, enforcement) protect non-participants and the neighborhood overall, while the City allows STR to be legal. Third, there is a unique enforcement mechanism and tourism-related governance structure; dedicated staff in the Department of Livability and Tourism enforce the STR laws in Charleston.Originality/valueEmpirical analysis of the STR regulations has never been established. The study provides useful and timely insights for local governments, destination management organizations, tourism scholars, and stakeholders in tourism cities to advance the discussions and debates around STR regulations.
Assesment of zakat distribution
Authors: Rahmat Rahman Saleh , Nurzaman Mohamad Soleh 
Published: November 2019

Purpose
This study aims to assess the feasibility of zakat distribution conducted by zakat community development program, one of the BAZNAS programs in Bringinsari village, Sukorejo subdistrict, Kendal Regency, Central Java using the zakat village index (IDZ).

Design/methodology/approach
IDZ has five components in measuring the effectiveness of charity for the welfare of the community is the economic dimension, the dimension of education, dimension of health, social dimension of humanity and dimensions of da’wah. The economic dimension consists of four variables, namely, the presence of productive economic activities, trade centers and access to transportation and logistics services and the presence of access to financial institutions.

Findings
Based on IDZ measurement in Bringinsari village, it is found that IDZ of this village is 0.47. It showed that the condition of the village is good enough. As the result of this calculation, Bringinsari village is under consideration to receive zakat funding.

Research limitations/implications
This research will be done in Bringinsari village Sukorejo district, Kendal regency from April 1, 2018 until July 2, 2018.

Practical implications
Based on the result of IDZ result done in Bringinsari Sukorejo district, Kendal regency, it is gained index calculation result for about 0.47. Therefore, the conclusion is drawn that the village deserves to get zakat funding. Economic dimension is still low, 0.32. Therefore, in this case, a motivation to help villagers is needed. The exact program in improving the people in Bringinsari based on priority is economic, health, education program in accordance with IDZ calculation.

Social implications
The welfare measurement of zakat receiver or mustahiq adopts welfare index (CIBEST). CIBEST model combines material and spiritual fulfillment of human needs quadrants. This index is split into four categories, namely, welfare, material poverty, spiritual poverty and absolute poverty (Puskas BAZNAS, 2016).

Originality/value
Based on measurement result that has been obtained, the authors can recommend some program activities to help improving their welfare. There are three dimensions, which IDZ prioritized or considered to be assisted, such as economic dimension, activity program for housewives. If Bringinsari has got an indicator as a good village and the impact is good as well, therefore, it can try to move zakat fund distribution to other villages.
The enigma of franchising in China
Authors: Alon Ilan , Kruesi Michael Alexander 
Published: November 2019

Purpose
Although China is among the most populated and fastest growing markets in the world, the reason why China’s franchising scale and scope is so limited remains an enigma. The purpose of this paper is to shed light on franchising in China and offer strategies for practitioners to more effectively operate under a franchise model in the Chinese market.

Design/methodology/approach
This paper reviews both literature and practical sources providing some primary data from interviews conducted with franchise industry practitioners in China.

Findings
The findings of this paper are that foreign entrants to China must carefully consider if they should enter the Chinese market through franchising or if the market may be too difficult to navigate. Franchisors, whose brand is unknown in China or have operations that are too Western-centric, should probably seek international markets closer to home. Those with a high value proposition in the local market and the capabilities to adapt effectively to China’s peculiarities may, however, find a promising and profitable niche to operate in.

Originality/value
This paper focuses on the under examined realities of franchising in the Chinese market. Based on the previous experiences of franchisors in China, the paper raises seven practical strategies that practitioners can use to more successfully enter the Chinese market through franchising. Although China is among the most populated and fastest growing markets in the world, the reason why China’s franchising scale and scope is so limited remains an enigma. According to the International Franchise Association and International Trade Administration reports, China is among the top-ranking countries in terms of trade opportunities, with the top 100 franchisor sales estimated at ¥428bn (about US$66bn). China is the largest franchise market in the world, with over 4,500 franchise systems and some 400,000 franchised outlets in over 70 industries. However, considering China’s US$13.6tn (2018) economy, this is still far lower than its full potential. In addition, the scope of franchising is limited and is mostly centered in food and beverage and retail outlets, which consists of approximately 40 per cent of all franchisors.
Partner or servant
Authors: Wu Yinghao , Jiang Jing 
Published: November 2019

Purpose
The purpose of this paper is to take the perspective of repairing the negative effect of social exclusion, discussing how anthropomorphized brand role (partner vs servant) releases the negative effect of social exclusion.

Design/methodology/approach
In this research, two behavioral studies are conducted. Study 1 uses a one-factor (social exclusion vs social inclusion) between-subjects design. The purpose of Study 1 is to test the effect of social exclusion on consumers’ WTP for the anthropomorphized brand (H1). Study 2 uses a 2 (self-esteem (SE): high vs low) × 2 (anthropomorphized brand role: servant vs partner) between-subjects design. The aim of Study 2 is to investigate that after being socially excluded, how anthropomorphized brand roles (servant vs partner) and SE interactively release individuals’ negative feelings (H2a and H2b) and how the need for control recovery mediates this interaction effect (H3).

Findings
This study proposes that when individuals are socially excluded, they are willing to pay more for anthropomorphized brands than those who are not because anthropomorphized brands provide a quasi-social relationship. This study further posits that socially excluded consumers prefer the different role of anthropomorphized brands, given a different level of SE to meet their needs for control recovery. High self-esteem (HSE) (vs low self-esteem (LSE)) consumers are willing to pay more for a servant-like brand because such brands help them recover from low control by regaining a master role. In contrast, HSE and LSE consumers have no significant differences in WTP for a partner-like brand.

Originality/value
Few research studies have discussed how social exclusion influences individuals’ WTP. To fill this gap, the authors used WTP as the dependent variable, showing that after being socially excluded, individuals tend to pay a higher price for the anthropomorphized brand. Also, the research not only adds a contribution to research on the need for control recovery but also indicates how HSE vs LSE individuals behave differently in socially excluded contexts.
When family firm corporate governance fails: the case of El Corte Inglés
Authors: Sacristán-Navarro María , Cabeza-García Laura 
Published: November 2019

PurposeThe purpose of this paper is to describe internal corporate governance mechanisms in family firms as well as conflicts that may arise among shareholders and family members in the absence of specific corporate governance mechanisms.Design/methodology/approachAfter presenting theoretical concepts, the authors study the case of Spanish family firm El Corte Inglés to understand some of the corporate governance difficulties the company has experienced over the past few years.FindingsThis case illustrates how corporate governance problems can arise because the right mechanisms have not been used, leading to conflicts among family members, valuation problems and power struggles.Practical implicationsThere is a need for family firms to employ suitable corporate governance mechanisms as governance complexity increases.Originality/valueThis study aims to contribute to the understanding of corporate governance problems among family members and their possible solutions.
In-app controls for small business accounting information system: a study of domain understanding
Authors: Ritchi Hamzah , Azis Yudi , Adrianto Zaldy , Setiono Kharisma , Sanjaya Selvia 
Published: November 2019

Purpose
The purpose of this paper is to examine the context of cognitive load and the role of in-app controls that serve as visual aids to promote business process understanding and the use of accounting information system (AIS) for small business users.

Design/methodology/approach
In total, 164 participants from small- and medium-sized enterprises were invited to participate in an experiment with between-subjects 2×2 factorial design. Researchers provided two sets of manipulations in the form of in-app control aids, namely Navigation and Guidance. Groups of individuals either received both navigation and guidance, only navigation or only guidance, or no treatment at all. These four different groups were then tested by a range of tasks to measure user understanding on small business domain knowledge and accounting business process provided by the system.

Findings
The findings indicate that although several early indications were visually observed wherein Navigation and Guidance may help reduce individual cognitive load and hence provide potential value for a better understanding of business process, the statistical analysis has not yet been able to substantiate the differences. Despite visually supporting the hypotheses, neither Navigation nor Guidance proved significant on accuracy (scores), efficiency (time) and individual cognitive difficulties. It appears that a systematic training on the accounting process is arguably imperative in order to reduce the extraneous cognitive load due to a relative gap of accounting logic and users’ knowledge of their business process. Ultimately, it would promote the germane knowledge where the integration of user’s own business process and accounting process can manifest effectively.

Research limitations/implications
Aligned with the findings of the research and its correlation with learning, apparently the learning process is not merely determined not only by the application control features being embedded, but also by the domain knowledge of individuals who interact with the system. Training related to the discussion of the accounting process should be conducted more intensively to minimize the gap between the knowledge upon the problems on individual business process and the mechanism of the accounting process.

Originality/value
This research takes a new approach in examining user acceptance toward an AIS by comparing task performance with and without the assistive devices, to assess how these visual aids may overcome the cognitive load of the individual.
Rebellious eating: older women misbehaving through indulgence
Authors: Moscato Emily M. , Ozanne Julie L. 
Published: November 2019

Purpose
Food rituals are an ever present part of consumers’ lives that have practical implications for well-being. This paper aims to explore how food and its relationship to pleasure evolve, as women navigate social norms around gender and aging.

Design/methodology/approach
Ethnographic data were collected using in-depth interviews and participant observations of members of the Red Hat Society (RHS) across 27 months. This approach provided a more nuanced perspective on how food experiences shape consumption rituals and communal ties over time.

Findings
Older women in the RHS eat rebelliously when they break social norms of gender and aging by indulging together in food and drink. Their rituals of rebellious eating have implications on well-being, heightening their experiential pleasure of food and conviviality and forging social support and a sense of community. The dark side of personal indulgence is explored within a larger framework of food well-being.

Originality/value
This study shows how older women challenge social expectations around age and gender through food pleasure rituals. The concept of rebellious eating is introduced to conceptualize how these older women rethink aging and indulgence within a supportive community of consumption and integrate the concepts into their personal narratives.
Leveraging the coffee experience as a tool for engagement with climate change
Authors: Eiseman Danielle , Jonsson Martin 
Published: November 2019

Purpose
This study aims to investigate the potential of the coffee drinking experience as an engagement tool for climate change. Review the current state of the coffee drinking experience, define it and examine links to climate change communication practices. The argument for the coffee drinking experience as a method for engaging the public on climate change is presented.

Design/methodology
The case study method was used to analyze a small number of existing research on the coffee drinking experience. This method is used to define the coffee drinking experience and identify examples in which it could be leveraged for engaging consumers in climate change.

Findings
The emotive and multi-sensory aspects of the coffee drinking experience, combined with the informal atmosphere of the coffee houses provide a non-threatening environment for discussing complex ideas. This study finds there is scope for further exploration and research on the coffee drinking experience as a tool for public engagement with climate change.

Research limitations/implications
This paper is limited in that it is only an initial exploratory study and has not reached the empirical stage yet. It is further limited to the analysis of secondary data.

Originality
Social science in general and experiential marketing specifically has yet to examine the ability of food or drinking experiences as an engagement tool for climate change.
Foliar fungal diseases respond differently to nitrogen and phosphorus additions in Tibetan alpine meadows
Authors: Liu Xiang , Lu Yawen , Zhang Zhenhua , Zhou Shurong 
Published: November 2019

AbstractThe effects of nitrogen addition on fungal diseases in agroecosystems have been widely documented, while largely unknown is how phosphorus fertilization will affect foliar fungal diseases in natural ecosystems. Here, we implemented a factorial experiment with nitrogen and phosphorus addition in an alpine meadow of the Qinghai‐Tibetan Plateau to test the effects of nutrition fertilization on foliar fungal diseases severity (i.e., % of the leaf area that was covered by fungal lesions) at plant species‐, family‐ and community‐levels, respectively, and also among different disease types (biotroph vs. necrotroph). At plant species‐level, nitrogen and phosphorus addition increased diseases severity for two and five plant species respectively. At plant family‐level, nitrogen addition increased pathogen load on Poaceae plants, while phosphorus fertilization increased pathogen load on Scrophulariaceae. Moreover, we found that addition of both nitrogen (p = .034; coefficient = 0.436) and phosphorus (p = .012; coefficient = 0.622) increased pathogen load of biotrophic pathogens, but only nitrogen addition (p = .010; coefficient = 1.769) increased pathogen load induced by necrotrophic pathogens. At plant community‐level, nitrogen addition (p = .009; coefficient = 0.218) alone, rather than phosphorus addition or the interaction of nitrogen and phosphorus addition, increased community pathogen load. We concluded that nitrogen addition affects foliar fungal diseases more than phosphorus at the community‐level, and fungal diseases caused by necrotrophic pathogens were more sensitive to nitrogen addition.