532 research outputs found

    Building governance and energy efficiency: Mapping the interdisciplinary challenge

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    Improving the energy efficiency of multi-owned properties (MoPs)—commonly known as apartment or condominium buildings—is central to the achievement of European energy targets. However, little work to date has focused on how to facilitate retrofit in this context. Drawing on interdisciplinary Social Sciences and Humanities expertise in academia, policy and practice, this chapter posits that decision-making processes within MoPs might provide a key to the retrofit challenge. Existing theories or models of decision-making, applied in the MoP context, might help to explain how collective retrofit decisions are taken—or overlooked. Insights from case studies and practitioners are also key. Theories of change might then be employed to develop strategies to facilitate positive retrofit decisions. The chapter maps the issues and sets an agenda for further interdisciplinary research in this novel area

    Facial-muscle weakness, speech disorders and dysphagia are common in patients with classic infantile Pompe disease treated with enzyme therapy

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    Classic infantile Pompe disease is an inherited generalized glycogen storage disorder caused by deficiency of lysosomal acid α-glucosidase. If left untreated, patients die before one year of age. Although enzyme-replacement therapy (ERT) has significantly prolonged lifespan, it has also revealed new aspects of the disease. For up to 11 years, we investigated the frequency and consequences of facial-muscle weakness, speech disorders and dysphagia in long-term survivors. Sequential photographs were used to determine the timing and severity of facial-muscle weakness. Using standardized articulation tests and fibreoptic endoscopic evaluation of swallowing, we investigated speech and swallowing function in a subset of patients. This study included 11 patients with classic infantile Pompe disease. Median age at the start of ERT was 2.4 months (range 0.1-8.3 months), and median age at the end of the study was 4.3 years (range 7.7 months −12.2 years). All patients developed facial-muscle weakness before the age of 15 months. Speech was studied in four patients. Articulation was disordered, with hypernasal resonance and reduced speech intelligibility in all four. Swallowing function was studied in six patients, the most important findings being ineffective swallowing with residues of food (5/6), penetration or aspiration (3/6), and reduced pharyngeal and/or laryngeal sensibility (2/6). We conclude that facial-muscle weakness, speech disorders and dysphagia are common in long-term survivors receiving ERT for classic infantile Pompe disease. To improve speech and reduce the risk for aspiration, early treatment by a speech therapist and regular swallowing assessments are recommended

    Lipid Metabolites Enhance Secretion Acting on SNARE Microdomains and Altering the Extent and Kinetics of Single Release Events in Bovine Adrenal Chromaffin Cells

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    Lipid molecules such as arachidonic acid (AA) and sphingolipid metabolites have been implicated in modulation of neuronal and endocrine secretion. Here we compare the effects of these lipids on secretion from cultured bovine chromaffin cells. First, we demonstrate that exogenous sphingosine and AA interact with the secretory apparatus as confirmed by FRET experiments. Examination of plasma membrane SNARE microdomains and chromaffin granule dynamics using total internal reflection fluorescent microscopy (TIRFM) suggests that sphingosine production promotes granule tethering while arachidonic acid promotes full docking. Our analysis of single granule release kinetics by amperometry demonstrated that both sphingomyelinase and AA treatments enhanced drastically the amount of catecholamines released per individual event by either altering the onset phase of or by prolonging the off phase of single granule catecholamine release kinetics. Together these results demonstrate that the kinetics and extent of the exocytotic fusion pore formation can be modulated by specific signalling lipids through related functional mechanisms

    The function of fear in institutional maintenance: Feeling frightened as an essential ingredient in haute cuisine

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    Fear is a common and powerful emotion that can regulate behaviour. Yet institutional scholars have paid limited attention to the function of fear in processes of institutional reproduction and stability. Drawing on an empirical study of elite chefs within the institution of haute cuisine, this article finds that the multifaceted emotion of fear characterised their experiences and served to sustain their institution. Chefs’ individual feelings of fear prompted conformity and a cognitive constriction, which narrowed their focus on to the precise reproduction of traditional practices whilst also limiting challenges to the norms underpinning the institution. Through fear work, chefs used threats and violence to connect individual experiences of fear to the violation of institutionalized rules, sustaining the conditions in which fear-driven maintenance work thrived. The study also suggests that fear is a normative element of haute cuisine in its own right, where the very experience and eliciting of fear preserved an essential institutional ingredient. In this way, emotions such as fear do not just accompany processes of institutionalization but can be intimately involved in the maintenance of institutions

    Mitochondrial ATP synthase inhibition and nitric oxide are involved in muscle weakness that occurs in acute exposure of rats to monocrotophos

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    Organophosphate poisoning in the context of self-harm is a common medical emergency in Asia. Prolonged muscle weakness is an important but poorly understood cause of morbidity and mortality of the poisoning. This study examined mitochondrial function and its modulation by nitric oxide in muscle weakness of rats exposed to an acute, oral (0.8LD50) dose of monocrotophos. Muscle mitochondrial ATP synthase activity was inhibited in the rat in acute exposure to monocrotophos while respiration per se was not affected. This was accompanied by decreased mitochondrial uptake of calcium and increased levels of nitric oxide. Reactive cysteine groups of ATP synthase subunits were reduced in number, which may contribute to decreased enzyme activity. The decrease in ATP synthase activity and reactive cysteine groups of ATP synthase subunits was prevented by treatment of animals with the nitric oxide synthase inhibitor, L-NG Nitroarginine methyl ester, at 12 mg/kg body weight for 9 days in drinking water, prior to monocrotophos exposure. This indicated a role for nitric oxide in the process. The alterations in mitochondrial calcium uptake may influence cytosolic calcium levels and contribute to muscle weakness of acute organophosphate exposure

    A role for diatom-like silicon transporters in calcifying coccolithophores

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    Biomineralization by marine phytoplankton, such as the silicifying diatoms and calcifying coccolithophores, plays an important role in carbon and nutrient cycling in the oceans. Silicification and calcification are distinct cellular processes with no known common mechanisms. It is thought that coccolithophores are able to outcompete diatoms in Si-depleted waters, which can contribute to the formation of coccolithophore blooms. Here we show that an expanded family of diatom-like silicon transporters (SITs) are present in both silicifying and calcifying haptophyte phytoplankton, including some globally important coccolithophores. Si is required for calcification in these coccolithophores, indicating that Si uptake contributes to the very different forms of biomineralization in diatoms and coccolithophores. Significantly, SITs and the requirement for Si are absent from highly abundant bloom-forming coccolithophores, such as Emiliania huxleyi. These very different requirements for Si in coccolithophores are likely to have major influence on their competitive interactions with diatoms and other siliceous phytoplankton

    Moving Beyond Mimicry: Developing Hybrid Spaces in Indian Business Schools

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    This article analyses the identity work of Indian management educators and scholars as they seek to establish, maintain and revise a sense of self in the context of business school globalization. We show how globalization, combined with the historical legacy of colonialism, renders Indian scholars precarious in their interactions with Western business schools. Based on a qualitative interview study, we explore how Indian business school scholars perform their identities in the context of neo-colonial relations, which are characterised by the dominance of English language and a pressure to conform to research norms set by globally-ranked journals. Drawing on postcolonial theory, our argument focuses on mimicry as a distinctive form of identity work that involves maintaining difference between Western and non-Western identities by 'Othering' Indian scholars, while simultaneously seeking to transform them. We draw attention to ambivalence within participants' accounts, which we suggest arises because the authority of Western scholarship relies on maintaining non-Western scholars in a position of alterity or 'not quite-ness'. We suggest that hybridity offers an opportunity to disrupt and question current practices of business school globalization and facilitate scholarly engagement that reflects more diverse philosophical positions and worldviews
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