War, Revolution and Design: exploring pedagogy, practice based research and costume for performance through the Russian avant-garde theatre

This article recounts an edited conversation that took place at the V & A symposium ‘Russian Avant-garde Theatre: War Revolution and Design’ held on the 24 January 2014, which accompanied the exhibition of the same name. Fashion historian Amber Jane Butchart (V&A and London College of Fashion, University of the Arts London) led Melissa Trimingham (University of Kent) and Donatella Barbieri (London College of Fashion, University of the Arts London) in a conversation discussing the relationship of Russian costume design with the avant-garde in early Modernism across Europe

Access and allocation in earth system governance: Water and climate change compared

A significant percentage of the global population does not yet have access to safe drinking water, sufficient food or energy to live in dignity. There is a continuous struggle to allocate the earth's resources among users and uses. This article argues that distributional problems have two faces: access to basic resources or ecospace; and, the allocation of environmental resources, risks, burdens, and responsibilities for causing problems. Furthermore, addressing problems of access and allocation often requires access to social processes (science, movements and law). Analysts, however, have tended to take a narrow, disciplinary approach although an integrated conceptual approach may yield better answers. This article proposes a multi-disciplinary perspective to the problem of access and allocation and illustrates its application to water management and climate change. © The Author(s) 2010

Genetic heterogeneity and pathophysiological mechanisms in congenital myasthenic syndromes

Congenital myasthenic syndromes (CMS) are a rare heterogeneous group of inherited neuromuscular disorders associated with distinctive clinical, electrophysiological, ultrastructural and genetic abnormalities. These genetic defects either impair neuromuscular transmission directly or result in secondary impairments, which eventually compromise the safety margin of neuromuscular transmission. In this report we will explore the significant progress made in understanding the molecular pathogenesis of CMS, which is important for both patients and clinicians in terms of reaching a definite diagnosis and selecting the most appropriate treatment

Mutation in the human acetylcholinesterase-associated collagen gene, COLQ, is responsible for congenital myasthenic syndrome with end-plate acetylcholinesterase deficiency (Type Ic).

Congenital myasthenic syndrome (CMS) with end-plate acetylcholinesterase (AChE) deficiency is a rare autosomal recessive disease, recently classified as CMS type Ic (CMS-Ic). It is characterized by onset in childhood, generalized weakness increased by exertion, refractoriness to anticholinesterase drugs, and morphological abnormalities of the neuromuscular junctions (NMJs). The collagen-tailed form of AChE, which is normally concentrated at NMJs, is composed of catalytic tetramers associated with a specific collagen, COLQ. In CMS-Ic patients, these collagen-tailed forms are often absent. We studied a large family comprising 11 siblings, 6 of whom are affected by a mild form of CMS-Ic. The muscles of the patients contained collagen-tailed AChE. We first excluded the ACHE gene (7q22) as potential culprit, by linkage analysis; then we mapped COLQ to chromosome 3p24.2. By analyzing 3p24.2 markers located close to the gene, we found that the six affected patients were homozygous for an interval of 14 cM between D3S1597 and D3S2338. We determined the COLQ coding sequence and found that the patients present a homozygous missense mutation, Y431S, in the conserved C-terminal domain of COLQ. This mutation is thought to disturb the attachment of collagen-tailed AChE to the NMJ, thus constituting the first genetic defect causing CMS-Ic

Diagnostic performance of QT interval variables from 24-h electrocardiography in the long QT syndrome

International audienceThe long QT syndrome is mainly defined by QT interval prolongation (QTc > 0.44s). However, data obtained in genotyped patients showed that resting QTc measurement alone may be inaccurate for ascertaining the phenotype. The aim of this study was to evaluate the diagnostic performance of QT interval rate-dependence in untreated chromosome 11-linked patients

Digital skin: how developments in digital imaging techniques and culture are informing the design of futuristic surface and fabrication concepts

In the last decade, technical advances in computer graphic (CG) texture mapping and surfacing processes have influenced broader cultural spheres and commercial practices. As emerging idioms from computer graphic imaging (CGI) sectors have become synonymous with related everyday terminology, the terms “skin,” “surface,” and “texture”—common parlance in real-world scenarios and prevalent in textile spheres—have come to intersect physical and digital meaning. Recent aspects of CGI development and use provide a particular framework for this article, which goes on to observe newly emerging textile and surface designers engaged in the creative development of digital skin and associated concepts. Imbued with science fiction, future scenarios, and advances in computer imaging software and hardware capability, these practitioners are hybridizing digital and material languages and challenging conventional approaches to design and fabrication processes. Digital media tools are instrumental in facilitating these practices. As design practitioners challenge perceived notions of textile, substrate, and skin through relatively newfound digital contexts, resulting propositions include high-tech interfaces for communication, surface embellishment, and guise. Also outlined is how such digital propositions are beginning to overlap emerging real and conceptual applications in wider industry and science realms, expanding the nature of and prospect for surface design. Resulting paradigms pertain to the virtual reinvention of skin as a conceptual material interface