147 research outputs found

    Non muscle stem cells and muscle regeneration

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    Star image, celebrity reality television and the fame cycle

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    In this article, I discuss the phenomenon of celebrity reality television and explore its function for those participating in it. Drawing on the success of their non-celebrity counterparts, programmes such as Celebrity Big Brother, I’m a Celebrity… Get Me Out of Here and Dancing With the Stars have become popular globally and, although arguably no longer at their peak, continue to attract large audiences and significant amounts of publicity. In the article I discuss the role these shows can serve for celebrities at different levels of their careers. I argue that reality television appeals in different ways to celebrities at different points in the fame ‘cycle’: ‘ordinary’ people or ‘pre-celebrities’ seeking to become known through it; proto-celebrities who wish to expand their fame; celebrities engaged in the work of promotion for their other endeavours; celebrities who wish to remake their existing star image through using reality television as a rehabilitative strategy or an opportunity to develop new skills; and those whose careers are in a period of ‘post-celebrity’ who seek to renew their fame. I explore how a successful reality show cast is one that combines celebrities who are at a range of points in the fame cycle as the interactions between the cast members and their debates about fame and hierarchy prove a key attraction for audiences

    High-Throughput Analysis of Promoter Occupancy Reveals New Targets for Arx, a Gene Mutated in Mental Retardation and Interneuronopathies

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    Genetic investigations of X-linked intellectual disabilities have implicated the ARX (Aristaless-related homeobox) gene in a wide spectrum of disorders extending from phenotypes characterised by severe neuronal migration defects such as lissencephaly, to mild or moderate forms of mental retardation without apparent brain abnormalities but with associated features of dystonia and epilepsy. Analysis of Arx spatio-temporal localisation profile in mouse revealed expression in telencephalic structures, mainly restricted to populations of GABAergic neurons at all stages of development. Furthermore, studies of the effects of ARX loss of function in humans and animal models revealed varying defects, suggesting multiple roles of this gene during brain development. However, to date, little is known about how ARX functions as a transcription factor and the nature of its targets. To better understand its role, we combined chromatin immunoprecipitation and mRNA expression with microarray analysis and identified a total of 1006 gene promoters bound by Arx in transfected neuroblastoma (N2a) cells and in mouse embryonic brain. Approximately 24% of Arx-bound genes were found to show expression changes following Arx overexpression or knock-down. Several of the Arx target genes we identified are known to be important for a variety of functions in brain development and some of them suggest new functions for Arx. Overall, these results identified multiple new candidate targets for Arx and should help to better understand the pathophysiological mechanisms of intellectual disability and epilepsy associated with ARX mutations

    Mediated Class-ifications: Representations of Class and Culture in Contemporary British Television

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    This article takes, as its point of departure, recent debates about the representation of working-class life, especially the lives of the 'feckless poor', on reality television in the UK. These issues are contextualized by reference to a set of wider-ranging historical debates about: a) the category of class as a mode of social determination (and as an explanatory model); b) the relations of language, class and culture in educational sociology and in community publishing; and, c) in relation to classical Marxism's theorization of both the 'respectable' working class and the lumpen proletariat. The article concludes with a consideration of debates about the representation of the working class in the contemporary British TV drama series Shameless

    Recessive mutations in muscle-specific isoforms of FXR1 cause congenital multi-minicore myopathy

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    FXR1 is an alternatively spliced gene that encodes RNA binding proteins (FXR1P) involved in muscle development. In contrast to other tissues, cardiac and skeletal muscle express two FXR1P isoforms that incorporate an additional exon-15. We report that recessive mutations in this particular exon of FXR1 cause congenital multi-minicore myopathy in humans and mice. Additionally, we show that while Myf5-dependent depletion of all FXR1P isoforms is neonatal lethal, mice carrying mutations in exon-15 display non-lethal myopathies which vary in severity depending on the specific effect of each mutation on the protein

    The Satellite Cell in Male and Female, Developing and Adult Mouse Muscle: Distinct Stem Cells for Growth and Regeneration

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    Satellite cells are myogenic cells found between the basal lamina and the sarcolemma of the muscle fibre. Satellite cells are the source of new myofibres; as such, satellite cell transplantation holds promise as a treatment for muscular dystrophies. We have investigated age and sex differences between mouse satellite cells in vitro and assessed the importance of these factors as mediators of donor cell engraftment in an in vivo model of satellite cell transplantation. We found that satellite cell numbers are increased in growing compared to adult and in male compared to female adult mice. We saw no difference in the expression of the myogenic regulatory factors between male and female mice, but distinct profiles were observed according to developmental stage. We show that, in contrast to adult mice, the majority of satellite cells from two week old mice are proliferating to facilitate myofibre growth; however a small proportion of these cells are quiescent and not contributing to this growth programme. Despite observed changes in satellite cell populations, there is no difference in engraftment efficiency either between satellite cells derived from adult or pre-weaned donor mice, male or female donor cells, or between male and female host muscle environments. We suggest there exist two distinct satellite cell populations: one for muscle growth and maintenance and one for muscle regeneration
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