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OBJECTIVE The objective of this study was to establish the feasibility of long-term gentamicin dosing to achieve stop codon readthrough and produce full-length dystrophin. Mutation suppression of stop codons, successfully achieved in the mdx mouse using gentamicin, represents an important evolving treatment strategy in Duchenne muscular dystrophy (DMD). (More)
BACKGROUND Meta-analyses of studies investigating the impact of maternal environmental tobacco smoke (ETS) on birth weight have not produced robust findings. Although, ante natal ETS exposure probably reduces infant's birth weights, the scale of this exposure remains unknown. We conducted a large, cohort study to assess the impact of ETS exposure on birth(More)
OBJECTIVE alpha-Sarcoglycan deficiency results in a severe form of muscular dystrophy (limb-girdle muscular dystrophy type 2D [LGMD2D]) without treatment. Gene replacement represents a strategy for correcting the underlying defect. Questions related to this approach were addressed in this clinical trial, particularly the need for immunotherapy and(More)
OBJECTIVE The aim of this study was to attain long-lasting alpha-sarcoglycan gene expression in limb-girdle muscular dystrophy, type 2D (LGMD2D) subjects mediated by adeno-associated virus (AAV) gene transfer under control of a muscle specific promoter (tMCK). METHODS rAAV1.tMCK.hSGCA (3.25 × 10¹¹ vector genomes) was delivered to the extensor digitorum(More)
OBJECTIVES To estimate global, regional (21 regions) and national (187 countries) sodium intakes in adults in 1990 and 2010. DESIGN Bayesian hierarchical modelling using all identifiable primary sources. DATA SOURCES AND ELIGIBILITY We searched and obtained published and unpublished data from 142 surveys of 24 h urinary sodium and 103 of dietary sodium(More)
Digital social media is dramatically changing the social landscape and the ways in which we understand 'participation'. As youth embraces these dynamic yet highly scripted forms of mediated social interaction, educators have struggled to find ways to harness these new participatory forms to support learning. This article considers the interactive structures(More)
BACKGROUND We have previously suggested that cryptogenic fibrosing alveolitis (CFA) may be caused by occupational exposures, particularly to metal or wood dust. We have specifically investigated this hypothesis in a case-control study of patients with CFA. METHODS We obtained lifetime occupational histories by postal questionnaire from 218 patients with(More)
An important element of evaluating a large wildfire is to assess its effects on the soil in order to predict the potential watershed response. After the 55 000 ha Hayman Fire on the Colorado Front Range, 24 soil and vegetation variables were measured to determine the key variables that could be used for a rapid field assessment of burn severity. The(More)
You may order additional copies of this publication by sending your mailing information in label form through one of the following media. Please specify the publication title and series number. AbstrAct This report provides managers with the current state of knowledge regarding the effectiveness of fuel treatments for mitigating severe wildfire effects. A(More)