Medication adherence in patients with myotonic dystrophy and facioscapulohumeral muscular dystrophy

Myotonic dystrophy (DM) and facioscapulohumeral muscular dystrophy (FSHD) are the two most common adult muscular dystrophies and have progressive and often disabling manifestations. Higher levels of medication adherence lead to better health outcomes, especially important to patients with DM and FSHD because of their multisystem manifestations and complexity of care. However, medication adherence has not previously been studied in a large cohort of DM type 1 (DM1), DM type 2 (DM2), and FSHD patients. The purpose of our study was to survey medication adherence and disease manifestations in patients enrolled in the NIH-supported National DM and FSHD Registry. The study was completed by 110 DM1, 49 DM2, and 193 FSHD patients. Notable comorbidities were hypertension in FSHD (44 %) and DM2 (37 %), gastroesophageal reflux disease in DM1 (24 %) and DM2 (31 %) and arrhythmias (29 %) and thyroid disease (20 %) in DM1. Each group reported high levels of adherence based on regimen complexity, medication costs, health literacy, side effect profile, and their beliefs about treatment. Only dysphagia in DM1 was reported to significantly impact medication adherence. Approximately 35 % of study patients reported polypharmacy (taking 6 or more medications). Of the patients with polypharmacy, the DM1 cohort was significantly younger (mean 55.0 years) compared to DM2 (59.0 years) and FSHD (63.2 years), and had shorter disease duration (mean 26 years) compared to FSHD (26.8 years) and DM2 (34.8 years). Future research is needed to assess techniques to ease pill swallowing in DM1 and to monitor polypharmacy and potential drug interactions in DM and FSHD

AAOmega spectroscopy of 29 351 stars in fields centered on ten Galactic globular clusters

Galactic globular clusters have been pivotal in our understanding of many astrophysical phenomena. Here we publish the extracted stellar parameters from a recent large spectroscopic survey of ten globular clusters. A brief review of the project is also presented. Stellar parameters have been extracted from individual stellar spectra using both a modified version of the Radial Velocity Experiment (RAVE) pipeline and a pipeline based on the parameter estimation method of RAVE. We publish here all parameters extracted from both pipelines. We calibrate the metallicity and convert this to [Fe/H] for each star and, furthermore, we compare the velocities and velocity dispersions of the Galactic stars in each field to the Besan\c{c}on Galaxy model. We find that the model does not correspond well with the data, indicating that the model is probably of little use for comparisons with pencil beam survey data such as this.Comment: 6 pages, 5 figures, 4 tables. Accepted for publication in A&A. Data described in tables will be available on CDS (at http://cdsweb.u-strasbg.fr/cgi-bin/qcat?J/A+A/530/A31) once publishe

Methods for Investigating the UPR in Filamentous Fungi

Filamentous fungi have a high-capacity secretory system and are therefore widely exploited for the industrial production of native and heterologous proteins. However, in most cases, the yields of nonfungal proteins are significantly lower than those obtained for fungal proteins. One well-studied bottleneck appears to be the result of slow or aberrant folding of heterologous proteins in the ER during the early stages of secretion within the endoplasmic reticulum, leading to stress responses in the host, including the unfolded protein response (UPR). Most of the key elements constituting the signal transduction pathway of the UPR in Saccharomyces cerevisiae have been identified in filamentous fungi, including the central activation mechanism of the pathway, that is, the stress-induced splicing of an unconventional (nonspliceosomal) intron in orthologs of the HAC1 mRNA. This splicing event relieves a translational block in the HAC1 mRNA, allowing for the translation of the bZIP transcription factor Hac1p that regulates the expression of UPR target genes. The UPR is involved in regulating the folding, yield, and delivery of secretory proteins and that has consequences for fungal lifestyles, including virulence and biotechnology. The recent releases of genome sequences of several species of filamentous fungi and the availability of DNA arrays, GeneChips, and deep sequencing methodologies have provided an unprecedented resource for exploring expression profiles in response to secretion stresses. Furthermore, genome-wide investigation of translation profiles through polysome analyses is possible, and here, we outline methods for the use of such techniques with filamentous fungi and, principally, Aspergillus niger. We also describe methods for the batch and controlled cultivation of A. niger and for the replacement and study of its hacA gene, which provides either a UPR-deficient strain or a constitutively activated UPR strain for comparative analysis with its wild type. Although we focus on A. niger, the utility of the hacA-deletion strategy is also described for use in investigating the virulence of the plant pathogen Alternaria brassicicola

Hydrodynamic coupling and rotational mobilities near planar elastic membranes

We study theoretically and numerically the coupling and rotational hydrodynamic interactions between spherical particles near a planar elastic membrane that exhibits resistance towards shear and bending. Using a combination of the multipole expansion and Faxen's theorems, we express the frequency-dependent hydrodynamic mobility functions as a power series of the ratio of the particle radius to the distance from the membrane for the self mobilities, and as a power series of the ratio of the radius to the interparticle distance for the pair mobilities. In the quasi-steady limit of zero frequency, we find that the shear- and bending-related contributions to the particle mobilities may have additive or suppressive effects depending on the membrane properties in addition to the geometric configuration of the interacting particles relative to the confining membrane. To elucidate the effect and role of the change of sign observed in the particle self and pair mobilities, we consider an example involving a torque-free doublet of counterrotating particles near an elastic membrane. We find that the induced rotation rate of the doublet around its center of mass may differ in magnitude and direction depending on the membrane shear and bending properties. Near a membrane of only energetic resistance toward shear deformation, such as that of a certain type of elastic capsules, the doublet undergoes rotation of the same sense as observed near a no-slip wall. Near a membrane of only energetic resistance toward bending, such as that of a fluid vesicle, we find a reversed sense of rotation. Our analytical predictions are supplemented and compared with fully resolved boundary integral simulations where a very good agreement is obtained over the whole range of applied frequencies.Comment: 14 pages, 7 figures. Revised manuscript resubmitted to J. Chem. Phy

A Perfect Script? Manchester United's Class of ‘92

The Class of ’92 is a documentary film featuring six Manchester United F.C. players who recount their time during a pivotal period for the club, English football and English society. The documentary claims to offer a commentary on Britain in the 1990s, but appears, without acknowledging the fact, to be a promotional vehicle to establish the six men as a brand labeled the Class of ’92 (CO92). Creating this brand necessarily involved presenting a selective account of their time and places with the film being little more than an advertisement, masquerading as an observational documentary. The film draws freely upon the symbolic capital held by the club and the city of Manchester and uses the Busby Babes/Munich chapter and the more recent “Madchester scene” to forge the Class of ’92 brand by editing out those elements that did not accord with this project. The article argues that a more complete representation of ’90s Britain, while disrupting the intended narrative, would acknowledge the significant structural and commercial changes experienced by the club, the sport, and the city in the last decade of the 20th century. We suggest that the Class of ’92 invites the viewer to consider how the documentary film genre can contribute to brand development and promotion

Regional variation in life history traits and plastic responses to temperature of the major malaria vector Nyssorhynchus darlingi in Brazil

The primary Brazilian malaria vector, Nyssorhynchus darlingi (formerly Anopheles darlingi), ranges from 0°S-23°S across three biomes (Amazonia, Cerrado, Mata Atlântica). Rising temperatures will increase mosquito developmental rates, and models predict future malaria transmission by Ny. darlingi in Brazil will shift southward. We reared F1 Ny. darlingi (progeny of field-collected females from 4 state populations across Brazil) at three temperatures (20, 24, 28 °C) and measured key life-history traits. Our results reveal geographic variation due to both genetic differences among localities and plastic responses to temperature differences. Temperature significantly altered all traits: faster larval development, shorter adult life and overall lifespan, and smaller body sizes were seen at 28 °C versus 20 °C. Low-latitude Amazonia mosquitoes had the fastest larval development at all temperatures, but at 28 °C, average development rate of high-latitude Mata Atlântica mosquitoes was accelerated and equivalent to low-latitude Amazonia. Body size of adult mosquitoes from the Mata Atlântica remained larger at all temperatures. We detected genetic variation in the plastic responses among mosquitoes from different localities, with implications for malaria transmission under climate change. Faster development combined with larger body size, without a tradeoff in adult longevity, suggests vectorial capacities of some Mata Atlântica populations may significantly increase under warming climates.NIH-NIAID [1R01AI110112]; Biodefense and Emerging Infectious Disease training fellowship [T32AI05532901]Open access journalThis item from the UA Faculty Publications collection is made available by the University of Arizona with support from the University of Arizona Libraries. If you have questions, please contact us at [email protected]

Footballers' Image Rights in the New Media Age

Image rights, broadly defined as the commercial appropriation of someone's personality, including indices of their image, voice, name and signature, have become increasingly important in the political economy of media sport. A range of legal, economic and political arguments have developed in the UK as to what image rights actually are, their legal efficacy and their potential impact on developments in the long-standing relationship between sport and the media. This paper focuses on the problematic definition of the term in the UK context and how it relates to certain economic and commercial transformations in British football. Using the English Premier League and the ‘celebrity footballer’ David Beckham as its primary focus, the paper traces the rise of image rights clauses in player contracts. This process is analysed in the context of rapid and dramatic change in the media coverage of the sport. The paper focuses on the growing legal complexities of protecting star images in relation to the Internet and the wider issues of football, fandom and popular culture

The PAndAS Field of Streams: stellar structures in the Milky Way halo toward Andromeda and Triangulum

We reveal the highly structured nature of the Milky Way stellar halo within the footprint of the PAndAS photometric survey from blue main sequence and main sequence turn-off stars. We map no fewer than five stellar structures within a heliocentric range of ~5 to 30 kpc. Some of these are known (the Monoceros Ring, the Pisces/Triangulum globular cluster stream), but we also uncover three well-defined stellar structures that could be, at least partly, responsible for the so-called Triangulum/Andromeda and Triangulum/Andromeda 2 features. In particular, we trace a new faint stellar stream located at a heliocentric distance of ~17 kpc. With a surface brightness of \Sigma_V ~ 32-32.5 mag/arcsec^2, it follows an orbit that is almost parallel to the Galactic plane north of M31 and has so far eluded surveys of the Milky Way halo as these tend to steer away from regions dominated by the Galactic disk. Investigating our follow-up spectroscopic observations of PAndAS, we serendipitously uncover a radial velocity signature from stars that have colors and magnitudes compatible with the stream. From the velocity of eight likely member stars, we show that this stellar structure is dynamically cold, with an unresolved velocity dispersion that is lower than 7.1 km/s at the 90-percent confidence level. Along with the width of the stream (300-650 pc), its dynamics points to a dwarf-galaxy-accretion origin. The numerous stellar structures we can map in the Milky Way stellar halo between 5 and 30 kpc and their varying morphology is a testament to the complex nature of the stellar halo at these intermediate distances.Comment: 11 pages, 8 figures, accepted for publication in the ApJ, Figure 3 is the money plo