Total protein analysis as a reliable loading control for quantitative fluorescent Western blotting.

Western blotting has been a key technique for determining the relative expression of proteins within complex biological samples since the first publications in 1979. Recent developments in sensitive fluorescent labels, with truly quantifiable linear ranges and greater limits of detection, have allowed biologists to probe tissue specific pathways and processes with higher resolution than ever before. However, the application of quantitative Western blotting (QWB) to a range of healthy tissues and those from degenerative models has highlighted a problem with significant consequences for quantitative protein analysis: how can researchers conduct comparative expression analyses when many of the commonly used reference proteins (e.

DUX4 and DUX4 downstream target genes are expressed in fetal FSHD muscles.

Facioscapulohumeral muscular dystrophy (FSHD) is one of the most prevalent adult muscular dystrophies. The common clinical signs usually appear during the second decade of life but when the first molecular dysregulations occur is still unknown. Our aim was to determine whether molecular dysregulations can be identified during FSHD fetal muscle development.

Tight coordination of protein translation and HSF1 activation supports the anabolic malignant state.

The ribosome is centrally situated to sense metabolic states, but whether its activity, in turn, coherently rewires transcriptional responses is unknown. Here, through integrated chemical-genetic analyses, we found that a dominant transcriptional effect of blocking protein translation in cancer cells was inactivation of heat shock factor 1 (HSF1), a multifaceted transcriptional regulator of the heat-shock response and many other cellular processes essential for anabolic metabolism, cellular proliferation, and tumorigenesis. These analyses linked translational flux to the regulation of HSF1 transcriptional activity and to the modulation of energy metabolism.

The failing right ventricle in congenital heart disease.

In congenital heart disease (CHD) the right ventricle (RV) dons a variety of hats. It might be situated in the normal subpulmonary position and function as half of a 2-pump circulation, but nonetheless be subjected to abnormal loading conditions, surgical interventions, or electrical disturbance. Alternatively, it can be required to generate systemic cardiac output; either in the context of a univentricular circulation or else in concert with a subpulmonary left ventricle.

Aging-associated exacerbation in fatty degeneration and infiltration after rotator cuff tear.

Background: Rotator cuff tears are one of the most common musculoskeletal complaints and a substantial source of morbidity in elderly patients. Chronic cuff tears are associated with muscle atrophy and an infiltration of fat to the area, a condition known as "fatty degeneration." To improve the treatment of cuff tears in elderly patients, a greater understanding of the changes in the contractile properties of muscle fibers and the molecular regulation of fatty degeneration is essential.

Deregulation of the protocadherin gene FAT1 alters muscle shapes: implications for the pathogenesis of facioscapulohumeral dystrophy.

Generation of skeletal muscles with forms adapted to their function is essential for normal movement. Muscle shape is patterned by the coordinated polarity of collectively migrating myoblasts. Constitutive inactivation of the protocadherin gene Fat1 uncoupled individual myoblast polarity within chains, altering the shape of selective groups of muscles in the shoulder and face.

Dysregulation of 4q35- and muscle-specific genes in fetuses with a short D4Z4 array linked to facio-scapulo-humeral dystrophy.

Facio-scapulo-humeral dystrophy (FSHD) results from deletions in the subtelomeric macrosatellite D4Z4 array on the 4q35 region. Upregulation of the DUX4 retrogene from the last D4Z4 repeated unit is thought to underlie FSHD pathophysiology. However, no one knows what triggers muscle defect and when alteration arises.

Synthesis and biological evaluation of novel substituted pyrrolo[1,2-a]quinoxaline derivatives as inhibitors of the human protein kinase CK2.

Herein we describe the synthesis and properties of substituted phenylaminopyrrolo[1,2-a]quinoxaline-carboxylic acid derivatives as a novel class of potent inhibitors of the human protein kinase CK2. A set of 15 compounds was designed and synthesized using convenient and straightforward synthesis protocols. The compounds were tested for inhibition of human protein kinase CK2, which is a potential drug target for many diseases including inflammatory disorders and cancer.

Broken symmetries, zero-energy modes, and quantum transport in disordered graphene: from supermetallic to insulating regimes.

The role of defect-induced zero-energy modes on charge transport in graphene is investigated using Kubo and Landauer transport calculations. By tuning the density of random distributions of monovacancies either equally populating the two sublattices or exclusively located on a single sublattice, all conduction regimes are covered from direct tunneling through evanescent modes to mesoscopic transport in bulk disordered graphene. Depending on the transport measurement geometry, defect density, and broken sublattice symmetry, the Dirac-point conductivity is either exceptionally robust against disorder (supermetallic state) or suppressed through a gap opening or by algebraic localization of zero-energy modes, whereas weak localization and the Anderson insulating regime are obtained for higher energies.

Hypertension, obesity, and coronary artery disease in the survivors of congenital heart disease.

Obesity, hypertension, and coronary artery disease are prevalent in the general population and well recognized as contributors to cardiac morbidity and mortality. With surgical and medical advances, there is a growing and aging population with congenital heart disease who are also at risk of developing these comorbidities. In addition, some congenital cardiac lesions predispose patients to conditions such as hypertension or coronary artery disease.

Physical and mental illnesses: implications of similarities and differences for services and law.

It appears self-evident that psychiatry should be classified as a particular specialty within the broader field of medicine. Psychiatrists, being first and foremost doctors, have undertaken an identical basic training to their physician and surgical peers and, as in general medicine and surgery, the biomedical model is a central pillar of psychiatric practice. Within psychiatry, signs and symptoms are elicited, diagnoses made and very often physical interventions (in the form of psychotropic agents) are employed.

Relation of right ventricular mechanics to exercise tolerance in children after tetralogy of Fallot repair.

Background: Progressive right ventricular (RV) dysfunction and exercise intolerance are common problems after tetralogy of Fallot (TOF) repair. We investigated RV myocardial deformation and dyssynchrony in children after TOF repair and their association with exercise capacity.

Methods: Asymptomatic children after TOF repair were investigated by 2-dimensional speckle tracking echocardiography, magnetic resonance, and metabolic exercise study.

Differentiation of transitional zone prostate cancer from benign hyperplasia nodules: evaluation of discriminant criteria at multiparametric MRI.

Aim: To assess topographical and magnetic resonance imaging (MRI) features in characterizing prostate transitional zone (TZ) nodules.

Materials And Methods: Two radiologists evaluated all TZ nodules visible at multiparametric MRI in 52 consecutive patients who underwent radical prostatectomy. The radiologists assessed topographical (anteroposterior and superior-inferior location, crossing of the sagittal midline) and T2-weighted (shape, presence and distinctness of capsule, distinctness of contours, presence of cysts) features, the apparent diffusion coefficient (ADC), and eight semi-quantitative and quantitative enhancement parameters derived from dynamic contrast-enhanced (DCE) imaging.

Critical care physicians: attitudes, beliefs, and knowledge about pressure ulcers.

Objective: The objective of this study was to determine critical care physicians' attitudes, beliefs, and knowledge toward pressure ulcer (PrU) prevention and treatment in critical care patients.

Design: Descriptive, correlational

Participants: 56 critical care physicians

Main Outcome Measures: Survey instrument developed to collect demographic information and information regarding attitudes and beliefs about PrUs and PrU knowledge.

Results: The majority of physicians (69%) reported poor to adequate basic medical education training on PrU prevention and treatment.

Non-perturbative effects of laser illumination on the electrical properties of graphene nanoribbons.

Floquet theory combined with a realistic description of the electronic structure of illuminated graphene and graphene nanoribbons is developed to assess the emergent non-adiabatic and non-perturbative effects on the electronic properties. Here we introduce an efficient computational scheme and illustrate its use by applying it to graphene nanoribbons in the presence of both linear and circular polarization. The interplay between confinement due to the finite sample size and laser-induced transitions is shown to lead to sharp features in the average conductance and density of states.

Splitting of the zero-energy Landau level and universal dissipative conductivity at critical points in disordered graphene.

We report on robust features of the longitudinal conductivity (σ(xx)) of the graphene zero-energy Landau level in the presence of disorder and varying magnetic fields. By mixing an Anderson disorder potential with a low density of sublattice impurities, the transition from metallic to insulating states is theoretically explored as a function of Landau-level splitting, using highly efficient real-space methods to compute the Kubo conductivities (both σ(xx) and Hall σ(xy)). As long as valley degeneracy is maintained, the obtained critical conductivity σ(xx) =/~ 1.

Scaling properties of charge transport in polycrystalline graphene.

Polycrystalline graphene is a patchwork of coalescing graphene grains of varying lattice orientations and size, resulting from the chemical vapor deposition (CVD) growth at random nucleation sites on metallic substrates. The morphology of grain boundaries has become an important topic given its fundamental role in limiting the mobility of charge carriers in polycrystalline graphene, as compared to mechanically exfoliated samples. Here we report new insights to the current understanding of charge transport in polycrystalline geometries.

Diversity of trends of viremia and T-cell markers in experimental acute feline immunodeficiency virus infection.

Objective: The early events of human immunodeficiency virus infection seem critical for progression toward disease and antiretroviral therapy initiation. We wanted to clarify some still unknown prognostic relationships between inoculum size and changes in various immunological and virological markers. Feline immunodeficiency virus infection could be a helpful model.

Three-dimensional models of topological insulators: engineering of Dirac cones and robustness of the spin texture.

We design three-dimensional models of topological insulator thin films, showing a tunability of the odd number of Dirac cones driven by the atomic-scale geometry at the boundaries. A single Dirac cone at the Γ-point can be obtained as well as full suppression of quantum tunneling between Dirac states at geometrically differentiated surfaces. The spin texture of surface states changes from a spin-momentum-locking symmetry to a surface spin randomization upon the introduction of bulk disorder.

Descriptive overview of the 2011 epidemic of arboviral disease in horses in Australia.

Objective: To provide an overview and descriptive analysis of the 2011 arboviral disease epidemic in horses that involved three important Australian mosquito-borne viruses: Murray Valley encephalitis virus, West Nile virus (Kunjin strain) and Ross River virus.

Methods: Data from states affected between January and June 2011 were collated and comprised reports of horses showing signs of neuromuscular disease and the associated laboratory findings. A summary of the data is presented, together with a spatiotemporal analysis of cases and preliminary assessment of rainfall patterns and case distribution.

Proximity effects induced in graphene by magnetic insulators: first-principles calculations on spin filtering and exchange-splitting gaps.

We report on first-principles calculations of spin-dependent properties in graphene induced by its interaction with a nearby magnetic insulator (europium oxide, EuO). The magnetic proximity effect results in spin polarization of graphene π orbitals by up to 24%, together with a large exchange-splitting band gap of about 36 meV. The position of the Dirac cone is further shown to depend strongly on the graphene-EuO interlayer.

GeoWeb and crisis management: issues and perspectives of volunteered geographic information.

Mapping, and more generally geopositioning, has become ubiquitous on the Internet. This democratization of geomatics through the GeoWeb results in the emergence of a new form of mapping based on Web 2.0 technologies.

Polyphasic characterization and genetic relatedness of low-virulence and virulent Listeria monocytogenes isolates.

Background: Currently, food regulatory authorities consider all Listeria monocytogenes isolates as equally virulent. However, an increasing number of studies demonstrate extensive variations in virulence and pathogenicity of L. monocytogenes strains.

Maternal and fetal tyrosinemia type I.

A 22 year-old woman with tyrosinemia type I (HT1) married her first cousin who is heterozygous for the same FAH mutation for which the patient is homozygous. During her pregnancy she was treated with diet (prescribed tyrosine intake 300 mg/day), and nitisinone (60 mg/day). Median plasma tyrosine levels were 560 μmol/L (range: 375-838, n = 21) and nitisinone 51 μmol/L (range: 41-57, n = 3) during pregnancy.