Proteomics identification of differentially expressed proteins in the muscle of dysferlin myopathy patients

The muscular dystrophies are a large and heterogeneous group of neuromuscular disorders that can be classified according to the mode of inheritance, the clinical phenotype and the molecular defect. To better understand the pathological mechanisms of dysferlin myopathy we compared the protein-expression pattern in the muscle biopsies of six patients with this disease with six patients with limb girdle muscular dystrophy 2A, five with facioscapulohumeral dystrophy and six normal control subjects. To investigate differences in the expression levels of skeletal muscle proteins we used 2-DE and MS. Western blot or immunohistochemistry confirmed relevant results. The study showed specific increase expression of proteins involved in fast-to-slow fiber type conversion (ankyrin repeat protein 2), type I predominance (phosphorylated forms of slow troponin T), sarcomere stabilization (actinin-associated LIM protein), protein ubiquitination (TRIM 72) and skeletal muscle differentiation (Rho-GDP-dissociation inhibitor ly-GDI) in dysferlin myopathy. As anticipated, we also found differential expression of proteins common to all the muscular dystrophies studied. This comparative proteomic analysis suggests that in dysferlin myopathy (i) the type I fiber predominance is an active process of fiber type conversion rather than a selective loss of type II fibers and (ii) the dysregulation of proteins involved in muscle differentiation further confirms the role of dysferlin in this process.     

Relating instance hardness to classification performance in a dataset: a visual approach

Machine Learning - Machine Learning studies often involve a series of computational experiments in which the predictive performance of multiple models are compared across one or more datasets. The...     

Scavenging of reactive oxygen and nitrogen species with nanomaterials

Reactive oxygen and nitrogen species (RONS) are essential for normal physiological processes and play important roles in cell signaling, immunity, and tissue homeostasis. However, excess radical species are implicated in the development and augmented pathogenesis of various diseases. Several antioxidants may restore the chemical balance, but their use is limited by disappointing results of clinical trials. Nanoparticles are an attractive therapeutic alternative because they can change the biodistribution profile of antioxidants, and possess intrinsic ability to scavenge RONS. Herein, we review the types of RONS, how they are implicated in several diseases, and the types of nanoparticles with inherent antioxidant capability, their mechanisms of action, and their biological applications.     

Acoustic evaluation and adjustment of an open-plan office through architectural design and noise control

Arranging office space into a single open room offers advantages in terms of easy exchange of information and interaction among coworkers, but reduces privacy and acoustic comfort. Thus, the purpose of this work was to evaluate the acoustic quality of a real open-plan office and to propose changes in the room to improve the acoustic conditioning of this office. The computational model of the office under study was calibrated based on RT and STI measurements. Predictions were made of the RT and STI, which generated the radius of distraction r(D), and the rate of spatial decay of sound pressure levels per distance doubling DL(2) in the real conditions of the office and after modifications of the room. The insertion of dividers between work stations and an increase in the ceiling's sound absorption improved the acoustic conditions in the office under study.     

Effects of ozonized water and heat treatment on the papaya fruit epidermis

This study examined the effects of O₃ and hot water treatments on the epidermis of Golden papaya fruit. Heat treatments were applied in a hot water brushing (HWB) system. Papayas were brushed under a pressurized hot water rinse stage at 45, 55 and 65 °C for 60 s. In the HWB treatment, 4 ppm ozone was applied to the papayas for 1 or 2 min. The results show that ozone applications did not affect the fruit's cuticular surface, while heat treatments allowed natural fissures on the fruit epidermis to recover. Several crystalloid forms were identified on the epidermis of the papayas after the heat treatments. The predominant crystalloid forms on papayas are tubular and there is a positive response to temperature; the higher the temperature, the larger and more frequent the tubular crystalloids.     

Influence of ring-type internals on the solids residence time distribution in the fuel reactor of a dual circulating fluidized bed system for chemical looping combustion

The intensification of gas-solids contact in the fuel reactor of a chemical looping combustion system is enhanced with the installation of ring-type internals. This can be a key issue for achieving the necessary fuel conversion rates. Wedged rings, previously designed and tested, were found to increase the particle concentration in the counter current section of the fuel reactor and hence, to achieve a more homogeneous particles concentration along this zone. The present work investigates the effect of the mentioned internals on the residence time distribution of particles in the fuel reactor of a dual circulating fluidized bed system for chemical looping. The study was carried out in a cold flow model especially designed for the fluid-dynamic analysis of the system equipped with a recently developed residence time measurement device based on the detection of ferromagnetic tracer particles through inductance measurement. Ring internals proved the positive effect on the particles residence time, the residence time distribution is more symmetric and shows lower dispersion, the flow pattern is more plug-flow-like, these effects are intensified with the reduction of the aperture ratio of the rings. On the other hand, the upward particle transport in the counter-current zone of the fuel reactor also increases with the installation of the rings, increasing the bypass flow of solids through the fuel reactor's return loop (internal circulation). For high internal circulation rates the solids residence time distribution of the fuel reactor is dominated by the bypass effect. The findings may be used for focused design improvement of the investigated fluidized bed system.     

Usability evaluation methods for mobile serious games applied to health: a systematic review

Universal Access in the Information Society - This paper aims to present the results of a systematic review focused on usability evaluation methods for serious games (SG) of mobile devices...     

Synthetic Modular Antibody Construction by Using the SpyTag/SpyCatcher Protein-Ligase System

Efforts to engineer recombinant antibodies for specific diagnostic and therapy applications are time consuming and expensive, as each new recombinant antibody needs to be optimized for expression, stability, bio-distribution, and pharmacokinetics. We have developed a new way to construct recombinant antibody-like “devices” by using a bottom-up approach to build them from well-behaved discrete recombinant antibody domains or “parts”. Studies on antibody structure and function have identified antibody constant and variable domains with specific functions that can be expressed in isolation. We used the SpyTag/SpyCatcher protein ligase to join these parts together, thereby creating devices with desired properties based on summed properties of parts and in configurations that cannot be obtained by using genetic engineering. This strategy will create optimized recombinant antibody devices at reduced costs and with shortened development times.     

Metabolomics of Genetically Modified Crops

Metabolomic-based approaches are increasingly applied to analyse genetically modified organisms (GMOs) making it possible to obtain broader and deeper information on the composition of GMOs compared to that obtained from traditional analytical approaches. The combination in metabolomics of advanced analytical methods and bioinformatics tools provides wide chemical compositional data that contributes to corroborate (or not) the substantial equivalence and occurrence of unintended changes resulting from genetic transformation. This review provides insight into recent progress in metabolomics studies on transgenic crops focusing mainly in papers published in the last decade.