Gene Therapy for Mitochondrial Diseases: Current Status and Future Perspective

Mitochondrial diseases (MDs) are a group of severe genetic disorders caused by mutations in the nuclear or mitochondrial genome encoding proteins involved in the oxidative phosphorylation (OXPHOS) system. MDs have a wide range of symptoms, ranging from organ-specific to multisystemic dysfunctions, with different clinical outcomes. The lack of natural history information, the limits of currently available preclinical models, and the wide range of phenotypic presentations seen in MD patients have all hampered the development of effective therapies. The growing number of pre-clinical and clinical trials over the last decade has shown that gene therapy is a viable precision medicine option for treating MD. However, several obstacles must be overcome, including vector design, targeted tissue tropism and efficient delivery, transgene expression, and immunotoxicity. This manuscript offers a comprehensive overview of the state of the art of gene therapy in MD, addressing the main challenges, the most feasible solutions, and the future perspectives of the field

The effects of fiber inclusion on pet food sensory characteristics and palatability

Citation: Koppel, K., Monti, M., Gibson, M., Alavi, S., Di Donfrancesco, B., & Carciofi, A. C. (2015). The effects of fiber inclusion on pet food sensory characteristics and palatability. Animals, 5(1), 110-125. doi:10.3390/ani5010110The objectives of this study were to determine (a) the influence of fiber on the sensory characteristics of dry dog foods; (b) differences of coated and uncoated kibbles for aroma and flavor characteristics; (c) palatability of these dry dog foods; and (d) potential associations between palatability and sensory attributes. A total of eight fiber treatments were manufactured: a control (no fiber addition), guava fiber (3%, 6%, and 12%), sugar cane fiber (9%; large and small particle size), and wheat bran fiber (32%; large and small particle size). The results indicated significant effects of fibers on both flavor and texture properties of the samples. Bitter taste and iron and stale aftertaste were examples of flavor attributes that differed with treatment, with highest intensity observed for 12% guava fiber and small particle size sugar cane fiber treatments. Fracturability and initial crispness attributes were lowest for the sugar cane fiber treatments. Flavor of all treatments changed after coating with a palatant, increasing in toasted, brothy, and grainy attributes. The coating also had a masking effect on aroma attributes such as stale, flavor attributes such as iron and bitter taste, and appearance attributes such as porosity. Palatability testing results indicated that the control treatment was preferred over the sugar cane or the wheat bran treatment. The treatment with large sugarcane fiber particles was preferred over the treatment with small particles, while both of the wheat bran treatments were eaten at a similar level. Descriptive sensory analysis data, especially textural attributes, were useful in pinpointing the underlying characteristics and were considered to be reasons that may influence palatability of dog foods manufactured with inclusion of different fibers. © 2015 by the authors; licensee MDPI, Basel, Switzerland

Optical measurements of atmospheric particles from airborne platforms: in situ and remote sensing instruments for balloons and aircrafts

Multiwavelength laser backscattersondes (MAS) have been widely used from a variety of airborne platforms for in situ measurements of optical properties of clouds and atmospheric particulate as well as their phase and composition. Recently, a new miniaturized LIDAR (MULID) has been developed using state-of-art technology for balloon borne profiling of the same quantities. A description of the two instruments, a survey of preliminary results obtained during test flights and indications for future use are given

Morphology of the tropopause layer and lower stratosphere above a tropical cyclone : a case study on cyclone Davina (1999)

During the APE-THESEO mission in the Indian Ocean the Myasishchev Design Bureau stratospheric research aircraft M55 Geophysica performed a flight over and within the inner core region of tropical cyclone Davina. Measurements of total water, water vapour, temperature, aerosol backscattering, ozone and tracers were made and are discussed here in comparison with the averages of those quantities acquired during the campaign time frame. Temperature anomalies in the tropical tropopause layer (TTL), warmer than average in the lower part and colder than average in the upper TTL were observed. Ozone was strongly reduced compared to its average value, and thick cirrus decks were present up to the cold point, sometimes topped by a layer of very dry air. Evidence for meridional transport of trace gases in the stratosphere above the cyclone was observed and perturbed water distribution in the TTL was documented. The paper discuss possible processes of dehydration induced by the cirrus forming above the cyclone, and change in the chemical tracer and water distribution in the lower stratosphere 400–430 K due to meridional transport from the mid-latitudes and link with Davina. Moreover it compares the data prior and after the cyclone passage to discuss its actual impact on the atmospheric chemistry and thermodynamics

Differences in Arctic and Antarctic PSC occurrence as observed by lidar in Ny-Ålesund (79° N, 12° E) and McMurdo (78° S, 167° E)

International audienceThe extent of springtime Arctic ozone loss does not reach Antarctic "ozone hole" dimensions because of the generally higher temperatures in the northern hemisphere vortex and consequent less polar stratospheric cloud (PSC) particle surface for heterogeneous chlorine activation. Yet, with increasing greenhouse gases stratospheric temperatures are expected to further decrease. To infer if present Antarctic PSC occurrence can be applied to predict future Arctic PSC occurrence, lidar observations from McMurdo station (78° S, 167° E) and Ny-Ålesund (79° N, 12° E) have been analysed for the 9 winters between 1995 (1995/1996) and 2003 (2003/2004). Although the statistics may not completely cover the overall hemispheric PSC occurrence, the observations are considered to represent the main synoptic cloud features as both stations are mostly situated in the centre or at the inner edge of the vortex. Since the focus is set on the occurrence frequency of solid and liquid particles, the analysis has been restricted to volcanic aerosol free conditions. In McMurdo, by far the largest part of PSC observations is associated with PSC type Ia. The observed constant background of NAT particles and their potential ability to cause denoxification and irreversible denitrification is presumably more important to Antarctic ozone chemistry than the scarcely observed PSC type II. Meanwhile in Ny-Ålesund, PSC type II has never been observed, while type Ia and Ib both occur in large fraction. Although they are also found solely, the majority of observations reveals solid and liquid particle layers in the same profile. For the Ny-Ålesund measurements, the frequent occurrence of liquid PSC particles yields major significance in terms of ozone chemistry, as their chlorine activation rates are more efficient. The relationship between temperature, PSC formation, and denitrification is nonlinear and the McMurdo and Ny-Ålesund PSC observations imply that for predicted stratospheric cooling it is not possible to directly apply current Antarctic PSC occurrence directly to the Arctic stratosphere. Future Arctic PSC occurrence, and thus ozone loss, will depend on the shape and barotropy of the vortex rather than on the minimum temperatures

The AMMA mulid network for aerosol characterization in West Africa

Three ground based portable low power consumption microlidars (MULID) have been built and deployed at three remote sites in Banizoumbou (Niger), Cinzana (Mali) and M'Bour (Senegal) in the framework of the African Monsoon Multidisciplinary Analyses (AMMA) project for the characterization of aerosols optical properties. A description of the instrument and a discussion of the data inversion method, including a careful analysis of measurement uncertainties (systematic and statistical errors) are presented. Some case studies of typical lidar profiles observed over the Banizoumbou site during 2006 are shown and discussed with respect to the AERONET 7-day back-trajectories and the biomass burning emissions from the Combustion Emission database for the AMMA campaign

Food Leftover Practices among Consumers in Selected Countries in Europe, South and North America

Citation: Koppel, K., Higa, F., Godwin, S., Gutierrez, N., Shalimov, R., Cardinal, P., . . . Chambers, E. (2016). Food Leftover Practices among Consumers in Selected Countries in Europe, South and North America. Foods, 5(3), 14. doi:10.3390/foods5030066Foodborne illnesses may be related to many food production factors with home practices of consumers playing an important role in food safety. Consumer behavior for handling food leftovers has been studied, however little work on comparisons among countries has been published. The objective of this study was to investigate home food leftover practices of people from North American, South American, and European countries. Surveys were conducted with approximately 100 or more consumers in Argentina, Colombia, the United States, Estonia, Italy, Russia, and Spain. The participants responded to questions related to the length of time different types of food leftovers; such as meat, fresh salads, or restaurant dishes would be kept refrigerated or would be left at room temperature before refrigeration. Researchers also investigated how consumers would determine if the food was still safe for consumption. Potentially risky behaviors were observed in all seven countries. For instance, 55.8% of Estonians, 25% of Russians and 25.8% of Argentinean participants left food out at room temperature for several hours before storing in the refrigerator. Furthermore, 25%-29% of Colombian, Estonian, and Spanish consumers would look, smell, and taste leftovers to determine its probable safety. Correct handling of leftovers is an important aspect of consumer food safety. Although the surveys cannot be representative of all consumers in each country, they do provide an initial overview of comparative practices for handling leftovers among different countries. This provides government and educators with information on potential universal and unique consumer food safety issues related to handling leftover foods among various countries

Biocompatibility and antibiofilm properties of calcium silicate-based cements: An in vitro evaluation and report of two clinical cases

Calcium silicate-based cements have reached excellent levels of performance in endodon-tics, providing predictable and successful results. To better assess the properties of these bioactive materials, the present study aimed to compare the biocompatibility and antibiofilm properties of ProRoot MTA and Biodentine. Human osteogenic sarcoma (Saos-2) cells were cultured on ProRoot MTA and Biodentine samples or in the presence of both cement extracts. Cell viability assay, meas-urement of reactive oxygen species (ROS), immunofluorescence analysis, as well as morphological evaluations were conducted. Moreover, Streptococcus mutans was used to assess the biofilm forming ability on ProRoot MTA and Biodentine disks. Finally, both cements were applied in vivo to treat immature permanent teeth affected by reversible pulpitis. Results: Cell viability assay demonstrated that Saos-2 cells had a dose-and time-dependent cytotoxicity to both analyzed cements, although cells exposed to ProRoot MTA showed a better cell vitality than those exposed to Biodentine (p < 0.001). Both cements demonstrated ROS production while this was greater in the case of Biodentine than ProRoot MTA (p < 0.001). Immunofluorescence images of the cytoskeleton and focal adhesions showed no differences in Saos-2 cells grown in the presence of ProRoot MTA eluate; whereas in the Biodentine groups, cells showed a morphology and focal adhesions more similar to that of the control sample, as the eluate concentration decreased. Morphological analysis revealed that Saos-2 cells were more flattened and exhibited better spreading when attached to ProRoot MTA disks than to Biodentine ones. The antibiofilm properties showed a time-dependent powerful inhibition of S. mutans superficial colonization and an antibiofilm effect of both cements. Clinically, complete root formation of the treated elements was achieved using the two studied cements, showing stable results over time. ProRoot MTA and Biodentine was demonstrated to be biocompatible and to possess antibiofilm properties. Their clinical application in vital pulp therapy provided successful outcomes after 2 years of follow-up

Comparison of vertical aerosol extinction coefficients from in-situ and LIDAR measurements

Vertical profiles of aerosol optical properties were explored in a case study near the San Pietro Capofiume (SPC) ground station during the PEGASOS Po Valley campaign in the summer of 2012. A Zeppelin NT airship was employed to investigate the effect of the dynamics of the planetary boundary layer at altitudes between ~ 50–800 m above ground. Determined properties included the aerosol size distribution, the hygroscopic growth factor, the effective index of refraction and the light absorption coefficient. The first three parameters were used to retrieve the light scattering coefficient. Simultaneously, direct measurements of both the scattering and absorption coefficient were carried out at the SPC ground station. Additionally, a LIDAR system provided aerosol extinction coefficients for a vertically resolved comparison between in-situ and remote sensing results. First, the airborne results at low altitudes were validated with the ground measurements. Agreement within approximately ±25 and ±20% was found for the dry scattering and absorption coefficient, respectively. The single scattering albedo, ranged between 0.83 to 0.95, indicating the importance of the absorbing particles in the Po Valley region. A clear layering of the atmosphere was observed during the beginning of the flight (until ~ 10 local time) before the mixed layer (ML) was fully developed. Highest extinction coefficients were found at low altitudes, in the new ML, while values in the residual layer, which could be probed at the beginning of the flight at elevated altitudes, were lower. At the end of the flight (after ~ 12 local time) the ML was fully developed, resulting in constant extinction coefficients at all altitudes measured on the Zeppelin NT. LIDAR results captured these dynamic features well and good agreement was found for the extinction coefficients compared to the in-situ results, using fixed LIDAR ratios (LR) between 30 and 70 sr for the altitudes probed with the Zeppelin. These LR are consistent with values for continental aerosol particles that can be expected in this region

Food Leftover Practices among Consumers in Selected Countries in Europe, South and North America

Foodborne illnesses may be related to many food production factors with home practices of consumers playing an important role in food safety. Consumer behavior for handling food leftovers has been studied, however little work on comparisons among countries has been published. The objective of this study was to investigate home food leftover practices of people from North American, South American, and European countries. Surveys were conducted with approximately 100 or more consumers in Argentina, Colombia, the United States, Estonia, Italy, Russia, and Spain. The participants responded to questions related to the length of time different types of food leftovers; such as meat, fresh salads, or restaurant dishes would be kept refrigerated or would be left at room temperature before refrigeration. Researchers also investigated how consumers would determine if the food was still safe for consumption. Potentially risky behaviors were observed in all seven countries. For instance, 55.8% of Estonians, 25% of Russians and 25.8% of Argentinean participants left food out at room temperature for several hours before storing in the refrigerator. Furthermore, 25%–29% of Colombian, Estonian, and Spanish consumers would look, smell, and taste leftovers to determine its probable safety. Correct handling of leftovers is an important aspect of consumer food safety. Although the surveys cannot be representative of all consumers in each country, they do provide an initial overview of comparative practices for handling leftovers among different countries. This provides government and educators with information on potential universal and unique consumer food safety issues related to handling leftover foods among various countries