Progressive Structural Defects in Canine Centronuclear Myopathy Indicate a Role for HACD1 in Maintaining Skeletal Muscle Membrane Systems

Mutations in HACD1/PTPLA cause recessive congenital myopathies in humans and dogs. Hydroxyacyl-coA dehydratases are required for elongation of very long chain fatty acids, and HACD1 has a role in early myogenesis, but the functions of this striated muscle-specific enzyme in more differentiated skeletal muscle remain unknown. Canine HACD1 deficiency is histopathologically classified as a centronuclear myopathy (CNM). We investigated the hypothesis that muscle from HACD1-deficient dogs has membrane abnormalities in common with CNMs with different genetic causes. We found progressive changes in tubuloreticular and sarcolemmal membranes and mislocalized triads and mitochondria in skeletal muscle from animals deficient in HACD1. Furthermore, comparable membranous abnormalities in cultured HACD1-deficient myotubes provide additional evidence that these defects are a primary consequence of altered HACD1 expression. Our novel findings, including T-tubule dilatation and disorganization, associated with defects in this additional CNM-associated gene provide a definitive pathophysiologic link with these disorders, confirm that dogs deficient in HACD1 are relevant models, and strengthen the evidence for a unifying pathogenesis in CNMs via defective membrane trafficking and excitation-contraction coupling in muscle. These results build on previous work by determining further functional roles of HACD1 in muscle and provide new insight into the pathology and pathogenetic mechanisms of HACD1 CNM. Consequently, alterations in membrane properties associated with HACD1 mutations should be investigated in humans with related phenotypes

Planning for Sustainability in Small Municipalities: The Influence of Interest Groups, Growth Patterns, and Institutional Characteristics

How and why small municipalities promote sustainability through planning efforts is poorly understood. We analyzed ordinances in 451 Maine municipalities and tested theories of policy adoption using regression analysis.We found that smaller communities do adopt programs that contribute to sustainability relevant to their scale and context. In line with the political market theory, we found that municipalities with strong environmental interests, higher growth, and more formal governments were more likely to adopt these policies. Consideration of context and capacity in planning for sustainability will help planners better identify and benefit from collaboration, training, and outreach opportunities

Insight Into the biological effects of non-ionizing Radiation through the properties of the electromagnetic waves

The widespread wireless technology initiated several decades ago, has been gradually occupying nearly all daily activities of the modern society. The major sources of this non-ionizing radiation (NIR) include cell phones (6 billion users worldwide), mobile phone base stations (thousands in a crowded city), FM and TV broadcast stations, wireless phones, Wi-Fi routers and units in iphones, i-pads, notebooks, laptops. All these sources comprise a frequency spectrum from 87 MHz (FM stations) to 2.5 GHz (Wi-Fi, blue-tooth, Microwave oven). We consider that in order to study the biological/human effects of NIR, it is necessary to know exactly the radiation source properties (single frequency or multiple frequencies, repetitive or discontinuous emission and precise knowledge of the peak and average values of the electromagnetic wave in each case). Given the controversy of the existing so far published data, we intend to explore the issue of different biological effectiveness from simple exposure (CW emission) to the more complex pulsed radiation using two major model systems; mice and insects. So far, we have shown, in mice, memory impairment, stress induction and brain protein expression changes and in insects, fecundity decrease and apoptotic cell death increase following microwave (MW) radiation. In addition, by using the NARDA SRM 3000 spectrum analyzer we have performed a mapping of frequencies and E field intensities near base stations and other radiation sources. In this study, we initially attempted to study biological effects on flies from environmental exposure, in Athens University campus region (Dept. of Biology building), to non ionizing radiation in the range of 87 MHz to 2.5 GHz containing FM stations, TV broadcast stations, GSM 900, GSM 1800 MHz and UMTS 2100 MHz. A control group of flies was present in the same area inside a custom made Faraday type cage.Our results under these conditions indicate: a) decrease on Drosophila melanogaster reproduction and b) increase in apoptotic cell death during oogenesis, although at a lower degree compared to mobile phone signals. The work shall be continued with more frequencies and modulation schemes in insects and afterwards in mice where other parameters shall be examine

A review of the distribution of particulate trace elements in urban terrestrial environments and its application to considerations of risk

We review the evolution, state of the art and future lines of research on the sources, transport pathways, and sinks of particulate trace elements in urban terrestrial environments to include the atmosphere, soils, and street and indoor dusts. Such studies reveal reductions in the emissions of some elements of historical concern such as Pb, with interest consequently focusing on other toxic trace elements such as As, Cd, Hg, Zn, and Cu. While establishment of levels of these elements is important in assessing the potential impacts of human society on the urban environment, it is also necessary to apply this knowledge in conjunction with information on the toxicity of those trace elements and the degree of exposure of human receptors to an assessment of whether such contamination represents a real risk to the city’s inhabitants and therefore how this risk can be addressed

ILC3 function as a double-edged sword in inflammatory bowel diseases

Inflammatory bowel diseases (IBD), composed mainly of Crohn’s disease (CD) and ulcerative colitis (UC), are strongly implicated in the development of intestinal inflammation lesions. Its exact etiology and pathogenesis are still undetermined. Recently accumulating evidence supports that group 3 innate lymphoid cells (ILC3) are responsible for gastrointestinal mucosal homeostasis through moderate generation of IL-22, IL-17, and GM-CSF in the physiological state. ILC3 contribute to the progression and aggravation of IBD while both IL-22 and IL-17, along with IFN-γ, are overexpressed by the dysregulation of NCR− ILC3 or NCR+ ILC3 function and the bias of NCR+ ILC3 towards ILC1 as well as regulatory ILC dysfunction in the pathological state. Herein, we feature the group 3 innate lymphoid cells’ development, biological function, maintenance of gut homeostasis, mediation of IBD occurrence, and potential application to IBD therapy

Knee pathology before and after SARS-CoV-2 pandemic: An analysis of 1139 patients

Background: The SARS-CoV-2 pandemic drastically changed daily life activities and medical practice, leading to a reorganization of healthcare activities. People spent two months in home-isolation, changing their daily habits and undertaking a more sedentary lifestyle. Change in lifestyle is related to important consequences in knee pathologies. The aim of this study was to evaluate the outpatient activity for knee pathologies before and after lockdown in terms of incidence, severity, diagnosis, and treatment. Methods: Medical records of patients with knee pathology in outpatient follow-up at IRCCS Istituto Ortopedico Galeazzi in Milan (Italy) were analyzed in the time frame 4 May–4 September 2020 and compared with patients examined between 4 May and 4 September 2019. Results: A significant increase of knee diagnoses associated to patellofemoral disorders in 2020 was found (p = 0.004). In addition, physiotherapy was significantly more prescribed in 2020 than in 2019 (p = 0.012). Conclusions: The SARS-CoV-2 pandemic lockdown did not drastically change knee pathology, but it may have had an impact on it, highlighting a summary worsening of patellofem-oral disorders associated with other knee diagnoses. Further studies are required to validate this result

Biogeochemical and oceanographic conditions provide insights about current status of an Antarctic fjord affected by relatively slow glacial retreat

Understand the origin, transport, and character of organic matter entering Antarctic fjords is essential as they are major components of the global carbon cycle and budget. Macromolecular pools of particulate organic matter, bulk organic geochemistry, major and trace elements in surface sediments from Collins Bay were analysed as source indicators. Oceanographic conditions, bathymetry (multibeam) and grain size were considered as environmental controlling factors. Sediment samples were taken with a van Veen grab, during the ANTAR XXV Peruvian expedition (February 2018), onboard the R/V “BAP Carrasco” from the Peruvian Navy. Biopolymeric composition revealed the predominance of fresh marine protein-rich organic matter in the seafloor of Collins Bay, denoting high quality food resource for marine benthic heterotrophs. Based on Igeo values (between 0 and 1) Collins Bay can be considered unpolluted with natural levels of As, Cd, Cr, Cu, Fe, Mn, Ni, Pb and Zn. Distribution of most of these elements with a gradient of decrease from the shallow inner fjord towards the outer deepest fjord, suggest their association with the deposition of detrital material and lithogenic particles supplied by Collins Glacier frontal ablation and runoff. This first comprehensive baseline information would assist in interpreting downcore sedimentary reconstructions and future climateinduce changes

Late snowfall and blizzards in Moldavia. April 2017 summary case of study

While these are normal phenomena for Romania’s geographical position, heavy snows and intense blizzards can have severely negative effects, especially when they occur unseasonably, before or after the typical period. The blizzard recorded on 19-20 April 2017 was particularly violent and one of the latest blizzard events in the history of meteorological observations. In comparison, the last significant late winter event, albeit lower in intensity, was recorded on 15-16 April 1996. However, the blizzard formation mechanism in the month of April in eastern regions is relatively similar. The presence of a high-pressure zone in the central-western European region led, in both cases, to fast large-scale penetrations of arctic air from high latitudes towards areas with high cyclogenetic potential in the Mediterranean Sea basin. Thus, the depressions of Mediterranean origin, which formed in the Genova Gulf area, advanced towards the Black Sea basin via trans-Balkan trajectories, where they regenerated and resulted in strong windintensification and abundant precipitation in the Moldavian region. With regard to the event this study analyses, the high thermobaric contrast and the presence of a sufficiently cold air mass favored, during the night between the 19th and the 20th of April, the occurrence of a strong blizzard event in Moldavia, following abundant wet snowfall during the day. At several meteorological stations across Moldavia and Transylvania, as well as in mountainous areas, the maximum winter snow layer thickness was exceeded on the 20th and 21st of April

Epidemiology and pattern of resistance of gram-negative bacteria isolated from blood samples in hospitalized patients: A single center retrospective analysis from southern italy

Background: Blood culturing remains the mainstream tool to inform an appropriate treatment in hospital-acquired bloodstream infections and to diagnose any bacteremia. Methods: A retrospective investigation on the prevalence of Gram-negative bacteria (GNB) and their resistance in hospitalized patients by age, sex, and units from blood cultures (BCs) was conducted from January 2018 to April 2020 at Sant’Elia hospital, Caltanissetta, southern Italy. We divided the patient age range into four equal intervals. Results: Multivariate demographic and microbiological variables did not show an association between bacteria distributions and gender and age. The distribution by units showed a higher prevalence of Klebsiella pneumoniae and Acinetobacter baumannii in the intensive care unit (ICU) and Escherichia coli in the non-intensive care units (non-ICUs). The analysis of antibiotic resistance showed that E. coli was susceptible to a large class of antibiotics such as carbapenem and trimethoprim-sulfamethoxazole. K. pneumoniae showed a significant susceptibility to colistin, tigecycline, and trimethoprim-sulfamethoxazole. From the survival analysis, patients with E. coli had a higher survival rate. Conclusions: The authors stress the importance of the implementation of large community-level programs to prevent E. coli bacteremia. K. pneumoniae and E. coli susceptibility patterns to antibiotics, including in the prescription patterns of general practitioners, suggest that the local surveillance and implementation of educational programs remain essential measures to slow down the spread of resistance and, consequently, increase the antibiotic lifespan

Ti-Zr-Si-Nb nanocrystalline alloys and metallic glasses: Assessment on the structure, thermal stability, corrosion and mechanical properties

The development of novel Ti-based amorphous or \u3b2-phase nanostructured metallic materials could have significant benefits for implant applications, due to improved corrosion and mechanical characteristics (lower Young's modulus, better wear performance, improved fracture toughness) in comparison to the standardized \u3b1+\u3b2 titanium alloys. Moreover, the devitrification phenomenon, occurring during heating, could contribute to lower input power during additive manufacturing technologies. Ti-based alloy ribbons were obtained by melt-spinning, considering the ultra-fast cooling rates this method can provide. The titanium alloys contain in various proportions Zr, Nb, and Si (Ti60Zr10Si15Nb15, Ti64Zr10Si15Nb11, Ti56Zr10Si15Nb19) in various proportions. These elements were chosen due to their reported biological safety, as in the case of Zr and Nb, and the metallic glass-forming ability and biocompatibility of Si. The morphology and chemical composition were analyzed by scanning electron microscopy and energy-dispersive X-ray spectroscopy, while the structural features (crystallinity, phase attribution after devitrification (after heat treatment)) were assessed by X-ray diffraction. Some of the mechanical properties (hardness, Young's modulus) were assessed by instrumented indentation. The thermal stability and crystallization temperatures were measured by differential thermal analysis. High-intensity exothermal peaks were observed during heating of melt-spun ribbons. The corrosion behavior was assessed by electrocorrosion tests. The results show the potential of these alloys to be used as materials for biomedical applications