Развитие экстремального туризма в Крыму

Целью данной работы является на основе географического анализа факторов становления и особенностей развития экстремального туризма в Крыму разработать рекомендации по усовершенствованию данной отрасли туристской деятельности для создания привлекательного образа Крыма на международной арене

Novel mutations expand the clinical spectrum of DYNC1H1-associated spinal muscular atrophy

OBJECTIVE To expand the clinical phenotype of autosomal dominant congenital spinal muscular atrophy with lower extremity predominance (SMA-LED) due to mutations in the dynein, cytoplasmic 1, heavy chain 1 (DYNC1H1) gene. METHODS Patients with a phenotype suggestive of a motor, non-length-dependent neuronopathy predominantly affecting the lower limbs were identified at participating neuromuscular centers and referred for targeted sequencing of DYNC1H1. RESULTS We report a cohort of 30 cases of SMA-LED from 16 families, carrying mutations in the tail and motor domains of DYNC1H1, including 10 novel mutations. These patients are characterized by congenital or childhood-onset lower limb wasting and weakness frequently associated with cognitive impairment. The clinical severity is variable, ranging from generalized arthrogryposis and inability to ambulate to exclusive and mild lower limb weakness. In many individuals with cognitive impairment (9/30 had cognitive impairment) who underwent brain MRI, there was an underlying structural malformation resulting in polymicrogyric appearance. The lower limb muscle MRI shows a distinctive pattern suggestive of denervation characterized by sparing and relative hypertrophy of the adductor longus and semitendinosus muscles at the thigh level, and diffuse involvement with relative sparing of the anterior-medial muscles at the calf level. Proximal muscle histopathology did not always show classic neurogenic features. CONCLUSION Our report expands the clinical spectrum of DYNC1H1-related SMA-LED to include generalized arthrogryposis. In addition, we report that the neurogenic peripheral pathology and the CNS neuronal migration defects are often associated, reinforcing the importance of DYNC1H1 in both central and peripheral neuronal functions

Sensitivity of labile carbon fractions to tillage and organic matter management and their potential as comprehensive soil quality indicators across pedoclimatic conditions in Europe

Soil quality is defined as the capacity of the soil to perform multiple functions, and can be assessed by measuring soil chemical, physical and biological parameters. Among soil parameters, labile organic carbon is considered to have a primary role in many soil functions related to productivity and environmental resilience. Our study aimed at assessing the suitability of different labile carbon fractions, namely dissolved organic carbon (DOC), hydrophilic DOC (Hy-DOC), permanganate oxidizable carbon (POXC, also referred to as Active Carbon), hot water extractable carbon (HWEC) and particulate organic matter carbon (POMC) as soil quality indicators in agricultural systems. To do so, we tested their sensitivity to two agricultural management factors (tillage and organic matter input) in 10 European long-term field experiments (LTEs), and we assessed the correlation of the different labile carbon fractions with physical, chemical and biological soil quality indicators linked to soil functions. We found that reduced tillage and high organic matter input increase concentrations of labile carbon fractions in soil compared to conventional tillage and low organic matter addition, respectively. POXC and POMC were the most sensitive fractions to both tillage and fertilization across the 10 European LTEs. In addition, POXC was the labile carbon fraction most positively correlated with soil chemical (total organic carbon, total nitrogen, and cation exchange capacity), physical (water stable aggregates, water holding capacity, bulk density) and biological soil quality indicators (microbial biomass carbon and nitrogen, and soil respiration). We conclude that POXC represents a labile carbon fraction sensitive to soil management and that is the most informative about total soil organic matter, nutrients, soil structure, and microbial pools and activity, parameters commonly used as indicators of various soil functions, such as C sequestration, nutrient cycling, soil structure formation and soil as a habitat for biodiversity. Moreover, POXC measurement is relatively cheap, fast and easy. Therefore, we suggest measuring POXC as the labile carbon fraction in soil quality assessment schemes in addition to other valuable soil quality indicators.</p

Behind the Scenes:The Two-Weeks Stay of Beamline for Schools Winning Students at DESY

In a previous paper1 the BL4S science competition has been presented from the technical point of view. This paper focuses on the organizational aspects of the period that the winning teams spent at DESY to perform their experiments. Together with a description of the event, this paper provides the unique point of view of the participants

Altered mitochondrial metabolism in the insulin-resistant heart.

Obesity-induced insulin resistance and type 2 diabetes mellitus can ultimately result in various complications, including diabetic cardiomyopathy. In this case, cardiac dysfunction is characterized by metabolic disturbances such as impaired glucose oxidation and an increased reliance on fatty acid (FA) oxidation. Mitochondrial dysfunction has often been associated with the altered metabolic function in the diabetic heart, and may result from FA-induced lipotoxicity and uncoupling of oxidative phosphorylation. In this review, we address the metabolic changes in the diabetic heart, focusing on the loss of metabolic flexibility and cardiac mitochondrial function. We consider the alterations observed in mitochondrial substrate utilization, bioenergetics and dynamics, and highlight new areas of research which may improve our understanding of the cause and effect of cardiac mitochondrial dysfunction in diabetes. Finally, we explore how lifestyle (nutrition and exercise) and pharmacological interventions can prevent and treat metabolic and mitochondrial dysfunction in diabetes.COST Action MitoEAGL

Soil Quality - a critical review

Sampling and analysis or visual examination of soil to assess its status and use potential is widely practiced from plot to national scales. However, the choice of relevant soil attributes and interpretation of measurements are not straightforward, because of the complexity and site-specificity of soils, legacy effects of previous land use, and trade-offs between ecosystem services. Here we review soil quality and related concepts, in terms of definition, assessment approaches, and indicator selection and interpretation. We identify the most frequently used soil quality indicators under agricultural land use. We find that explicit evaluation of soil quality with respect to specific soil threats, soil functions and ecosystem services has rarely been implemented, and few approaches provide clear interpretation schemes of measured indicator values. This limits their adoption by land managers as well as policy. We also consider novel indicators that address currently neglected though important soil properties and processes, and we list the crucial steps in the development of a soil quality assessment procedure that is scientifically sound and supports management and policy decisions that account for the multi-functionality of soil. This requires the involvement of the pertinent actors, stakeholders and end-users to a much larger degree than practiced to date

Effects of agricultural management practices on soil quality : A review of long-term experiments for Europe and China

In this paper we present effects of four paired agricultural management practices (organic matter (OM) addition versus no organic matter input, no-tillage (NT) versus conventional tillage, crop rotation versus monoculture, and organic agriculture versus conventional agriculture) on five key soil quality indicators, i.e., soil organic matter (SOM) content, pH, aggregate stability, earthworms (numbers) and crop yield. We have considered organic matter addition, no-tillage, crop rotation and organic agriculture as “promising practices”; no organic matter input, conventional tillage, monoculture and conventional farming were taken as the respective references or “standard practice” (baseline). Relative effects were analysed through indicator response ratio (RR) under each paired practice. For this we considered data of 30 long-term experiments collected from 13 case study sites in Europe and China as collated in the framework of the EU-China funded iSQAPER project. These were complemented with data from 42 long-term experiments across China and 402 observations of long-term trials published in the literature. Out of these, we only considered experiments covering at least five years. The results show that OM addition favourably affected all the indicators under consideration. The most favourable effect was reported on earthworm numbers, followed by yield, SOM content and soil aggregate stability. For pH, effects depended on soil type; OM input favourably affected the pH of acidic soils, whereas no clear trend was observed under NT. NT generally led to increased aggregate stability and greater SOM content in upper soil horizons. However, the magnitude of the relative effects varied, e.g. with soil texture. No-tillage practices enhanced earthworm populations, but not where herbicides or pesticides were applied to combat weeds and pests. Overall, in this review, yield slightly decreased under NT. Crop rotation had a positive effect on SOM content and yield; rotation with ley very positively influenced earthworms’ numbers. Overall, crop rotation had little impact on soil pH and aggregate stability − depending on the type of intercrop; alternatively, rotation of arable crops only resulted in adverse effects. A clear positive trend was observed for earthworm abundance under organic agriculture. Further, organic agriculture generally resulted in increased aggregate stability and greater SOM content. Overall, no clear trend was found for pH; a decrease in yield was observed under organic agriculture in this review

Biomarkers of Dietary Omega-6 Fatty Acids and Incident Cardiovascular Disease and Mortality: An Individual-Level Pooled Analysis of 30 Cohort Studies

BACKGROUND: Global dietary recommendations for and cardiovascular effects of linoleic acid, the major dietary omega-6 fatty acid, and its major metabolite, arachidonic acid, remain controversial. To address this uncertainty and inform international recommendations, we evaluated how in vivo circulating and tissue levels of linoleic acid (LA) and arachidonic acid (AA) relate to incident cardiovascular disease (CVD) across multiple international studies. METHODS: We performed harmonized, de novo, individual-level analyses in a global consortium of 30 prospective observational studies from 13 countries. Multivariable-adjusted associations of circulating and adipose tissue LA and AA biomarkers with incident total CVD and subtypes (coronary heart disease, ischemic stroke, cardiovascular mortality) were investigated according to a prespecified analytic plan. Levels of LA and AA, measured as the percentage of total fatty acids, were evaluated linearly according to their interquintile range (ie, the range between the midpoint of the first and fifth quintiles), and categorically by quintiles. Study-specific results were pooled using inverse-variance–weighted meta-analysis. Heterogeneity was explored by age, sex, race, diabetes mellitus, statin use, aspirin use, omega-3 levels, and fatty acid desaturase 1 genotype (when available). RESULTS: In 30 prospective studies with medians of follow-up ranging 2.5 to 31.9 years, 15 198 incident cardiovascular events occurred among 68 659 participants. Higher levels of LA were significantly associated with lower risks of total CVD, cardiovascular mortality, and ischemic stroke, with hazard ratios per interquintile range of 0.93 (95% CI, 0.88–0.99), 0.78 (0.70–0.85), and 0.88 (0.79–0.98), respectively, and nonsignificantly with lower coronary heart disease risk (0.94; 0.88–1.00). Relationships were similar for LA evaluated across quintiles. AA levels were not associated with higher risk of cardiovascular outcomes; in a comparison of extreme quintiles, higher levels were associated with lower risk of total CVD (0.92; 0.86–0.99). No consistent heterogeneity by population subgroups was identified in the observed relationships. CONCLUSIONS: In pooled global analyses, higher in vivo circulating and tissue levels of LA and possibly AA were associated with lower risk of major cardiovascular events. These results support a favorable role for LA in CVD prevention