Heparan sulfate proteoglycans: The sweet side of development turns sour in mucopolysaccharidoses

Abstract Heparan sulfate proteoglycans (HSPGs) are complex carbohydrate-modified proteins ubiquitously expressed on cell surfaces, extracellular matrix and basement membrane of mammalian tissues. Beside to serve as structural constituents, they regulate multiple cellular activities. A critical involvement of HSPGs in development has been established, and perturbations of HSPG-dependent pathways are associated with many human diseases. Recent evidence suggest a role of HSPGs in the pathogenesis of mucopolysaccharidoses (MPSs) where the accumulation of undigested HS results in the loss of cellular functions, tissue damage and organ dysfunctions accounting for clinical manifestations which include central nervous system (CNS) involvement, degenerative joint disease and reduced bone growth. Current therapies are not curative but only ameliorate the disease symptoms. Here, we highlight the link between HSPG functions in the development of CNS and musculoskeletal structures and the etiology of some MPS phenotypes, suggesting that HSPGs may represent potential targets for the therapy of such incurable diseases

A Numerical Approach for Assigning a Reputation to Users of an IoT Framework

AbstractNowadays, in the Internet of Things (IoT) society, the massive use of technological devices available to the people makes possible to collect a lot of data describing tastes, choices and behaviours related to the users of services and tools. These information can be rearranged and interpreted in order to obtain a rating (i.e., evaluation) of the subjects (i.e., users) interacting with specific objects (i.e., items). Generally, reputation systems are widely used to provide ratings to products, services, companies, digital contents and people. Here, we focus on this issue, adopting a Collaborative Reputation System (CRS) to evaluate the visitors' behaviour in a real cultural event. The results obtained, compared with those obtained by other methods (i.e., classification), have confirmed the reliability and the usefulness of CRSes for deeply understand dynamics related to visiting styles

The relationship between drive to thinness, conscientiousness and bulimic traits during adolescence: a comparison between younger and older cases in 608 healthy volunteers

Adolescence represents one of the critical transitions in the life span and is characterized by a tremendous pace in growth and change that is second only to that of infancy. Both biological and psychological changes occurring during early adolescence may also influence the definition of subsequent late adolescence or early adulthood physiological or (psycho)-pathological features, including bulimia nervosa (BN) whenever occurring. Therefore, a pre-emptive assessment of suggestive psychological traits, including bulimic ones, during early and late years of adolescence, is recommended and represents the goal of the present study

Influence of Organic and Conventional Farming on Grain Yield and Protein Composition of Chickpea Genotypes

Chickpea is a key crop in sustainable cropping systems and for its nutritional value. Studies on agronomic and genetic influences on chickpea protein composition are missing. In order to obtain a deep insight into the genetic response of chickpeas to management in relation to agronomic and quality traits, a two-year field trial was carried out with eight chickpea genotypes under an organic and conventional cropping system. Protein composition was assessed by SDS-PAGE in relation to the main fractions (vicilin, convicilin, legumin, lectin, 2s-albumin). Crop response was highly influenced by year and presumably also by management, with a −50% decrease in grain yield under organic farming, mainly due to a reduction in seed number per m2. No effect of crop management was observed on protein content, despite significant differences in terms of protein composition. The ratio between the major globulins, 7s vicilin and 11s legumin, showed a negative relationship with grain yield and was found to be higher under organic farming. Among genotypes, black-seed Nero Senise was characterized by the highest productivity and water-holding capacity, associated with low lectin content. These findings highlight the importance of the choice of chickpea genotypes for cultivation under organic farming in relation to both agronomic performance and technological and health quality

Competitive binding of extracellular accumulated heparan sulfate reduces lysosomal storage defects and triggers neuronal differentiation in a model of Mucopolysaccharidosis IIIB.

Mucopolysaccharidoses (MPSs) are a group of inherited lysosomal storage disorders associated with the deficiency of lysosomal enzymes involved in glycosaminoglycan (GAG) degradation. The resulting cellular accumulation of GAGs is responsible for widespread tissue and organ dysfunctions. The MPS III, caused by mutations in the genes responsible for the degradation of heparan sulfate (HS), includes four subtypes (A, B, C, and D) that present significant neurological manifestations such as progressive cognitive decline and behavioral disorders. The established treatments for the MPS III do not cure the disease but only ameliorate non-neurological clinical symptoms. We previously demonstrated that the natural variant of the hepatocyte growth factor NK1 reduces the lysosomal pathology and reactivates impaired growth factor signaling in fibroblasts from MPS IIIB patients. Here, we show that the recombinant NK1 is effective in rescuing the morphological and functional dysfunctions of lysosomes in a neuronal cellular model of the MPS IIIB. More importantly, NK1 treatment is able to stimulate neuronal differentiation of neuroblastoma SK-NBE cells stable silenced for the NAGLU gene causative of the MPS IIIB. These results provide the basis for the development of a novel approach to possibly correct the neurological phenotypes of the MPS IIIB as well as of other MPSs characterized by the accumulation of HS and progressive neurodegeneration

Pro-inflammatory RNA:DNA hybrids are p53 independently boosted by hyperbaric oxygen: a subcellular distribution analysis by automated quantitative imaging

Purpose: RNA:DNA hybrids are co-transcriptional products with acknowledged cytoplasmic pro-inflammatory role as activators of the cGAS-STING pathway. We recently proved them also as radiation-induced senescence messages for the abscopal effect mediation, demonstrating the need for a functional p53 for their production and release in A549 and H1299 tumour cells. However, little is known about their role under different stress conditions, especially in cancer cells. Methods: In this work, we open the investigation making use of automated quantitative imaging to characterize the hybrid subcellular distribution in HeLa cells grown under different oxygen pressures or exposed to different ionizing radiation doses. After cell imaging by confocal fluorescent microscopy, we apply automated imaging methods developed on purpose to quantify hybrid foci and nuclear cluster intensity, regional and local density and dimension. Results: We show that alteration of culture oxygenation increases hybrid cytoplasmic presence, especially when caused by an hyperoxic environment, with evident hybrid gathering at the cell membrane. Ionizing radiations always fail to increase hybrids, in accordance with the absence of functional p53 in HeLa cells. However, dose-dependent effects are still evident and suggest a threshold dose of 7.5 Gy for remarkable hybrid reduction. Conclusion: Together with our previous results, these data demonstrate for the first time that different types of stress can increase hybrid production in cancer cells and by at least two different pathways, one p53-dependent triggerable by ionizing radiations and one p53-independent triggerable by oxidative stress. Together, our findings provide a starting point for understanding hybrid role in tumour stress response

Estimation of evapotranspiration and crop coefficients of tendone vineyards using multi-sensor remote sensing data in a mediterranean environment

The sustainable management of water resources plays a key role in Mediterranean viticulture, characterized by scarcity and competition of available water. This study focuses on estimating the evapotranspiration and crop coefficients of table grapes vineyards trained on overhead "tendone" systems in the Apulia region (Italy). Maximum vineyard transpiration was estimated by adopting the "direct" methodology for ETp proposed by the Food and Agriculture Organization in Irrigation and Drainage Paper No. 56, with crop parameters estimated from Landsat 8 and RapidEye satellite data in combination with ground-based meteorological data. The modeling results of two growing seasons (2013 and 2014) indicated that canopy growth, seasonal and 10-day sums evapotranspiration values were strictly related to thermal requirements and rainfall events. The estimated values of mean seasonal daily evapotranspiration ranged between 4.2 and 4.1 mm·d-1, while midseason estimated values of crop coefficients ranged from 0.88 to 0.93 in 2013, and 1.02 to 1.04 in 2014, respectively. The experimental evapotranspiration values calculated represent the maximum value in absence of stress, so the resulting crop coefficients should be used with some caution. It is concluded that the retrieval of crop parameters and evapotranspiration derived from remotely-sensed data could be helpful for downscaling to the field the local weather conditions and agronomic practices and thus may be the basis for supporting grape growers and irrigation managers

Cognitive functioning and subjective quality of life in relapsing-remitting multiple sclerosis patients before and after percutaneous transluminal angioplasty: a preliminary report

Multiple sclerosis (MS) is a disease of the nervous system that has profound effects on everyday functioning and quality of life of not only the person who is diagnosed, but also her/his family and acquaintances. Despite this, the uncertainties of the actual etiological basis of MS make it difficult to reach a conclusive statement about the optimal therapeutic management of the disease, which may differ depending on the given case and phase of illness. This has led to an interest in potential novel therapeutic avenues, including percutaneous transluminal angioplasty (PTA). Yet, evidence in support of PTA in the management of MS is scarce and contradictory. The aim of the present study was to provide a preliminary assessment as to whether PTA may impact subjective quality of life and cognitive functioning in severe MS

Effect of rootstock on growth, yield and fruit characteristics in cv 'Bianca' pistachio (Pistacia vera L.) trees

This paper, following preliminary field evaluation trials started in 1993, reports a study on the effect of eight different in vitro-propagated clonal rootstocks (P. atlantica and P. integerrima) and one seedling rootstock (P. terebinthus) on the vegetative and productive behaviour of pistachio cultivar 'Bianca'. The trees, budded in 1991, were grown using standard cultural practices for dry-land farming in a sandy clay loam soil, located inland in Sicily. On average, clones of P. integerrima (I-6 and I-2) were the most vigorous rootstocks. Clones of P. atlantica had intermediate vigour. Rootstock significantly affect yield but not fruit weight, kernel to nut ratio, splitting and blanking percentages. The highest yield efficiencies were observed with I-4 and A-3 and A-5, whereas the lowest were obtained with I-6 and I-2 and A-8. Nut shape was significantly affected by the rootstock. Slight differences in leaf mineral content were observed among the tested graft combinations