Myelin pathology: Involvement of molecular chaperones and the promise of chaperonotherapy

The process of axon myelination involves various proteins including molecular chaperones. Myelin alteration is a common feature in neurological diseases due to structural and functional abnormalities of one or more myelin proteins. Genetic proteinopathies may occur either in the presence of a normal chaperoning system, which is unable to assist the defective myelin protein in its folding and migration, or due to mutations in chaperone genes, leading to functional defects in assisting myelin maturation/migration. The latter are a subgroup of genetic chaperonopathies causing demyelination. In this brief review, we describe some paradigmatic examples pertaining to the chaperonins Hsp60 (HSPD1, or HSP60, or Cpn60) and CCT (chaperonin-containing TCP-1). Our aim is to make scientists and physicians aware of the possibility and advantages of classifying patients depending on the presence or absence of a chaperonopathy. In turn, this subclassification will allow the development of novel therapeutic strategies (chaperonotherapy) by using molecular chaperones as agents or targets for treatment

Development of a Vessel Scheduling Optimization Model to improve Maritime Transport sustainability

The social and economic development of the islands is generally dependent on the interconnection level with the mainland obtained through maritime transport services. When connecting many islands, the route planning is essential and typically a variety of constraints must be considered. Various optimization methods have been established to improve cost-efficiency but today environmental concerns, like the reduction of CO2 emissions, have become mandatory. This paper proposes a vessel scheduling optimization model able to simultaneously consider compulsory and optional tasks and vehicle-dependent profits. The algorithm was applied to seven islands of the Tyrrhenian Sea located in front of Sicily, named “Aeolian Islands”. Considering the regional requirements in terms of minimum number of routes and maximum fare prices for each season, this research compared the optimal vessels option obtained maximizing the profit with the one obtained minimizing the emissions. In particular, we have conducted three different analyses, in the first one we have considered only the mandatory routes while the second one was carried out identifying a series of potentially attractive additional activities based on historical demand data provided by the company that currently manages the service. Finally, the third analysis addresses a scenario where older fuel-powered vessels were replaced with hybrid electric ones

Economic and Environmental Assessment of Biomass Power Plants in Southern Italy

In 2019, Europe adopted the New Green Deal as a strategic plan to become a competitive, resource-efficient, and driven economy by reducing its gas emissions and carbon footprint. Due the COVID-19 pandemic, this strategic plan was recently updated to expedite the green transition of European industries. Therefore, the present paper deals with the problem of deciding an appropriate size for a biomass plant that directly produces electric energy by means of two different conversion processes: combustion and gasification. After an initial estimation of the energy potential in western Sicily, GIS data of biomass growth were used to identify the appropriate size for the power plants under investigation. The economic feasibility of biomass utilization was evaluated over a capacity range of 10 to 30 MW, considering total capital investments, revenues from energy sales, and total operating costs. Moreover, the effect of variations on incentive prices was analyzed by means of a sensitivity analysis. Comparing the different plant solutions considered, the environmental sustainability was also analyzed using the life cycle assessment (LCA) approach. The results showed that the combustion solution had a higher profitability and a lower environmental impact for each plant size. The obtained results also demonstrated that providing power from residual biomass in small agricultural communities would significantly reduce their environmental impacts while improving the economic feasibility of their waste management practices

Life cycle analysis of innovative building materials based on circular coffee ground supply chain

The construction sector is widely recognized as one of the most polluting mainly due to its intensive exploitation of natural resources and large energy consumption to produce traditional building materials. In the last years, alternative building materials have been developed with the aim to reduce the environmental burden of this sector. In particular, the use of geopolymer mortars as alternative cementitious materials is gaining increasing acceptance among scientists. Numerous laboratory studies demonstrate their suitability for construction applications, highlighting the potential environmental benefits that can be obtained from their large-scale production. This study aims to perform a preliminary evaluation of the environmental performance of a geopolymer mortar, whose production includes the reuse of a food waste: Spent Coffee Ground (SCG). By using the Life Cycle Assessment (LCA) approach, an environmental comparison with a traditional production of cement mortar was carried out on the basis of the Global Warming Potential (GWP) indicator

Microstructural and morphological properties of homoepitaxial (001)ZnTe layers investigated by x-ray diffuse scattering

The microstructural and morphological properties of homoepitaxial (001)ZnTe layers are investigated by x-ray diffuse scattering. High resolution reciprocal space maps recorded close to the ZnTe (004) Bragg peak show different diffuse scattering features. One kind of cross-shaped diffuse scattering streaks along directions can be attributed to stacking faults within the epilayers. Another kind of cross-shaped streaks inclined at an angle of about 80deg with respect to the in-plane direction arises from the morphology of the epilayers. (abridged version

A spectroscopic study of the open cluster NGC 6250

We present the chemical abundance analysis of 19 upper main-sequence stars of the young open cluster NGC 6250 (log t∼7.42 yr). Thiswork is part of a project aimed at setting observational constraints on the theory of atomic diffusion in stellar photospheres, by means of a systematic study of the abundances of the chemical elements of early F-, A- and late B-type stars of well-determined age. Our data set consists of low-, medium- and high-resolution spectra obtained with the Fibre Large Array Multi Element Spectrograph (FLAMES) instrument of the ESO Very Large Telescope (VLT). To perform our analysis, we have developed a new suite of software tools for the chemical abundance analysis of stellar photospheres in local thermodynamical equilibrium. Together with the chemical composition of the stellar photospheres, we have provided new estimates of the cluster mean radial velocity, proper motion, refined the cluster membership, and we have given the stellar parameters including masses and fractional age. We find no evidence of statistically significant correlation between any of the parameters, including abundance and cluster age, except perhaps for an increase in Ba abundance with cluster age.We have proven that our new software tool may be successfully used for the chemical abundance analysis of large data sets of stellar spectra

A 3D-Shape Similarity-based Contrastive Approach to Molecular Representation Learning

Molecular shape and geometry dictate key biophysical recognition processes, yet many graph neural networks disregard 3D information for molecular property prediction. Here, we propose a new contrastive-learning procedure for graph neural networks, Molecular Contrastive Learning from Shape Similarity (MolCLaSS), that implicitly learns a three-dimensional representation. Rather than directly encoding or targeting three-dimensional poses, MolCLaSS matches a similarity objective based on Gaussian overlays to learn a meaningful representation of molecular shape. We demonstrate how this framework naturally captures key aspects of three-dimensionality that two-dimensional representations cannot and provides an inductive framework for scaffold hopping

Histopathology of Skeletal Muscle in a Distal Motor Neuropathy Associated with a Mutant CCT5 Subunit: Clues for Future Developments to Improve Differential Diagnosis and Personalized Therapy

Genetic chaperonopathies are rare but, because of misdiagnosis, there are probably more cases than those that are recorded in the literature and databases. This occurs because practitioners are generally unaware of the existence and/or the symptoms and signs of chaperonopathies. It is necessary to educate the medical community about these diseases and, with research, to unveil their mechanisms. The structure and functions of various chaperones in vitro have been studied, but information on the impact of mutant chaperones in humans, in vivo, is scarce. Here, we present a succinct review of the most salient abnormalities of skeletal muscle, based on our earlier report of a patient who carried a mutation in the chaperonin CCT5 subunit and suffered from a distal motor neuropathy of early onset. We discuss our results in relation to the very few other published pertinent reports we were able to find. A complex picture of multiple muscle-tissue abnormalities was evident, with signs of atrophy, apoptosis, and abnormally low levels and atypical distribution patterns of some components of muscle and the chaperone system. In-silico analysis predicts that the mutation affects CCT5 in a way that could interfere with the recognition and handling of substrate. Thus, it is possible that some of the abnormalities are the direct consequence of defective chaperoning, but others may be indirectly related to defective chaperoning or caused by other different pathogenic pathways. Biochemical, and molecular biologic and genetic analyses should now help in understanding the mechanisms underpinning the histologic abnormalities and, thus, provide clues to facilitate diagnosis and guide the development of therapeutic tools