ABSTRACT
Introduction: The combination of gene content on the marker chromosome, chromosomal origin, level of mosaicism, origin mechanism (chromothripsis), and uniparental disomy can influence the final characterization of sSMCs. Several chromosomal aberrations, including sSMCs, have been observed in 30%-60% of patients with pigmentary mosaicism, and in more than 80%, chromosomal abnormalities are present in the mosaic state. In patients with pigmentary mosaicism the most representative chromosomes involved in sSMCs are 3, 5, 6, 9, 10, 13, 15, 18, 20, and X. In this study, we included the complete clinical, cytogenetic, and molecular characterization of seven patients with pigmentary mosaicism associated with the presence of SMCs of different chromosomal origins. Methods: The patients were diagnosed by the Genetics and Dermatology Department of three different hospitals. Cytogenetic and FISH analyses were performed on peripheral blood, light skin, and dark skin. FISH analysis was performed using different probes, depending on the marker chromosome description. Different array analysis was performed. Results: To date, of the seven cases studied, the chromosomal origins of six were successfully identified by FISH or array analysis. The chromosomes involved in SMCs were 6, 9, 15, and 18, X. The most frequently found was the centric minute structure. Discussion: To date, this group of seven patients constitutes the largest clinical and cytogenetically finely described study of cases with pigmentary mosaicism associated with sSMCs. Undoubtedly, analysis of the two skin types is a fundamental part of our study, as numerical differences may occur in the cell lines found in each skin type. The knowledge generated in this study will help delineate a very heterogeneous entity more accurately, and in the future, analyzing more patients with PM will likely establish a more definite association with the presence of this genetic alteration.
ABSTRACT
The share of variable renewable energy (VRE) is forecasted to increase in the energy sector to meet decarbonization targets and/or reduce their dependence on fossil fuels. The modeling of future power system scenarios is crucial to assess the role of different flexibility options, including low-carbon technologies. The data presented here support the research article "The role of energy storage in Great Britain's future power system: focus on hydrogen and biomass". These data include updated parameters, inputs, equations, biomass resource potential and biomass demand to balance bio-power and bio-hydrogen requirements. The Future Renewable Energy Performance into the Power System Model (FEPPS), a rule-based model that includes flexibility and stability constraints, has been used, and the hourly results of future scenarios by 2030 and 2040 are provided. Researchers, policymakers, and investors could use this paper as these data provide insights into the role of different technologies (including hydrogen and biomass) in power generation, system flexibility, decarbonization and costs.
ABSTRACT
An increasing share of dispatchable renewable generation is required to achieve energy decarbonisation goals and ensure a reliable supply to power grids. Concentrating solar power (CSP) plants hybridised with biomass boilers are promising alternatives to replace part of the peaking and baseload power generated from fossil fuel-based systems. This paper includes data related to the design variables, equations, valuation parameters and detailed results that support the research article "Market profitability of CSP-Biomass hybrid power plants: Towards a firm supply of renewable energy." The profitability assessment is based on integrating the hourly variation of electricity prices in the Iberian day-ahead market (MIBEL) to the results of the techno-economic model through a novel economic metric named Profitability Factor. In addition, stochastic simulations were conducted to capture the uncertainty of relevant input variables on the profitability of the proposed hybrid plants. The resulting datasets presented in this paper will provide insights for researchers looking to address the economic performance of renewable generation concepts from a market profitability approach. Furthermore, the data can be used by investors and policymakers to better understand the risks and implications associated with the profitability potential of these systems.
