Showing papers by "Cristina Prieto published in 2017"

Review of commercial thermal energy storage in concentrated solar power plants: Steam vs. molten salts

Abstract: Thermal energy storage systems are key components of concentrating solar power plants in order to offer energy dispatchability to adapt the electricity power production to the curve demand. This paper presents a review of the current commercial thermal energy storage systems used in solar thermal power plants: steam accumulators and molten salts. It describes the mentioned storage concepts and the results of their economic evaluation. The economic value of the TES system is assessed by the Levelized Cost of Electricity (LCOE) calculation, an economic performance metric commonly used in power generation in order to compare cost of electricity among different power generation sources. Lots of studies have been done in the past to compare the LCOE of a complete solar thermal power plant using thermal energy storage systems. However, no specific studies related to the thermal energy storage levelized cost of electricity itself were done. The objective of this study is focused in the comparison of the TES LCOE where calculations are done for a 100 MW Rankine cycle with different plant configuration and for different storage sizes ranging from 1 to 9 h of equivalent full capacity operation.

Genetic Rescue of Mitochondrial and Skeletal Muscle Impairment in an Induced Pluripotent Stem Cells Model of Coenzyme Q10 Deficiency

Abstract: Coenzyme Q10 (CoQ10) plays a crucial role in mitochondria as an electron carrier within the mitochondrial respiratory chain (MRC), and is an essential antioxidant. Mutations in genes responsible for CoQ10 biosynthesis (COQ genes) cause primary CoQ10 deficiency, a rare and heterogeneous mitochondrial disorder with no clear genotype-phenotype association, mainly affecting tissues with high-energy demand including brain and skeletal muscle (SkM). Here, we report a 4-year old girl diagnosed with minor mental retardation and lethal rhabdomyolysis harboring a heterozygous mutation (c.483G>C (E161D)) in COQ4. The patient's fibroblasts showed a decrease in [CoQ10], CoQ10 biosynthesis, MRC activity affecting complexes I/II+III, and respiration defects. Bona fide induced pluripotent stem cell (iPSCs) lines carrying the COQ4 mutation (CQ4-iPSCs) were generated, characterized and genetically edited using the CRISPR-Cas9 system (CQ4ed-iPSCs). Extensive differentiation and metabolic assays of control-iPSCs, CQ4-iPSCs and CQ4ed-iPSCs demonstrated a genotype association, reproducing the disease phenotype. The COQ4 mutation in iPSC was associated with CoQ10 deficiency, metabolic dysfunction, and respiration defects. iPSC differentiation into SkM was compromised, and the resulting SkM also displayed respiration defects. Remarkably, iPSC differentiation in dopaminergic or motor neurons was unaffected. This study offers an unprecedented iPSC model recapitulating CoQ10 deficiency-associated functional and metabolic phenotypes caused by COQ4 mutation. This article is protected by copyright. All rights reserved.