Centro de Investigaciones Energéticas, Medioambientales y Tecnológicas
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TECHNOLOGY ROADMAP FOR HYBRIDISATION OF ENERGY STORAGE - Technological pathways for the integration of multi-technology storage systems to support Europe???s clean energy transition
Premature ageing of lung alveoli and bone marrow cells from Terc deficient mice with different telomere lengths
Telomeres are terminal protective chromosome structures. Genetic variants in genes coding for proteins required for telomere maintenance cause rare, life-threatening Telomere Biology Disorders (TBDs) such as dyskeratosis congenita, aplastic anemia or pulmonary fibrosis. The more frequently used mice strains have telomeres much longer than the human ones which question their use as in vivo models for TBDs. One mice model with shorter telomeres based on the CAST/EiJ mouse strain carrying a mutation in the Terc gene, coding for the telomerase RNA component, has been studied in comparison with C57BL/6J mice, carrying the same mutation and long telomeres. The possible alterations produced in lungs and the haematopoietic system, frequently affected in TBD patients, were determined at different ages of the mice. Homozygous mutant mice presented a very shortened life span, more notorious in the short-telomeres CAST/EiJ strain. The lungs of mutant mice presented a transitory increase in fibrosis and a significant decrease in the relative amount of the alveolar epithelial type 2 cells from six months of age. This decrease was larger in mutant homozygous animals but was also observed in heterozygous animals. On the contrary the expression of the senescence-related protein P21 increased from six months of age in mutant mice of both strains. The analysis of the haematopoietic system indicated a decrease in the number of megakaryocyte-erythroid progenitors in homozygous mutants and an increase in the clonogenic potential of bone marrow and LSK cells. Bone marrow cells from homozygous mutant animals presented decreasing in vitro expansion capacity. The alterations observed are compatible with precocious ageing of lung alveolar cells and the bone marrow cells that correlate with the alterations observed in TBD patients. The alterations seem to be more related to the genotype of the animals that to the basal telomere length of the strains although they are more pronounced in the short-telomere CAST/EiJ-derived strain than in C57BL/6J animals. Therefore, both animal models, at ages over 6???8 months, could represent valuable and convenient models for the study of TBDs and for the assay of new therapeutic products
Development of amorphous AlN thin films on ITO-glass and 2 ITO-PET at low temperature by rf-sputtering.
Aluminum nitride (AlN) is a material of wide interest in the optoelectronics and highpower
electronics industry. The deposition of AlN thin films at elevated temperatures is
a well-established process, but its implementation on flexible substrates with conductive
oxides, such as ITO-glass or ITO-PET, poses challenges due to the thermal degradation
of these materials. In this work, the deposition and characterization of AlN thin films
by reactive sputtering at a low temperature (RT and 100 ???C) on ITO-glass and ITO-PET
substrates are presented. The structural, optical, and electrical properties of the samples
have been analysed as a function of the sputtering power and the deposition temperature.
XRD analysis revealed the absence of peaks of crystalline AlN, indicative of the formation
of an amorphous phase. EDX measurements performed on the ITO-glass substrate with a
radiofrequency power applied to the Al target of 175Wconfirmed the presence of Al and N,
corroborating the deposition of AlN. SEM analyses showed the formation of homogeneous
and compact layers, and transmission optical measurements revealed a bandgap of around
5.82 eV, depending on the deposition conditions. Electrical resistivity measurements indicated
an insulating character. Overall, these findings confirm the potential of amorphous
AlN for applications in flexible optoelectronic devices
Blend design tools for Medium Combustion Plants (MCP) firing biomass wastes
Accepted manuscriptA feasible alternative for agricultural or forestry waste management is the operation of a distributed network of sustainable Medium Combustion Plants (MCPs). However, one of the main factors that hinder its
development is the propensity to operational problems derived from corrosion, slagging and fouling characteristics of both bottom and fly ashes. Therefore, a cost-effective approach for these multi-product MCP
could be based on predictive tools for an optimal formulation of a fuel blend. This work focuses on the
assessment of the ability of these methods to provide guidance for preventing ash-related operational problems and to provide fuel-blending rules.
The more widespread tools pertain to two types: compositional classification based on chemical analysis of laboratory ashes, and thermodynamic prediction of the most likely species and phases. Both criterion
numbers and compositional maps are ranking methods based on the chemical analysis at a given ashing temperature. Thermodynamic equilibrium modeling is not constrained by any difference in the physical conditions of the MCP compared to those in the laboratory.
Both kind of prediction tools have been validated in an MCP firing olive tree pruning residues as well as its typical blends in order to mimic a plausible pattern of fuels along a full year operating campaign. An
intensive experimental campaign encompasses plant monitoring and off-line analysis of the ashes along the process line.
Interpretation of compositional plots has revealed to be potentially sensitive to ashing temperature.
Here are presented examples showing how this variable could lead to either insignificant differences or to a substantial disparity in the a priori fuel diagnosis.
Some inconsistencies have been observed between the predictions based on criterion numbers, even for the same fuel and for ranking rules specifically formulated for biomasses. Moreover, it does not match
consistently with the information obtained from phase diagrams. Therefore, their use should be limited to the case of a well-established selection of a fuel index for a well-defined fuel provided empirical evidence
of an enough good description of the ash behavior, which is not the most frequent case.
Thermodynamic equilibrium calculations allow a more precise prediction of the main species in the condensed phase, without the constraint of the ashing temperature. Elemental closure of main ashforming
elements with the chemical analysis of the process ashes presents small differences, and their proximity localization on the phase diagrams denote similar prediction between predicted and process ashes
Array-based comparative genomic hybridization as a tool for solving practical biological and medical questions
Genetic aberrations, as losses of genetic material (deletions) or localized gains that affect certain regions of the genome, have been shown to be the basis of many diseases or human pathologies. Rare diseases, such as developmental abnormalities or mental retardation, or much more prevalent pathologies, such as cancer, are characterized by the occurrence of one or more of such genetic alterations in the genome that lead to changes in DNA sequence copy number
Detailed Magnetic and Mechanical Design of the Nested Orbit Correctors for HL-LHC
The high luminosity upgrade will enhance the discovery
potential of the LHC in the next decade. Among other magnets
and technologies currently under development, the MCBXF orbit
correctors will be required to this end. In order to save space, they
consist in two perpendicularly and coaxially arranged dipole coils
under a large torque. Coils cannot be glued due to the high radiation
dose expected, and mechanical clamping is mandatory. With
the goal of turning the conceptual design into a tangible reality, this
paper depicts the final magnetic design, with special attention to
3-D electromagnetic calculations and the different operation scenarios.
It also includes more accurate mechanical FE models results,
achieved by measuring the Young???s modulus of impregnated
cable stacks. Besides assembly spring-back, cool-down, and energization,
simulations have been also carried out for the pressing
process of both dipoles, analysing stress distribution, and displacements.
Finally, a short mechanical model has been designed, fabricated,
and tested. Its main purpose is to assess the feasibility of the
proposed clamping structure, the reliability of the FE mechanical
models and the design of an important part of the tooling required
for the magnet fabrication
Targeted gene therapy into a safe harbor site in human hematopoietic progenitor cells
Directed gene therapy mediated by nucleases has become a new alternative to lead targeted integration of therapeutic genes in specific regions in the genome. In this work, we have compared the efficiency of two nuclease types, TALEN and meganucleases (MN), to introduce an EGFP reporter gene in a specific site in a safe harbor locus on chromosome 21 in an intergenic region, named here SH6. The efficiency of targeted integration mediated by SH6v5-MN and SH6-TALEN in
HEK-293H cells was up to 16.3 and 15.0%. A stable expression was observed both in the pool of transfected cells and in established pseudoclones, with no detection of off-target integrations by Southern blot. In human hematopoietic stem and progenitor CD34+ cells, the nucleofection process preserved the viability and clonogenic capacity of nucleofected cells, reaching up to 3.1% of specific integration of the transgene in colony forming cells when the SH6-TALEN was used, although no expression of the transgene could be found in these cells. Our results show the possibility to specifically integrate genes at the SH6 locus in CD34+ progenitor cells, although further improvements in the efficacy of the procedure are required before this approach could be used for the gene editing of hematopoietic stem cells in patients with hematopoietic diseases
Electroluminiscencias de modulos fotovoltaicos en diferentes estadios de su reparaci??n
Se presentan las Electroluminiscencias de modulos fotovoltaicos que ha sido reparados en el marco del proyecto FOTOVOL3R.Incluyen las electroluminiscencias en diferentes estadios de la reparaci??n de los modulos.Se presentan las Electroluminiscencias de modulos fotovoltaicos que ha sido reparados en el marco del proyecto FOTOVOL3R.Incluyen las electroluminiscencias en diferentes estadios de la reparaci??n de los modulos
Deep Active Learning applied to gravitational waves
The detection of gravitational waves (GW) has opened a new window to progress in our understanding of astrophysical events and objects. The instruments used for direct detection (interferometers) require high sensitivity due to the tiny signals these GWs generate. In addition, proper characterisation of the detector is crucial for identifying noise sources and enhancing the performance. This thesis explores the implementation of Deep Active Learning (DAL) to identify and characterise short duration transient noise in the GW signal stream. We employ a convolutional neural network (CNN) combined with the DBSCAN clustering algorithm to classify glitches detected by interferometers. Moreover, an Attention Layer is implemented to highlight the relevant areas of the images for the final classification. Our approach recognises patterns similar to previously identified signals and detects anomalous ones that could correspond to previously unseen phenomena
Engraftment and in vivo proliferation advantage of gene-corrected mobilized CD34+ cells from Fanconi anemia patients
Previous Fanconi anemia (FA) gene therapy studies have failed to demonstrate engraftment of gene-corrected hematopoietic stem and progenitor cells (HSPCs) from FA patients, either after autologous transplantation or infusion into immunodeficient mice.Inthisstudy,wedemonstratethatavalidatedshorttransductionprotocolofG-CSF plus plerixafor-mobilized CD341 cells from FA-A patients with a therapeutic FANCAlentiviral vector corrects the phenotype of in vitro cultured hematopoietic progenitor cells. Transplantation of transduced FA CD341 cells into immunodeficient mice resulted in reproducible engraftment of myeloid, lymphoid, and CD341 cells. Importantly, a marked increase in the proportion of phenotypically corrected, patient-derived hematopoietic cells was observed after transplantation with respect to the infused CD341 graft, indicating the proliferative advantage of corrected FA-A hematopoietic repopulating cells. Our data demonstrate for the first time that optimized protocols of hematopoietic stem cell collection from FA patients, followed by the short and clinically validated transductionofthesecellswithatherapeuticlentiviralvector,resultsinthegenerationof phenotypically corrected HSPCs capable of repopulating and developing proliferation advantage in immunodeficient mice. Our results suggest that clinical approaches for FA gene therapy similar to those used in this studywillfacilitatehematopoieticrepopulationinFApatientswithgenecorrectedHSPCs,openingnewprospectsforgenetherapy of FApatient