The Commissariat à l'énergie atomique et aux énergies alternatives or CEA, is a French public government-funded research organisation in the areas of energy, defense and security, information technologies and health technologies. The CEA maintains a cross-disciplinary culture of engineers and researchers, building on the synergies between fundamental and technological research Wikipedia.
Galat A.,French Atomic Energy Commission
Cellular and Molecular Life Sciences | Year: 2013
From 5 to 12 FK506-binding proteins (FKBPs) are encoded in the genomes of disparate marine organisms, which appeared at the dawn of evolutionary events giving rise to primordial multicellular organisms with elaborated internal body plan. Fifteen FKBPs, several FKBP-like proteins and some splicing variants of them are expressed in humans. Human FKBP12 and some of its paralogues bind to different macrocyclic antibiotics such as FK506 or rapamycin and their derivatives. FKBP12/(macrocyclic antibiotic) complexes induce diverse pharmacological activities such as immunosuppression in humans, anticancerous actions and as sustainers of quiescence in certain organisms. Since the FKBPs bind to various assemblies of proteins and other intracellular components, their complexes with the immunosuppressive drugs may differentially perturb miscellaneous cellular functions. Sequence-structure relationships and pharmacological profiles of diverse FKBPs and their involvement in crucial intracellular signalization pathways and modulation of cryptic intercellular communication networks were discussed. © 2012 Springer Basel.
Galat A.,French Atomic Energy Commission
Cellular and Molecular Life Sciences | Year: 2011
The transforming growth factor-β (TGFβ) superfamily of proteins and their receptors are crucial developmental factors for all metazoan organisms. Cystine-knot (CK) motif is a spatial feature of the TGFβ superfamily of proteins whereas the extra-cellular domains (ectodomains) of their respective receptors form three-fingered protein domain (TFPD), both stabilized by tight cystine networks. Analyses of multiple sequence alignments of these two domains encoded in various genomes revealed that the cystines forming the CK and TFPD folds are conserved, whereas the remaining polypeptide patches are diversified. Orthologues of the human TGFβs and their respective receptors expressed in diverse vertebrates retain high sequence conservation. Examination of 3D structures of various TGFβ factors bound to their receptors have revealed that the CK and TFPD domains display several similar spatial traits suggesting that these two different protein folds might have been acquired from a common ancestor. © 2011 Springer Basel AG.
Payen E.,French Atomic Energy Commission
Hematology / the Education Program of the American Society of Hematology. American Society of Hematology. Education Program | Year: 2012
High-level production of β-globin, γ-globin, or therapeutic mutant globins in the RBC lineage by hematopoietic stem cell gene therapy ameliorates or cures the hemoglobinopathies sickle cell disease and beta thalassemia, which are major causes of morbidity and mortality worldwide. Considerable efforts have been made in the last 2 decades in devising suitable gene-transfer vectors and protocols to achieve this goal. Five years ago, the first β(E)/β(0)-thalassemia major (transfusion-dependent) patient was treated by globin lentiviral gene therapy without injection of backup cells. This patient has become completely transfusion independent for the past 4 years and has global amelioration of the thalassemic phenotype. Partial clonal dominance for an intragenic site (HMGA2) of chromosomal integration of the vector was observed in this patient without a loss of hematopoietic homeostasis. Other patients are now receiving transplantations while researchers are carefully weighing the benefit/risk ratio and continuing the development of further modified vectors and protocols to improve outcomes further with respect to safety and efficacy.
Agency: Cordis | Branch: H2020 | Program: RIA | Phase: INFRAIA-1-2014-2015 | Award Amount: 10.00M | Year: 2016
ENSAR2 is the integrating activity for European nuclear scientists who are performing research in three of the major subfields defined by NuPECC: Nuclear Structure and Dynamics, Nuclear Astrophysics and Nuclear Physics Tools and Applications. It proposes an optimised ensemble of Networking (NAs), Joint Research (JRAs) and Transnational Access Activities (TAs), which will ensure qualitative and quantitative improvement of the access provided by the current ten infrastructures, which are at the core of this proposal. The novel and innovative developments that will be achieved by the RTD activities will also assure state-of-the-art technology needed for the new large-scale projects. Our community of nuclear scientists profits from the diverse range of world-class research infrastructures all over Europe that can supply different ion beams and energies and, with ELI-NP, high-intensity gamma-ray beams up to 20 MeV. We have made great effort to make the most efficient use of these facilities by developing the most advanced and novel equipment needed to pursue their excellent scientific programmes and applying state-of-the-art developments to other fields and to benefit humanity (e.g. archaeology, medical imaging). Together with multidisciplinary and application-oriented research at the facilities, these activities ensure a high-level socio-economic impact. To enhance the access to these facilities, the community has defined a number of JRAs, using as main criterion scientific and technical promise. These activities deal with novel and innovative technologies to improve the operation of the facilities. The NAs of ENSAR2 have been set-up with specific actions to strengthen the communities coherence around certain resarch topics and to ensure a broad dissemination of results and stimulate multidisciplinary, application-oriented research and innovation at the Research Infrastructures.
Agency: Cordis | Branch: H2020 | Program: IA | Phase: LCE-09-2015 | Award Amount: 27.97M | Year: 2016
This proposal is an application to the EU programme Horizon 2020 and its topic Large scale energy storage (LCE-09-2015). The presented project STORE&GO will demonstrate three innovative Power to Gas storage concepts at locations in Germany, Switzerland and Italy in order to overcome technical, economic, social and legal barriers. The demonstration will pave the way for an integration of PtG storage into flexible energy supply and distribution systems with a high share of renewable energy. Using methanation processes as bridging technologies, it will demonstrate and investigate in which way these innovative PtG concepts will be able to solve the main problems of renewable energies: fluctuating production of renewable energies; consideration of renewables as suboptimal power grid infrastructure; expensive; missing storage solutions for renewable power at the local, national and European level. At the same time PtG concepts will contribute in maintaining natural gas or SNG with an existing huge European infrastructure and an already advantageous and continuously improving environmental footprint as an important primary/secondary energy carrier, which is nowadays in doubt due to geo-political reasons/conflicts. So, STORE&GO will show that new PtG concepts can bridge the gaps associated with renewable energies and security of energy supply. STORE&GO will rise the acceptance in the public for renewable energy technologies in the demonstration of bridging technologies at three living best practice locations in Europe.