Data-Gouv-ML/pcri-informations-sur-les-projets-laureats huggingface dataset:
The Molecular Communication Mechanism of Motor Neuron Survival and Synapse Maintenance (NEUROCOMMUNICATION)

Framework: FP7 — Ideas
Programme: European Research Council
Theme: European research council
Destination: Starting grants
Action type: Support for frontier research (ERC)
Panel: Neurosciences and neural disorders
Countries involved: Israel
Call:  (ERC-2012-StG_20111109, 2012)
Start date: 2013-02-01
End date: 2019-01-31
Duration (months): 72.0
Total project cost (EUR): 1499800.0
EU funding (EUR): 1499800.0
Participants: 1
Project status: CLOSED

In order to survive and maintain normal function, the cell depends on a dynamic system of spatial specificity and fidelity of signaling pathways that can respond to both internal and external changes over space and time. This cell-cell communication is mediated by ligand-receptor mechanisms. In the case of highly polarized cells such as neurons trafficking mechanisms mediated by motor proteins are used to achieve precise signal targeting. Alterations in the trafficking machinery may results in incorrect signaling, that in some cases leads to neurodegeneration. An example for such phenomenon may be found in Amyotrophic Lateral Sclerosis (ALS). ALS is a motor neuron disease characterized by a non-cell autonomous neurodegeneration process, which involves neighboring cells via an unknown mechanism. This proposal focuses on the elucidation of basic cell-cell communication mechanisms by using the motor neuron degeneration process as a model. I aim to reveal critical communication mechanisms between the neuron and its environment for cell survival and synapse maintenance. My working hypothesis is that alterations in extrinsic and intrinsic signals may lead to the neurodegeneration seen in ALS. I will develop unique compartmental platforms mimicking the natural environment of the motor neuron. Then using differential “omics” approaches followed by functional assays I will reveal and characterized vital factors essential to neuron synapse integrity and neuron survival. Using state of the art live-cell imaging techniques I will reveal also the molecular mechanism for signals localization and targeting driven by the motor protein dynein. I will elucidate the molecular mechanism of neuronal communication with its diverse environment essential to its survival and proper function. The project will bring revolutionary new mechanistic insight to a truly fundamental problem in cell biology, how the cell communicates and how signals arrive at the right place at the right time?

Keywords: neurodegeneration,retrograde signaling,axonal transport,neuromuscular junction,dynein,glia,motor neuron disease,als trophic factors,p75,trk,condition media,cellular communication mechanisms
new MAterials and REliability in offshore WINd Turbines technology (MARE-WINT)

Framework: FP7 — People
Programme: Marie-Curie Actions
Theme: Marie Skłodowska-Curie
Destination: Initial Training Networks
Action type: Support for training and career development of researchers (Marie Curie)
Countries involved: Poland,Denmark,Netherlands,United Kingdom,Belgium,Belgium,Norway,Sweden,Italy
Call:  (FP7-PEOPLE-2012-ITN, 2012)
Start date: 2012-10-01
End date: 2016-09-30
Duration (months): 48.0
Total project cost (EUR): 3822753.44
EU funding (EUR): 3822753.44
Participants: 13
Project status: CLOSED

One of the strategic objectives of the industrial initiative of the SET Plan on wind energy is to reduce cost of energy by improving reliability of wind turbines and their components and optimizing operation and maintenance (O&M) strategies. Increasing reliability and optimizing O&M have a direct impact on the availability of wind turbines and thus reduce cost and increase energy output. This strategy considerably contributes to making wind energy fully competitive. This is particularly evident in the offshore sector, where O&M represents a high percentage of total costs. MARE-WINT will contribute to the achievement of this goal by proving training in the context of doctoral programmes for 14 researchers in multi-disciplinary area of future generation of Offshore Wind Turbines (OWT) engineering focusing on issues having a major impact on the mechanical loading of OWT and which are still not sufficiently understood. OWT is a complex energy conversion fluid flow machine which entails coupled hydro-aero-mechanical issues. To design, built and operate a reliable OWT knowledge from disciplines like mechanical engineering, material science, metrology, fluid mechanics, condition monitoring, and computer simulation need to be combined. It is the ambition of MARE-WINT network to bring together specific partners capabilities and know-how to realize tailored training trajectories, focusing on increased reliability OWT design. Balanced industry-academia network consortium includes 6 Universities, 7 Research Institutes, 4 SME’s and 7 Large Industry Partners. The participation of 13 private sector Partners active in off-shore developments is essential to achieving the full impact of the project. Industrial partners are involved in hosting, training and defining the training needs of the researchers. Strong involvement of the industry will give PhD students the widest possible employment prospects. There are 4 “Industrial PhD” programmes identified within MARE-WINT

Keywords: wind energy technology,environmental protection,mechanical engineering,off-shore structures,marine technology
Signals for accommodative responses in humans (SACCO)

Framework: FP7 — Ideas
Programme: European Research Council
Theme: European research council
Destination: Starting grants
Action type: Support for frontier research (ERC)
Panel: Diagnostic tools, therapies and public health
Countries involved: Spain
Call:  (ERC-2012-StG_20111109, 2012)
Start date: 2012-11-01
End date: 2017-10-31
Duration (months): 60.0
Total project cost (EUR): 1384200.0
EU funding (EUR): 1384200.0
Participants: 1
Project status: CLOSED

Long-term objectives are to identify optical signals that control accommodation and emmetropization of the eye, and to identify the mechanisms that mediate the optical signals. A primary goal is to determine whether accommodation responds to the wavefront characteristics of light [defocus and higher-order aberrations (HOAs)], without feedback from change in defocus astigmatism or HOAs. Accommodation, pupillary responses and aberrations will be monitored by an imaging simulator that incorporates Hartmann-Shack wavefront sensing, adaptive optics and Hirschberg x-y eye tracking. Adaptive optics and Badal optics will compensate for the refractive error of the eye, alter or remove HOAs, and remove feedback from changes in accommodation and/or HOAs. Accommodation will be driven by defocus with normal HOAs present or removed. To determine whether accommodation responds to wavefront aberration per se, or simply to the shape or skewing of blur, aberrations will be removed while simulation of defocus targets are imaged on the retina, with and without the effects of HOAs on the blur. Standard model that accommodation operates as a closed-loop negative feedback system to maximize the contrast retinal image, will be tested by using adaptive optics to remove defocus, astigmatism and HOAs and by establishing a closed feedback loop between accommodation and target contrast. An alternative hypothesis that accommodation responds to wavefront defocus rather than blur of the retinal image, will be tested by driving accommodation with defocus without feedback from blur, in the absence of HOAs. A final experiment will determine whether the pupil changes independently of lenticular accommodation to provide better acuity in the presence of spherical aberration. Better understanding of the optical signals and mechanisms that the eye used to detect myopic and hyperopic defocus may lead to early detection of individual at risk for developing myopia, and new treatments to prevent/reduce myopia

Keywords: accommodation,refractive error
analysis of postmitotic chromatin decondensation (CHROMDECON)

Framework: FP7 — Ideas
Programme: European Research Council
Theme: European research council
Destination: Starting grants
Action type: Support for frontier research (ERC)
Panel: Molecular and Structural Biology and Biochemistry
Countries involved: Germany
Call:  (ERC-2012-StG_20111109, 2012)
Start date: 2013-03-01
End date: 2018-02-28
Duration (months): 60.0
Total project cost (EUR): 1499880.0
EU funding (EUR): 1499880.0
Participants: 2
Project status: CLOSED

Chromatin undergoes fascinating structural and functional changes during the metazoan cell cycle. It massively condenses at the beginning of mitosis with a degree of compaction up to fiftyfold higher than in interphase. At the end of mitosis, mitotic chromosomes decondense to re-establish their interphase chromatin structure. This process is indispensable for reinitiating transcription and treplication, and is thus of central importance in the cellular life cycle. Despite its significance to basic research as well as its potential medical implications, postmitotic chromatin decondensation is only poorly understood. It has been well described cytologically, but we lack an understanding of the underlying molecular events. We are ignorant about the proteins that mediate chromatin decondensation, the distinct steps in this multi-step procedure and their regulation. Using a novel in vitro assay, which recapitulates the process in the simplicity of a cell free reaction, we will identify the molecular machinery mediating postmitotic chromatin decondensation and define the different steps of the process. The cell free assay offers the unique possibility to isolate and purify activities responsible for individual steps in chromatin decondensation, to identify their molecular composition and to analyse the molecular changes they induce on chromatin. Accompanied by live cell imaging in mammalian tissue culture cells, the proposed approach will not only facilitate the elucidation of the factors involved in chromatin decondensation, but will also provide insight into how this process is integrated into mitotic exit and nuclear reformation and linked to other concomitant processes such as nuclear envelope assembly or nuclear body formation. Thus, using an unprecedented approach to study the ill-defined but important cell biological process of postmitotic chromatin decondensation, we aim to expand the frontiers in our knowledge on this topic.

Keywords: analysis of post-mitotic chromatin decondensation and identification of the factors involved in a biochemical/cell free assay
The Role of Non-coding RNA in Protein Networks and Neurodegenerative Diseases (RIBOMYLOME)

Framework: FP7 — Ideas
Programme: European Research Council
Theme: European research council
Destination: Starting grants
Action type: Support for frontier research (ERC)
Panel: Genetics,Genomics,Bioinformatics and Systems Biology
Countries involved: Spain
Call:  (ERC-2012-StG_20111109, 2012)
Start date: 2013-01-01
End date: 2018-12-31
Duration (months): 72.0
Total project cost (EUR): 1465351.0
EU funding (EUR): 1465351.0
Participants: 1
Project status: CLOSED

A major portion of the eukaryotic genome is occupied by DNA sequences whose transcripts do not code for proteins. This part of the genome is transcribed in a developmentally regulated manner and in response to external stimuli to produce large numbers of long non-coding RNAs (lncRNAs). From the beginning of transcription through splicing and translation, RNA molecules are associated with numerous RNA binding proteins that regulate their processing, stability, transport and translation. Both coding and non-coding RNAs and their associated binding proteins are involved in numerous cellular pathways. These pathways, which include RNA processing and the regulation of transcription and translation, are critical determinants of neuronal differentiation and plasticity. Alterations in these pathways have been identified to contribute to a wide variety of neurodegenerative diseases. Mutations in two RNA binding proteins involved in RNA splicing, the Tar DNA binding protein of 43kd (TDP-43) and Fused in Sarcoma (FUS), cause amyloid aggregation and are associated with Amyotrophic Lateral Sclerosis (ALS). My main interest is to understand the role played by RNA molecules in protein networks. Characterizing protein-RNA associations is key to unravel the complexity and functionality of mammalian genomes. In this project, I propose to study associations of lncRNAs with proteins involved in i) transcriptional regulation and epigenetics (such as polymerases, transcription factors and chromatin-modifiers) and ii) neurodegenerative diseases (such as Parkinson’s -synuclein, Alzheimer’s disease amyloid protein APP, TDP-43 and FUS). In particular, I will investigate if RNA molecules are involved in regulatory mechanisms that control protein production and prevent formation of toxic aggregates. In a multidisciplinary effort, I aim to discover protein-RNA interactions using advanced computational methods developed in my group and state of the art experimental techniques.

Keywords: long non-coding rna,protein aggregation,alzheimer's and parkinson's disease.
Reversibility and tissue specificity of mitochondrial translation defects in early childhood (REVERSIBLECOX)

Framework: FP7 — Ideas
Programme: European Research Council
Theme: European research council
Destination: Starting grants
Action type: Support for frontier research (ERC)
Panel: Physiology, Pathophysiology and Endocrinology
Countries involved: United Kingdom
Call:  (ERC-2012-StG_20111109, 2012)
Start date: 2013-03-01
End date: 2018-02-28
Duration (months): 60.0
Total project cost (EUR): 1432075.0
EU funding (EUR): 1432075.0
Participants: 1
Project status: CLOSED

We have recently identified the molecular genetic cause of a puzzling clinical syndrome, initially termed “benign infantile mitochondrial myopathy due to reversible cytochrome c oxidase (COX) deficiency”. While childhood-onset mitochondrial encephalomyopathies are usually severe, relentlessly progressive conditions with fatal outcome, this syndrome stands out by showing complete (or almost complete) spontaneous recovery. We have detected the homoplasmic m.14674T>C mutation in the mitochondrial mt-tRNAGlu gene in 17 affected individuals from 12 independent families of different ethnic origins. The m.14674T>C mutation affects the discriminator base of mt-tRNAGlu, the last base at the 3´-end of the molecule, where the amino acid via the terminal CCA is attached, therefore thought to impair mitochondrial translation, as reflected by the COX-negative fibres and the multiple respiratory chain defects in skeletal muscle. The spontaneous recovery of the patients suggests the existence of so far unknown cellular compensatory mechanisms. We will investigate, i) why patients with reversible COX deficiency show an isolated muscle involvement, ii) why symptoms start uniformly in the first days or weeks of life, iii) what is the molecular basis of the age-dependent, spontaneous recovery, and iv) which factors influence mitochondrial protein synthesis in human cells, skeletal muscle and different tissues. We will study these factors in v) different types of mitochondrial disease. From a scientific standpoint, this is one of the few hereditary conditions with a life-threatening onset showing recovery. Finding a clearly pathogenic homoplasmic mtDNA mutation offers a new paradigm of mtDNA pathogenesis, and studying this unique disease may unveil factors that are important in other mitochondrial disease. The long-term goal would be to upregulate or boost compensatory factors in patients with mitochondrial disease with the aim to open new avenues for therapy.

Keywords: mitochondrial disease,mitochondrial trna,mitochondrial translation,reversible cytochrome c oxidase (cox) deficiency myopathy,spontaneous recovery
Novel technologies for dissimilar materials joining (YBRIDIO)

Framework: FP7 — Cooperation
Programme: Nanosciences, Nanotechnologies, Materials and new Production Technologies - NMP
Destination: Materials
Action type: Collaborative project
Countries involved: Spain,Germany,Germany,Italy,Ireland,Switzerland,Spain
Call:  (FP7-NMP-2012-SMALL-6, 2012)
Start date: 2013-02-01
End date: 2016-01-31
Duration (months): 36.0
Total project cost (EUR): 3340616.8
EU funding (EUR): 2196827.0
Participants: 7
Project status: CLOSED

The requirements of innovation and efficiency are pushing industrial manufacturing methods to a new stage, where novel materials and components with enhanced properties are required. Polymers and polymer based composites are able to provide strength, light weight, corrosion resistance, impact resistance, etc. which are highly demanded from advanced technological sectors such as automotive, aerospace, electronics, consumer goods and others. For this reason the main objective presented in this project is the development of innovative and reliable thermal joining methods to join dissimilar materials, such as metal-plastic hybrid parts, with the aim to produce high quality joints between dissimilar materials, ensuring the integrity of the structure throughout the design, production and life cycle performance. Therefore, Ybridio is focused on thermal joining methods, specifically laser transmission joining (LTJ) and induction joining (IJ), which have demonstrated excellent potential to be implanted in industry. Both techniques deliver the energy directly to the base material and transmit the heat by conduction to the polymer part, so that the entire surfaces of both materials are attached to each other without any adhesive. The Ybridio project aims to achieve the following results: •Development of innovative and reliable new thermal joining techniques to join two or more different types of materials creating hybrid structures, such as metal with polymer and with composite laminates •Improvement of the characteristics of structures and components, combining and complementing the properties of each material •Simplification of industrial plants and processes to reduce manufacturing cost •To create a specific control system based on IR and NIR cameras, to ensure the quality of the components during the manufacturing process •Recycling and environmental impact: thermoplastic-metal joints can be disassembled easily by applying heat again which allows easy separation

Keywords: laser,induction,joining,hybrid materials,plastics,metals,thermoplastics,cfrp,susceptors,bonding
Keeping gene expression in check: eliciting the role of transcription in the maintenance of genome integrity. (TRANSARREST)

Framework: FP7 — Ideas
Programme: European Research Council
Theme: European research council
Destination: Starting grants
Action type: Support for frontier research (ERC)
Panel: Molecular and Structural Biology and Biochemistry
Countries involved: Greece
Call:  (ERC-2012-StG_20111109, 2012)
Start date: 2012-11-01
End date: 2018-10-31
Duration (months): 72.0
Total project cost (EUR): 1500000.0
EU funding (EUR): 1500000.0
Participants: 1
Project status: CLOSED

Genomic integrity is essential for accurate gene expression and epigenetic inheritance. On the other hand, a prolonged transcriptional arrest can challenge genome stability, contributing to genetic and epigenetic defects and the mechanisms of ageing and disease. Here we aim to identify the molecular mechanisms that couple transcriptional arrest to chromatin alteration and repair. We wish to explore the idea that transcription suppresses cellular toxicity and preserves genetic and epigenetic inheritance. Towards these goals our work will be focused on: 1. Deciphering the molecular events impinging on the manner cells respond when the progress of a transcribing RNA polymerase II is blocked. 2. Exploring a novel, so far unanticipated function of key players of the transcription-associated repair pathways, such as the Cockayne Syndrome (CS) proteins, not related to repair. 3. Understanding the role of transcription in chemotherapeutic-driven toxicity. 4. Investigating novel post-translational modifications of CS and determining their function. These objectives will be addressed using advanced proteomics and genome wide technologies in combination with biochemical and cellular techniques in normal human cells and a large battery of patient-derived cell lines. Our rational is that better understanding of CS function will help reach our ultimate goal, which is to identify the regulatory cascades involved in the interplay between genomic stability and transcription. The novel key idea put forward in this proposal is that active transcription itself directly contributes to genome integrity. While the role of DNA damage-driven transcription blockage in promoting repair is well established, the protective role of active transcription in genome stability is entirely unexplored. If successful, the proposed studies may help reveal the underlying causes of related disorders and explain their clinical features.

Keywords: dna damage-driven transcriptional arrest,histone modifications,dna repair,chip-on protein analysis,chip/rna-seq,cockayne syndrome,neurodegeneration,development,sumoylation
Understanding the development and function of cerebral cortex folding (CORTEXFOLDING)

Framework: FP7 — Ideas
Programme: European Research Council
Theme: European research council
Destination: Starting grants
Action type: Support for frontier research (ERC)
Panel: Neurosciences and neural disorders
Countries involved: Spain
Call:  (ERC-2012-StG_20111109, 2012)
Start date: 2013-01-01
End date: 2018-06-30
Duration (months): 66.0
Total project cost (EUR): 1701116.0
EU funding (EUR): 1701116.0
Participants: 1
Project status: CLOSED

The mammalian cerebral cortex was subject to a dramatic expansion in surface area during evolution. This process is recapitulated during development and is accompanied by folding of the cortical sheet, which allows fitting a large cortical surface within a limited cranial volume. A loss of cortical folds is linked to severe intellectual impairment in humans, so cortical folding is believed to be crucial for brain function. However, developmental mechanisms responsible for cortical folding, and the influence of this on cortical function, remain largely unknown. The goal of this proposal is to understand the genetic and cellular mechanisms that control the developmental expansion and folding of the cerebral cortex, and what is the impact of these processes on its functional organization. Human studies have identified genes essential for the proper folding of the human cerebral cortex. Genetic manipulations in mice have unraveled specific functions for some of those genes in the development of the cerebral cortex. But because the mouse cerebral cortex does not fold naturally, the mechanisms of cortical expansion and folding in larger brains remain unknown. We will study these mechanisms on ferret, an ideal model with a naturally folded cerebral cortex. We will combine the advantages of ferrets with cell biology, genetics and next-generation transcriptomics, together with state-of-the-art in vivo, in vitro and in silico approaches, including in vivo imaging of functional columnar maps. The successful execution of this project will provide insights into developmental and genetic risk factors for anomalies in human cortical topology, and into mechanisms responsible for the early formation of cortical functional maps.

Keywords: cerebral cortex,radial glia,migration,neurogenesis,ferret,transcriptomics,2-photon calcium imaging,cortical map,lissencephaly,osvz,optical imaging,intermediate progenitor