La maladie de Sanfilippo

La maladie de Sanfilippo ou mucopolysaccharidose de type III (MPS III) est une maladie génétique rare. Il s agit d une maladie lysosomale. Une anomalie dans le phénomène de dégradation de l héparane sulfate est à l origine de cette maladie. Cette anomalie aboutit à une accumulation de l héparane sulfate dans différents organes. Les patients présentent une agressivité, un sommeil perturbé, une hyperactivité ainsi qu une incoordination motrice. A l heure actuelle, il n existe pas de traitement spécifique de la maladie de Sanfilippo. La prise en charge consiste en un traitement symptomatique des différents troubles observés. Différentes stratégie thérapeutique sont étudiées mais seule la thérapie génique semble prometteuse.Sanfilippo syndrome or mucopolysaccharidosis type III (MPS III) is a rare genetic disease. It is a lysosomal storage disorder due to impaired degradation of heparan sulphate. So the mucopolysaccharidies remain stored in the cells causing progresive damage. Patients exhibit aggression, disturbed sleep, hyperactivity and failure of muscle coordination. At the present time, there is no cure for Sanfilippo syndrome. The therapy consists of a symptomatic treatement. Several therapeutic strategies are studied but only gene therapy seems ti bo promising.AMIENS-BU Santé (800212102) / SudocSudocFranceF

SOTERIA D5.4 HARDWARE-BASED PRIVACY

In the framework of the privacy-preserving concerns of the SOTERIA project, and concerning future Machine Learning (ML) implementations, we explore hardware-based privacy computations for secure and private data storage and ML computations. Current supported hardware on cloud and consumer devices most notably include Trusted Execution Environments (TEEs), which have emerged as a critical technology in modern computing, offering hardware-enforced security measures to protect sensitive data and computations from potential threats. This report presents a comprehensive review of TEEs, focusing on the advantages, vulnerabilities, and implementations of prominent TEE technologies, and the state-of-the-art on TEE-based methods for privacy-preserving sensitive data storage and ML operations. Finally, we provide a discussion on these commercial implementations, methods, possible implementations for improving the security and privacy on the SOTERIA platform for current and future applications, and our conclusion

Deciphering the Influence of Electrolytes on the Energy Storage Mechanism of Vertically-Oriented Graphene Nanosheet Electrodes by Using Advanced Electrogravimetric Methods

International audienceElectrolyte composition is a crucial factor determining the capacitive properties of a supercapacitor device. However, its complex influence on the energy storage mechanisms has not yet been fully elucidated. For this purpose, in this study, the role of three different types of electrolytes based on a propylene carbonate (PC) solution containing tetrabutylammonium perchlorate (TBAClO4), lithium perchlorate (LiClO4) and butyltrimethylammonium bis(trifluoromethylsulfonyl)imide (N1114TFSI) ionic liquid on vertically-oriented graphene nanosheet electrodes has been investigated. Herein, in situ electrochemical quartz crystal microbalance (EQCM) and its coupling with electrochemical impedance spectroscopy (EIS), known as ac-electrogravimetry, have allowed the dynamic aspects of the (co)electroadsorption processes at the electrode-electrolyte interface to be examined. A major contribution of ClO4- anions (TBAClO4) was evidenced, whereas in the PC/N1114TFSI mixture (50:50 wt%) both anions (TFSI-) and cations (N1114+) were symmetrically exchanged during cycling. In the particular case of LiClO4, solvation of Li+ cations in PC was involved, affecting the kinetics of electroadsorption. These results demonstrate the suitability of dynamic electrogravimetric methods to unveil the interfacial exchange properties of mobile species for the conception of new high performance energy storage devices

Controlling the Hydrophilicity of the Electrochemical Interface to Modulate the Oxygen-Atom Transfer in Electrocatalytic Epoxidation Reactions

The electrocatalytic epoxidation of alkenes at heterogeneous catalysts using water as the sole oxygen source is a promising safe route toward the sustainable synthesis of epoxides, which are essential building blocks in organic chemistry. However, the physico-chemical parameters governing the oxygen-atom transfer to the alkene and the impact of the electrolyte structure on the epoxidation reaction are yet to be understood. Here, we study the electrocatalytic epoxidation of cyclooctene at the surface of gold in hybrid organic/aqueous mixtures using acetonitrile (ACN) solvent. Gold was selected, as in ACN/water electrolytes gold oxide is formed by reactivity with water at potentials less anodic than the oxygen evolution reaction (OER). This unique property allows us to demonstrate that a sacrificial mechanism is responsible for cyclooctene epoxidation at metallic gold surfaces, proceeding through cyclooctene activation, while epoxidation at gold oxide shares similar reaction intermediates with the OER and proceeds via the activation of water. More importantly, we show that the hydrophilicity of the electrode/electrolyte interface can be tuned by changing the nature of the supporting salt cation, hence affecting the reaction selectivity. At low overpotential, hydrophilic interfaces formed by using strong Lewis acid cations are found to favor gold passivation. Instead, hydrophobic interfaces created by the use of large organic cations favor the oxidation of cyclooctene and the formation of epoxide. Our study directly demonstrates how tuning the hydrophilicity of electrochemical interfaces can improve both the yield and selectivity of anodic reactions at the surface of heterogeneous catalysts

Controlling the Hydrophilicity of the Electrochemical Interface to Modulate the Oxygen-Atom Transfer in Electrocatalytic Epoxidation Reactions

The electrocatalytic epoxidation of alkenes at heterogeneous catalysts using water as the sole oxygen source is a promising safe route toward the sustainable synthesis of epoxides, which are essential building blocks in organic chemistry. However, the physicochemical parameters governing the oxygen-atom transfer to the alkene and the impact of the electrolyte structure on the epoxidation reaction are yet to be understood. Here, we study the electrocatalytic epoxidation of cyclooctene at the surface of gold in hybrid organic/aqueous mixtures using acetonitrile (ACN) solvent. Gold was selected, as in ACN/water electrolytes gold oxide is formed by reactivity with water at potentials less anodic than the oxygen evolution reaction (OER). This unique property allows us to demonstrate that a sacrificial mechanism is responsible for cyclooctene epoxidation at metallic gold surfaces, proceeding through cyclooctene activation, while epoxidation at gold oxide shares similar reaction intermediates with the OER and proceeds via the activation of water. More importantly, we show that the hydrophilicity of the electrode/electrolyte interface can be tuned by changing the nature of the supporting salt cation, hence affecting the reaction selectivity. At low overpotential, hydrophilic interfaces formed using strong Lewis acid cations are found to favor gold passivation. Instead, hydrophobic interfaces created by the use of large organic cations favor the oxidation of cyclooctene and the formation of epoxide. Our study directly demonstrates how tuning the hydrophilicity of electrochemical interfaces can improve both the yield and selectivity of anodic reactions at the surface of heterogeneous catalysts

Enterobacteriaceae facilitate the anaerobic degradation of glucose by a forest soil

Anoxic micro zones that occur in soil aggregates of oxic soils may be temporarily extended after rainfall and thus facilitate the anaerobic degradation of organic compounds in soils. The microbial degradation of glucose by anoxic slurries of a forest soil yielded acetate, CO2, H-2, succinate, and ethanol, products indicative of mixed acid fermentation. Prokaryotes involved in this process were identified by time-resolved 16S rRNA gene-targeted stable isotope probing with [C-13-U]-glucose. All labeled phylotypes from the C-13-enriched 16S rRNA gene were most closely related to Rahnella and Ewingella, enterobacterial genera known to catalyze mixed acid fermentation. These results indicate that facultative aerobes, in particular Enterobacteriaceae, (1) can outcompete obligate anaerobes when conditions become anoxic in forest soils and (2) may be involved in the initial decomposition of monosaccharides in anoxic micro zones of aerated forest soils