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Understanding neurodevelopmental proteasomopathies as new rare disease entities:A review of current concepts,molecular biomarkers,and perspectives

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Genes & Diseases 2024年第6期11卷 168-191页

作者： Silvestre Cuinat Stephane Bezieau Wallid deb Sandra Mercier Virginie Vignard Bertrand Isidor Sebastien Kury Frederic Ebstein nantes universite CHU NantesService de Genetique MedicaleNantes F-44000France nantes universite CHU NantesCNRSINSERMl'institut du thoraxNantes F-44000France

The recent advances in high throughput sequencing technology have drastically changed the practice of medical diagnosis,allowing for rapid identification of hundreds of genes causing human *** unprecedented progress has made clear that most forms of intellectual disability that affect more than 3%of individuals worldwide are monogenic ***,a substantial fraction of the mendelian forms of intellectual disability is asso-ciated with genes related to the ubiquitin-proteasome system,a highly conserved pathway made up of approximately 1200 genes involved in the regulation of protein *** this group is currently emerging a new class of neurodevelopmental disorders specifically caused by proteasome pathogenic variants which we propose to designate"neurodevelopmen-tal proteasomopathies".Besides cognitive impairment,these diseases are typically associated with a series of syndromic clinical manifestations,among which facial dysmorphism,motor delay,and failure to thrive are the most prominent *** recent efforts have been made to uncover the effects exerted by proteasome variants on cell and tissue landscapes,the mo-lecular pathogenesis of neurodevelopmental proteasomopathies remains *** this re-view,we discuss the cellular changes typically induced by genomic alterations in proteasome genes and explore their relevance as biomarkers for the diagnosis,management,and potential treatment of these new rare disease entities.

关键词： Biomarkers Loss-of-function variants Neurodevelopmental disorders Proteasome Rare diseases Therapeutic targets

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A fully bio-sourced adsorbent of heavy metals in water fabricated by immobilization of quinine on cellulose paper

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Journal of Environmental Sciences 2019年第10期31卷 174-183页

作者： Ehu C.Aka Medy C.Nongbe Tchirioua Ekou Lynda Ekou Vincent Coeffard Francois-Xavier Felpin universite de nantes CNRS UMR 6230CEISAM2 rue de la Houssinière44322 Nantes Cedex 3France universite Nangui Abrogoua Laboratoire de Thermodynamique et de Physico-Chimie du Milieu02 BP 801 Abidjan 02Cote d'lvoire

The fabrication of a fully bio-sourced adsorbent of Cd(Ⅱ)by covalent immobilization of quinine on cellulose paper is *** double bond of commercially available quinine was converted to a terminal alkyne function which was reacted with cellulose paper,chemically modified with azide functions,through a 1,3-dipolar cycloaddition,leading to *** adsorption efficiency of Cell-Quin was investigated to determine the optimal pH,contact time and dose of adsorbent,ultimately leading to high levels of *** mechanism of adsorption of Cell-Quin was deeply rationalized through kinetic experiments and isotherm *** also showed that Cell-Quin could adsorb other heavy metals such as Cu(Ⅱ),Pb(Ⅱ),Ni(Ⅱ)and Zn(Ⅱ).

关键词： Cellulose paper Quinine Heavy metal removal Adsorbent

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Assessment of water body change and sedimentation rate in Moulay Bousselham wetland, Morocco, using geospatial technologies

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International Journal of Sediment Research 2019年第1期34卷 65-72页

作者： Mounir Karim Mehdi Maanan Mohamed Maanan Hassan Rhinane Henri Rueff Lahssen Baidder Hassan Ⅱ University of Casablanca Faculty of Sciences An ChockCeosciences Laboratory universite de nantes Institut de Geographie University of Basel Department of Environmental Sciences

Coastal lagoons have significant biodiversity and ecologic and socio-economic interest. Several resident and migratory birds live in coastal lagoons as well as fish and other aquatic biota. Over the years, lagoons worldwide have been subject to considerable sediment deposition coming especially from soil erosion due of agricultural activities, and the lagoon’s inflows have resulted in an accelerated sedimentation which can drastically affect the aquatic ecosystem balance. A new, fast, and cost-effective approach was developed, using water surface change as a proxy, to evaluate the sediment deposition rate. In the current research, this methodology was applied in the Moulay Bousselham Lagoon in Morocco, and a combination of Landsat 8 and Landsat 5 imagery acquired at four points in time over 30 years(1985-2015) was used. Following geometric correction and enhancement, the interpretation of the acquired images provided important insight on the current conditions of the water surface change using the Normalized Difference Water Index(NDWI). Several iterations were done to determine a suitable threshold for effective water surface detection. In order to validate the automatically extracted water surface features, many band combinations of Landsat and Sentinel 2 were used as references to run the accuracy assessment. The results show that the Moulay Bousselham Lagoon lost 17.60%(106 ha) of its water surface area, and 530,000 m~3 to 1,060,000 m~3 of its water volume during the last three *** water surface loss into sedimentation returned a rate of 0.58 cm/yr. These results have been confirmed by previous field work using sedimentological approaches. Proper management should be applied to sustain the vitality of this lagoon environment.

关键词： Environmental monitoring Remote sensing GIS Spatial analyst Human/nature interaction Sustainability planning

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Protective behaviors regarding coastal flooding risk in a context of climate change

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Advances in Climate Change Research 2020年第4期11卷 310-316页

作者： Colin LEMEE Oscar NAVARRO Diego RESTREPO-OCHOA denis MERCIER Ghozlane FLEURY-BAHI University of Brest Brest29200France Laboratoire de Psychologie des Pays de La Loire University of NantesNantes44312France CES University Medellin050022Colombia Laboratoire de Geographie Physique(UMR 8591-CNRS) Sorbonne UniversiteParis75000France

The physical vulnerability of coastal areas due to rising sea level and the flooding risk consequent,does not guarantee the implementation of protective behaviors by these risk zones’*** study aims to establish the link between the willingness to carry out protective behaviors and physical and perceived indicators of vulnerability.A typology of coastal flooding vulnerability,uses various physical indicators and their perceived counterparts which have been collected from 490 inhabitants of Cartagena(Colombia,declared world heritage of humanity by UNESCO in 1984),resident in areas of coastal flooding *** item-response theory(IRT)approach has been *** results reveal that the implementation of protective behaviors is more related to perceived indicators,such as distance to the sea,than to actual physical *** observe that physical vulnerability is linked to the intention to carry out protective *** presence of a defensive structure against coastal flooding could be considered as a visual cue and be a good predictor of the willingness to carry out protective *** the contrary,people in the most vulnerable situation(single-storey house)do not demonstrate a higher level of willingness to carry out protective behavior,as well of participants who lived in residential buildings which have demonstrated lower level of willingness to carry out such ***,vulnerability of the house is not seen as a criterion that encourages participants to better protect themselves.

关键词： Coastal flooding risk Protective behaviors Climate change Vulnerability Item response theory

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Modelling of photovoltaic production and electrochemical storage in an autonomous solar drone

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eScience 2022年第2期2卷 235-241页

作者： Mickael Cosson Benjamin David Ludovic Arzel Philippe Poizot Ahmed Rhallabi nantes universite CNRSInstitut des Materiaux de Nantes Jean Rouxel2 rue de la Houssiniere44322NantesFrance XSun 1 Route de la Croix Moriau44350GuerandeFrance

A simple,efficient simulator has been developed to predict the generation of photovoltaic energy and its storage in Li-ion batteries,for an autonomous drone with four wings covered by solar panels based on thin-film gallium arsenide photovoltaic cells(III–V).This simulator allows prediction of the effective photovoltaic power produced by the solar panels as well as the battery pack voltage when the drone is *** parameters such as irra-diance,sun inclination angles,and drone Euler angles are considered as input *** measured photovoltaic power and battery pack voltage are in good agreement with the simulated values,making practical use by the XSun company *** parametric study shows the effects of climatic and geographic conditions on drone *** optimal weather conditions on a sunny day,drone flight can last 12 h.

关键词： Unmanned aerial vehicle Drone flight simulator Simulation Energy storage Photovoltaic energy

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Electrical Conductivity and Current-Voltage Characteristics of Individual Conducting Polymer PEDOT Nanowires

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Chinese Physics Letters 2008年第9期25卷 3474-3477页

作者：龙云泽 DUVAIL Jean-Luc 陈兆甲金爱子顾长志 College of Physics Science Qingdao University Qingdao 266071 Institut des Materiaux Jean Rouxel Universite de Nantes CNRS 44322 Nantes Prance Beijing National Laboratory for Condensed Matter Physics Institute of Physics Chinese Academy of Sciences Beijing 100190

We report the current-voltage （Ⅰ-Ⅴ） characteristics and electrical conductivity of individual template-synthesized poly（3,4-ethylenedioxythiophene）（PEDOT） nanowires （190 ± 6 nm in diameter and δRT = 11.2 ± 2Ω^-1 cm^-1） over a wide temperature range from 300 to 10K. With lowering temperature, the Ⅰ - Ⅴ characteristics become nonlinear around 50K, and a clear Coulomb gap-like structure appears in the differential conductance （dI/dV） spectra. The temperature dependence of the resistance below 70 K follows In R α T^-1/2, which can be interpreted as Efros-Shklovskii hopping conduction in the presence of a Coulomb gap. In addition, the influences of measurement methods such as the applied bias voltage magnitude, the two-probe and four-probe techniques used in the resistance measurements are also reported and discussed.

关键词： ELECTRIC conductivity CONDUCTING polymers NANOWIRES ELECTRIC potential HOPPING conduction THIOPHENES

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TIME-deLAY AND SPECTRAL deNSITY FOR STARK HAMILTONIANS(Ⅱ)——ASYMPTOTICS OF TRACE FORMULAE

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Chinese Annals of Mathematics,Series B 1991年第3期12卷 358-383页

作者： D.ROBERT 王雪平 departement de Mathematiques Universite de Nantes 44072 Nantes Cedex 03 France. department of Mathematics Beijing University Beijing China.

This paper studies the Schrodinger operator with a homogeneous electric field of the form -△+x1+V（x）, where x= （x1,…, xn）∈Rn. It is proved that in the specctral representation of the free Stark Hamiltonian, the time-delay operator in scattering theory can be expressed in trems of scattering matrix and under reasonable assumptions on the decay of the potential V, the on-shell time-delay operator is of trace class and its trace is related to the local spectral density via an explicit integral formula. Some asymptotics for the trace are estabhshed when the energy tends to infinity.

关键词： infinity explicit Hamiltonian assumptions Stark decay 刁刀 derive kernel 二尹

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Improved photoconductive properties of composite nanofibers based on aligned conjugated polymer and single-walled carbon nanotubes

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Nano Research 2013年第2期6卷 149-158页

作者： Florian Massuyeau Yuanchun Zhao Abdel Aziz El Mel Abu Yaya Frederic Geschier Eric Gautron Serge Lefrant Jean Yves Mevellec Chris Ewels Chain-Shu Hsu Eric Faulques Jany Wery Jean Luc Duvail fnstitut des Materiaux Jean Rouxel UMR6502 CNRS Universite de Nantes 2 rue de la Houssiniere F-44322 Nantes France department of Applied Chemistry Chiao Tung University 1001 Ta Hsueh Road Hsin-Chu 30010 Taiwan China

We successfully address the challenge of aligning single-walled carbon nanotubes （SWNTs） and conjugated polymer chains in composite nanofibers for enhancing their opto-electrical properties. A pore-filling template strategy has been developed to prepare such nanocomposites from SWNTs and poly（para-phenylene vinylene）（PPV） chains, with both species well-oriented aligned along the pore axis. Addition of the SWNTs leads to a remarkable increase in photocurrent of four orders of magnitude as compared to equivalent pristine PPV nanofibers. Further analysis indicates that the strong photocurrent enhancement is not simply an effect of alignment, but additionally benefits from alignment-enhanced interaction of polymer chains with SWNTs, as supported by density functional theory （DFT） calculations.

关键词： Tubular nanocomposites single-walled carbonnanotube (SWNT) photoconductivity transport properties conjugated polymer density functional theory (DFT)calculation

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Interest of molecular functionalization for electrochemical storage

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Nano Research 2017年第12期10卷 4175-4200页

作者： Bihag Anothumakkool Dominique Guyomard Joel Gaubicher Lenaic Madec Institut des Materiaux Jean Rouxel (IMN) Universite de Nantes CNRS 2 rue de la Houssiniere BP32229 44322 Nantes Cedex 3 France IPREM-ECP CNRS UMR 5254 Universite de Pau Helioparc Pau Pyrenees 2 av. PierreAngot 64053 Pau Cedex 9 France Reseau sur le Stockage Electrochimique de I'Energie (RS2E) CNRS FR3459 33 Rue Saint Leu 80039 Amiens Cedex France

despite great interests in electrochemical energy storage systems for numerous applications, considerable challenges remain to be overcome. Among the various approaches to improving the stability, safety, performance, and cost of these systems, molecular functionalization has recently been proved an attractive method that allows the tuning of material surface reactivity while retaining the properties of the bulk material. For this purpose, the reduction of aryldiazonium salt, which is a versatile method, is considered suitable; it forms robust covalent bonds with the material surface, however, with the formation of multilayer structures and sp3 defects （for carbon substrate） that can be detrimental to the electronic conductivity. Alternatively, non-covalent molecular functionalization based on π-π interactions using aromatic ring units has been proposed. In this review, the various advances in molecular functionalization concerning the current limitations in lithium-ion batteries and electrochemical capacitors are discussed. According to the targeted applications and required properties, both covalent and non-covalent functionalizafion methods have proved to be very efficient and versatile. Fundamental aspects to achieve a better understanding of the funcfionalization reactions as well as molecular layer properties and their effects on the electrochemical performance are also discussed. Finally, perspectives are proposed for future implementation of molecular functionalization in the field of electrochemical storage.

关键词： electrochemical storage molecularfunctionalization covalent diazoniumchemistry non-covalent π-π interactions

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NumericalMethods for Balance Laws with Space dependent Flux:Application to Radiotherapy Dose Calculation

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Communications in Computational Physics 2011年第10期10卷 1184-1210页

作者： Christophe Berthon Martin Frank Celine Sarazin Rodolphe Turpault universite de nantes Laboratoire de Mathematiques Jean LerayUMR66292 rue de la HoussiniereBP 9220844322 Nantes Cedex 3France RWTH Aachen University MathematicsCenter for Computational Engineering ScienceSchinkelstrasse 252062 AachenGermany

The present work is concerned with the derivation of numerical methods to approximate the radiation dose in external beam *** address this issue,we consider a moment approximation of radiative transfer,closed by an entropy minimization *** model under consideration is governed by a system of hyperbolic equations in conservation form supplemented by source *** main difficulty coming from the numerical approximation of this system is an explicit space dependence in the flux ***,this dependence will be seen to be stiff and specific numerical strategiesmust be derived in order to obtain the needed accuracy.A first approach is developed considering the 1D case,where a judicious change of variables allows to eliminate the space dependence in the flux *** is not possible in *** therefore reinterpret the 1D scheme as a scheme on two meshes,and generalize this to 2D by alternating transformations between separate *** call this procedure projection *** numerical experiments,coming from medical physics,illustrate the potential applicability of the developed method.

关键词： Radiotherapy hyperbolic system of conservation laws source term approximations finite volume methods

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