Recently, Vaz et al. reported four families with complex hereditary spastic paraplegia (cHSP) and biallelic variants in PCYT2 encoding CTP: phosphoethanolamine cytidylyltransferase (ET), the rate-limiting enzyme for phosphatidylethanolamine biosynthesis. Patient-derived fibroblasts and plasma had significant abnormalities in both neutral etherlipid and etherphospholipid metabolism (Vaz et al., 2019). We wish to broaden the phenotypic and genetic spectrum of PCYT2-related disorders with two additional patients. Clinical features are detailed in Table 1. ; This study was supported by the Centre for Biomedical Research on Rare Diseases (CIBERER) [ACCI19-759], the URDCat program (PERIS SLT002/16/00174), the Hesperia Foundation and the Secretariat for Universities and Research of the Ministry of Business and Knowledge of the Government of Catalonia [2017SGR1206] to A.P., and Instituto de Salud Carlos III [PI14/00581] (Co-funded by European Regional Development Fund. V.V., E.V. and L.P.S. were funded by grants from Instituto de Salud Carlos III, co-funded by European Social Fund. ESF investing in your future (Rio Hortega, CM18/00145; Sara Borrell, CD19/00221; PFIS, FI18/00141) ; Peer reviewed
How can we explain that some Popular education militants are also referring to the Information Society and thus seem to join this plan, carried to a great extent by merchants and the authorities ? Which are the stakes at work in this "meeting" ? Popular education, in addition to a long and plural history, is not homogeneous. However, Popular education is marked by a common philosophy aiming at developing social, cultural and political people's emancipation. In the mean time, political and economic authorities need to get the support of social actors to carry out the Information Society. Within this framework, associations would be the relay of the development of this society ; the necessary social mediator of this plan. Meanwhile, Popular education movements are seeking ways to appropriate this concept in order to make it able to serve the interests of Popular education. But they also question the specific purposes of this model. Indeed, the reference to the Information Society allows the militants of Popular education to update their traditional matters, and also to come out of the crisis they are facing. Lastly, if this meeting seems, at first sight, to generate consensus, the inherent conflicts in the confrontation of the values and identities do not therefore disappear and question the real stakes at work.
In 1985 the French government created a unique circuit for the dissemination of doctoral theses: References went to a national database "Téléthèses" whereas the documents were distributed to the university libraries in microform. In the era of the electronic document this French network of deposit of and access to doctoral theses is changing. How do you discover and locate a French thesis today, how do you get hold of a paper copy and how do you access the full electronic text? What are the catalogues and databases referencing theses since the disappearance of "Téléthèses"? Where are the archives, and are they open? What is the legal environment that rules the emerging structures and tools? This paper presents national plans on referencing and archiving doctoral theses coordinated by the government as well as some initiatives for creating full text archives. These initiatives come from universities as well as from research institutions and learned societies. "Téléthèses" records have been integrated in a union catalogue of French university libraries SUDOC. University of Lyon-2 and INSA Lyon developed procedures and tools covering the entire production chain from writing to the final access in an archive: "Cyberthèses" and "Cither". The CNRS Centre for Direct Scientific Communication at Lyon (CCSD) maintains an archive ("TEL") with about 2000 theses in all disciplines. Another repository for theses in engineering, economics and management called "Pastel" is proposed by the Paris Institute of Technology (ParisTech), a consortium of 10 engineering and commercial schools of the Paris region.
ANPCyT, Argentina ; YerPhI, Armenia ; ARC, Australia ; BMWFW, Austria ; FWF, Austria ; ANAS, Azerbaijan ; SSTC, Belarus ; Conselho Nacional de Desenvolvimento Científico e Tecnológico (CNPq) ; Fundação de Amparo à Pesquisa do Estado de São Paulo (FAPESP) ; NSERC, Canada ; NRC, Canada ; CFI, Canada ; CERN ; CONICYT, Chile ; CAS, China ; MOST, China ; NSFC, China ; COLCIENCIAS, Colombia ; MSMT CR, Czech Republic ; MPO CR, Czech Republic ; VSC CR, Czech Republic ; DNRF, Denmark ; DNSRC, Denmark ; IN2P3-CNRS, CEA-DRF/IRFU, France ; SRNSFG, Georgia ; BMBF, Germany ; HGF, Germany ; MPG, Germany ; GSRT, Greece ; RGC, Hong Kong SAR, China ; ISF, Israel ; Benoziyo Center, Israel ; INFN, Italy ; MEXT, Japan ; JSPS, Japan ; CNRST, Morocco ; NWO, Netherlands ; RCN, Norway ; MNiSW, Poland ; NCN, Poland ; FCT, Portugal ; MNE/IFA, Romania ; MES of Russia, Russian Federation ; NRC KI, Russian Federation ; JINR ; MESTD, Serbia ; MSSR, Slovakia ; ARRS, Slovenia ; MIZS, Slovenia ; DST/NRF, South Africa ; MINECO, Spain ; SRC, Sweden ; Wallenberg Foundation, Sweden ; SERI, Switzerland ; SNSF, Switzerland ; Canton of Bern, Switzerland ; MOST, Taiwan ; TAEK, Turkey ; STFC, United Kingdom ; DOE, United States of America ; NSF, United States of America ; BCKDF, Canada ; CANARIE, Canada ; CRC, Canada ; Compute Canada, Canada ; COST, European Union ; ERC, European Union ; ERDF, European Union ; Horizon 2020, European Union ; Marie Sk lodowska-Curie Actions, European Union ; Investissements d' Avenir Labex and Idex, ANR, France ; DFG, Germany ; AvH Foundation, Germany ; Greek NSRF, Greece ; BSF-NSF, Israel ; GIF, Israel ; CERCA Programme Generalitat de Catalunya, Spain ; Royal Society, United Kingdom ; Leverhulme Trust, United Kingdom ; BMBWF (Austria) ; FWF (Austria) ; FNRS (Belgium) ; FWO (Belgium) ; Coordenação de Aperfeiçoamento de Pessoal de Nível Superior (CAPES) ; Fundação de Amparo à Pesquisa do Estado do Rio de Janeiro (FAPERJ) ; FAPERGS (Brazil) ; MES (Bulgaria) ; CAS (China) ; MoST (China) ; NSFC (China) ; COLCIENCIAS (Colombia) ; MSES (Croatia) ; CSF (Croatia) ; RPF (Cyprus) ; SENESCYT (Ecuador) ; MoER (Estonia) ; ERC IUT (Estonia) ; ERDF (Estonia) ; Academy of Finland (Finland) ; MEC (Finland) ; HIP (Finland) ; CEA (France) ; CNRS/IN2P3 (France) ; BMBF (Germany) ; DFG (Germany) ; HGF (Germany) ; GSRT (Greece) ; NKFIA (Hungary) ; DAE (India) ; DST (India) ; IPM (Iran) ; SFI (Ireland) ; INFN (Italy) ; MSIP (Republic of Korea) ; NRF (Republic of Korea) ; MES (Latvia) ; LAS (Lithuania) ; MOE (Malaysia) ; UM (Malaysia) ; BUAP (Mexico) ; CINVESTAV (Mexico) ; CONACYT (Mexico) ; LNS (Mexico) ; SEP (Mexico) ; UASLP-FAI (Mexico) ; MOS (Montenegro) ; MBIE (New Zealand) ; PAEC (Pakistan) ; MSHE (Poland) ; NSC (Poland) ; FCT (Portugal) ; JINR (Dubna) ; MON (Russia) ; RosAtom (Russia) ; RAS (Russia) ; RFBR (Russia) ; NRC KI (Russia) ; MESTD (Serbia) ; SEIDI (Spain) ; CPAN (Spain) ; PCTI (Spain) ; FEDER (Spain) ; MOSTR (Sri Lanka) ; MST (Taipei) ; ThEPCenter (Thailand) ; IPST (Thailand) ; STAR (Thailand) ; NSTDA (Thailand) ; TAEK (Turkey) ; NASU (Ukraine) ; SFFR (Ukraine) ; STFC (United Kingdom ; DOE (U.S.A.) ; NSF (U.S.A.) ; Marie-Curie programme ; Horizon 2020 Grant (European Union) ; Leventis Foundation ; A.P. Sloan Foundation ; Alexander von Humboldt Foundation ; Belgian Federal Science Policy Office ; Fonds pour la Formation a la Recherche dans l'Industrie et dans l'Agriculture (FRIA-Belgium) ; Agentschap voor Innovatie door Wetenschap en Technologie (IWT-Belgium) ; F.R.S.-FNRS (Belgium) ; Beijing Municipal Science & Technology Commission ; Ministry of Education, Youth and Sports (MEYS) of the Czech Republic ; Hungarian Academy of Sciences (Hungary) ; New National Excellence Program UNKP (Hungary) ; Council of Science and Industrial Research, India ; HOMING PLUS programme of the Foundation for Polish Science ; European Union, Regional Development Fund ; Mobility Plus programme of the Ministry of Science and Higher Education ; National Science Center (Poland) ; National Priorities Research Program by Qatar National Research Fund ; Programa Estatal de Fomento de la Investigacion Cientfica y Tecnica de Excelencia Maria de Maeztu ; Programa Severo Ochoa del Principado de Asturias ; EU-ESF ; Greek NSRF ; Rachadapisek Sompot Fund for Postdoctoral Fellowship, Chulalongkorn University (Thailand) ; Chulalongkorn Academic into Its 2nd Century Project Advancement Project (Thailand) ; Welch Foundation ; Weston Havens Foundation (U.S.A.) ; Canton of Geneva, Switzerland ; Herakleitos programme ; Thales programme ; Aristeia programme ; European Research Council (European Union) ; Horizon 2020 Grant (European Union): 675440 ; FWO (Belgium): 30820817 ; Beijing Municipal Science & Technology Commission: Z181100004218003 ; NKFIA (Hungary): 123842 ; NKFIA (Hungary): 123959 ; NKFIA (Hungary): 124845 ; NKFIA (Hungary): 124850 ; NKFIA (Hungary): 125105 ; National Science Center (Poland): Harmonia 2014/14/M/ST2/00428 ; National Science Center (Poland): Opus 2014/13/B/ST2/02543 ; National Science Center (Poland): 2014/15/B/ST2/03998 ; National Science Center (Poland): 2015/19/B/ST2/02861 ; National Science Center (Poland): Sonata-bis 2012/07/E/ST2/01406 ; Programa Estatal de Fomento de la Investigacion Cientfica y Tecnica de Excelencia Maria de Maeztu: MDM-2015-0509 ; Welch Foundation: C-1845 ; This paper presents the combinations of single-top-quark production cross-section measurements by the ATLAS and CMS Collaborations, using data from LHC proton-proton collisions at = 7 and 8 TeV corresponding to integrated luminosities of 1.17 to 5.1 fb(-1) at = 7 TeV and 12.2 to 20.3 fb(-1) at = 8 TeV. These combinations are performed per centre-of-mass energy and for each production mode: t-channel, tW, and s-channel. The combined t-channel cross-sections are 67.5 +/- 5.7 pb and 87.7 +/- 5.8 pb at = 7 and 8 TeV respectively. The combined tW cross-sections are 16.3 +/- 4.1 pb and 23.1 +/- 3.6 pb at = 7 and 8 TeV respectively. For the s-channel cross-section, the combination yields 4.9 +/- 1.4 pb at = 8 TeV. The square of the magnitude of the CKM matrix element V-tb multiplied by a form factor f(LV) is determined for each production mode and centre-of-mass energy, using the ratio of the measured cross-section to its theoretical prediction. It is assumed that the top-quark-related CKM matrix elements obey the relation |V-td|, |V-ts| « |V-tb|. All the |f(LV)V(tb)|(2) determinations, extracted from individual ratios at = 7 and 8 TeV, are combined, resulting in |f(LV)V(tb)| = 1.02 +/- 0.04 (meas.) +/- 0.02 (theo.). All combined measurements are consistent with their corresponding Standard Model predictions.