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Multi-tissue transcriptomics of a unique monozygotic discordant twin case of severe progressive osseous heteroplasia

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Genes & Diseases 2024年第3期11卷 22-25页

作者： Alberto Gomez-Carballa Maria jose Curras-Tuala Sara Pischedda Miriam Cebey-lopez jose Gomez-Rial Irene Rivero-Cale Jacobo Pardo-seco Xabier Bello Sandra Viz-lasheras antonio Justicia-Grande Julian Montoto-louzao Alba Camino-Mera Isabel Ferreiros-Vidal Maximo Fraga jose RAntunez Rodolfo Gomez Federico Martinon-Torres antonio Salas Genetics Vaccines and Infections Research Group(GENVIP)Instituto de Investigacion Sanitaria de SantiagoSantiagode CompostelaGalicia 15706Spain Translational Pediatrics and Infectious Diseases Department of PediatricsHospital Clinico Universitario de Santiago de CompostelaServicio Galego de Saude(SERGAS)Galicia 15706Spain laboratorio de Inmunologia Servicio de Andlisis ClinicosHospital Clinico Universitario de Santiago de CompostelaServicio Galego de Saude(SERGAS)Galicia 15706Spain Unidade de Xenetica Instituto de Ciencias Forenses(INCIFOR)Facultade de MedicinaUniversidade de Santiago de Compostelaand GenPoB Research GroupInstituto de Investigacion SanitariaHospital Clinico Universitario de Santiago de CompostelaServicio Galego de Saude(SERGAS)Galicia 15706Spain Musculoskeletal Pathology Group Instituto de Investigacion Sanitaria de SantiagoServicio Galego de Saude(SERGAS)Santiago de CompostelaGalicia 15706Spain Department of Pathology Hospital Clinico Universitario de Santiago de Compostela(SERGAS)Galicia 15706Spain Unidad Integrada de BiobancosBiomodelos 3D y Modelos Animales IDIS(lnstituto de Investigacion Sanitaria de Santiagode Compostela)Galicia15706Spain Centro de Investigacion Biomedica en Red de Enfermedades Respiratorias(CIBER-ES) MadridSpain

Progressive osseous heteroplasia(POH)is an ultra-rare autosomal dominant disabling disorder characterized by heterotopic ossification(HO).It is caused by heterozygous inactivating mutations in the GNAS(guanine nucleotide-binding protein alpha-stimulating activity polypeptide)***,the molecular mechanisms underlying HO remain poorly *** a treatment for POH is not yet available,the identification of the mechanisms driving POH in affected tissues using gene expression may be of great help to underestand the molecular basis of POH and develop new therapeutic approaches.

关键词： stimulating driving asi

Twenty-four-hour movement guidelines during middle adolescence and their association with glucose outcomes and type 2 diabetes mellitus in adulthood

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Journal of Sport and Health Science 2023年第2期12卷 167-174页

作者： antonio García-Hermoso joséFrancisco lópez-Gil Yasmin Ezzatvar Robinson Ramírez-Vélez Mikel Izquierdo Navarrabiomed Hospital Universitario de Navarra(HUN)Universidad Publica de Navarra(UPNA)IdiSNAPamplona 31008Spain Health and Social Research Center Universidad de Castilla-La ManchaCuenca 16007Spain Department of Nursing Universitat de ValenciaValencia 46010Spain

Purpose:The aim of the present study was to determine the association between adherence to the 24-h movement guidelines during middle adolescence and glucose outcomes(glycated hemoglobin and fasting glucose)and type 2 diabetes mellitus(T2DM)in adulthood,14 and 22 years ***:We analyzed data from apparently healthy adolescents aged 12-18 years who participated in WavesⅠandⅡ(1994-1996,n=14,738),WaveⅣ(2008-2009,n=8913),and Wave V(2016-2018,n=3457)of the National longitudinal Study of Adolescent to Adult Health(Add Health)in the United *** activity,screen time,and sleep duration were measured using questionnaires,and the 24-h guidelines were defined as:5 or more times moderate-to-vigorous physical activity per week,≤2 h per day of screen time,and 9-11 h of sleep for 12-13 years and 8-10 h for 14-17 *** and venous whole blood was collected and analyzed to determine glycated hemoglobin and fasting glucose for WavesⅣandⅤ,***:Only 2.1%of the adolescents met all the 3 guidelines,and 37.8%met none of *** both wavesⅣandⅤ,adolescents who met physical activity and screen time guidelines had lower odds of T2DM in adulthood than those who did not meet any of these guidelines(WaveⅣ;prevalence ratio(PR)=0.57,95%confidence interval(95%CI):0.21-0.89;Wave V:PR=0.43,95%CI:0.32-0.74).Only for Wave V did adolescents who met all 3 guidelines have lower odds of T2DM at follow-up compared with those who did not meet any of these guidelines(PR=0.47,95%CI:0.24-0.91).Also,for each increase in meeting one of the 24-h recommendations,the odds of T2DM decreased by 18%(PR=0.82,95%CI:0.61-0.99)and 15%(PR=0.85,95%CI:0.65-0.98)in adulthood for WavesⅣandⅤ,***:Promoting all 24-h movement guidelines in adolescence,especially physical activity and screen time,is important for lowering the potential risk of T2DM in adulthood.

关键词： Glucose metabolism Glycated hemoglobin Physical activity Screen time Sleep duration

Recent Advances on Early-Stage Fire-Warning Systems: Mechanism, Performance, and Perspective

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Nano-Micro letters 2022年第12期14卷 106-136页

作者： Xiaolu li antonio Vazquez-lopez jose Sanchez del Rio Saez De-Yi Wang IMDEA Materials Institute C/Eric Kandel228906 GetafeMadridSpain E.T.S.de Ingenieros de Caminos Universidad Politecnica de MadridCalle Profesor Aranguren 328040 MadridSpain Departamento de Ingenieria Electrica Electronica Automatica y Fisica AplicadaETSIDIUniversidad Politecnica de MadridRonda de Valencia 328012 MadridSpain

Early-stage fire-warning systems(EFWSs)have attracted significant attention owing to their superiority in detecting fire situations occurring in the pre-combustion *** progress on EFWSs has been achieved recently,and they have presented a considerable possibility for more evacuation time to control constant unintentional fire hazards in our daily *** review mainly makes a comprehensive summary of the current EFWSs,including the working mechanisms and their *** to the different working mechanisms,fire alarms can be classified into graphene oxide-based fire alarms,semiconductor-based fire alarms,thermoelectric-based fire alarms,and fire alarms on other working ***,the challenge and prospect for EFWSs are briefly provided by comparing the art of state of fire *** work can propose a more comprehensive understanding of EFWSs and a guideline for the cutting-edge development direction of EFWSs for readers.

关键词： Smart thermosensitive fire sensors Working mechanism Response time Signal conversion

Prevalence of meeting 24-Hour Movement Guidelines from pre-school to adolescence: A systematic review and meta-analysis including 387,437 participants and 23 countries

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Journal of Sport and Health Science 2022年第4期11卷 427-437页

作者： Miguel Angel Tapia-Serrano Javier Sevil-Serrano Pedro antonio Sanchez-Miguel jose Francisco lopez-Gil Mark STremblay antonio Garcıa-Hermoso Department of Didactics of Musical Plastic and Body ExpressionFaculty of Teaching TrainingUniversity of ExtremaduraAvenida de la UniversidadS/NCaceres 10071Spain Department of Physical Activity and Sport Faculty of Sport ScienceUniversity of Murcia(UM)San Javier 30720Spain Healthy Active living and Obesity Research Group Children’s Hospital of Eastern Ontario(CHEO)Research InstituteOttawaON K1H 8L1Canada Navarrabiomed Public University of Navarra(UPNA)Health Research Institute of Navarra(IdiSNA)Navarra Hospital Complex(CHN)Pamplona 310008Spaia

Background:Meeting the 24-Hour Movement Guidelines(physical activity,screen time,and sleep duration) has been associated with positive health ***,there are no previous meta-analyses that have examined the overall adherence to the 24-Hour Movement Guidelines from pre-school to adolescence across the ***,the main purpose of this systematic review and meta-analysis was to examine the overall(non)adherence to the 24-Hour Movement Guidelines among preschoolers,children,and adolescents ***:Four electronic databases(MEDlINE,Scopus,Web of Science,and Cochrane Database of Systematic Reviews) were searched for quantitative studies published in Spanish and English between January 2016 and May *** that were conducted with apparently healthy participants and reported the overall(non)adherence to the 24-Hour Movement Guidelines in preschoolers and/or children and/or adolescents were ***:Sixty-three studies comprising 387,437 individuals(51% girls) aged 3-18 years from 23 countries were ***,7.12%(95%confidence interval(95%CI):6.45%-7.78%) of youth met all three 24-Hour Movement Guidelines,and 19.21%(95%CI:16.73%-21.69%)met none of the 3 *** sex,adherence to all recommendations was significantly lower in girls(3.75%,95%CI:3.23%-4.27%) than in boys(6.89%,95%CI:5.89%-7.89%)(p l recommendations(girls,15.66%,95%CI:8.40%-22.92%;boys,12.95%,95%CI:6.57%-19.33%).In terms of age group,adherence to the 3 recommendations was 11.26%(95%CI:8.68%-13.84%),10.31%(95%CI:7.49%-13.12%),and 2.68%(95%CI:1.78%-3.58%) in preschoolers,children,and adolescents,***,8.81%(95%CI:5.97%-11.64%) of preschoolers,15.57%(95%CI:11.60%-19.54%) of children,and 28.59%(95%CI:22.42%-34.75%) of adolescents did not meet any of the *** America was the region with the lowest adherence(all:2.93%;none:3

关键词： Exercise lifestyle Movement behaviors Sedentary behaviors Youth

Tropical tree community composition and diversity variation along a volcanic elevation gradient

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Journal of Mountain Science 2022年第12期19卷 3475-3486页

作者： Ruben MARTINEZ-CAMIlO Manuel MARTINEZ-MElENDEZ Nayely MARTINEZ-MIElENDEZ Derio antonio JIMENEZ-lOpez Mauricio josE-RIOS Unidad Villa Corzo Facultad de IngenieríaUniversidad de Ciencias y Artes de Chiapas30520 Villa CorzoChiapasMexico Instituto de Ciencias Biológicas Universidad de Ciencias y Artes de Chiapas29039 Tuxtla GutiérrezChiapasMexico Eizia A.C. 29045 Tuxtla GutiérrezChiapasMexico Departamento de Conservación de la Biodiversidad El Colegio de la Frontera Sur29290 San Cristóbal de las CasasChiapasMexico Orquidiario y Jardín Botánico de Comitán Secretaría de Medio Ambiente e Historia Natural30106 Comitán de DomínguezChiapasMexico Instituto de Investigación en Gestión de Riesgos y Cambio Climático Universidad de Ciencias y Artes de Chiapas29039 Tuxtla GutiérrezChiapasMexico

Unraveling the factors that determine variation of diversity in tropical mountain systems is a topic for debate in plant *** is especially true in areas where topography is complex due to volcano elevational gradients and where forests are vulnerable to human *** this study we used a set of climatic(temperature,rainfall,and radiation solar),topographic(elevation,slope aspect,and slope orientation)and human disturbance variables to determine their effect on diversity and composition patterns of a tree community,considering three slope aspects of a tropical volcano in southeastern *** sampled trees in seventy 0.1-ha plots distributed on three slope aspects of the Tacanávolcano along an elevational gradient of 1500 to 2500 *** determined diversity patterns(general tree richness,exponential of Shannon index,and pioneer species richness)with linear regression models,and for beta diversity,we used a dissimilarity index(within and between elevational bands 100 m wide).The effect of a set of environmental and human disturbance variables on tree diversity and community composition was analyzed with general linear models and multivariate analyses,*** registered 2,949 individual trees belonging to 176 species and *** average species richness and alpha diversity per plot were 13(standard deviation±6)and 9(±5),*** tree richness and alpha diversity increased in the middle part(unimodal patterns)of the elevational gradient,but pioneer species richness decreased linearly with *** variance explained by general linear models was greater in richness(32%)than in alpha diversity(25.3%).The most important predictor variables were temperature(elevational gradient),which explained the unimodal pattern(richness and alpha diversity increase at intermediate levels of temperature),and slope orientation,which explained the increase in richness and alpha diversity toward the geographic *** temperature had a signific

关键词： Composition Environmental gradient Tree diversity patterns Tropical humid cloud forest Tropical volcanoes

Study of reverse osmosis membranes fouling by inorganic salts and colloidal particles during seawater desalination

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Chinese Journal of Chemical Engineering 2020年第3期28卷 733-742页

作者： Santiago Gutiérrez Ruiz Juan antonio lópez-Ramírez Mohammed Hassani Zerrouk Agata Egea-Corbacho lopera joséMaría Quiroga Alonso Department of Environmental Technologies Faculty of Marine and Environmental SciencesUniversity of Cadiz11510Puerto RealCadizSpain

Fouling phenomenon is considered among the major reasons that cause significant increase of operating cost of desalination plants equipped with reverse osmosis(RO)*** phenomenon is studied in the present work in the case of RO polyamide aromatic membranes using model seawater containing inorganic salts and colloidal *** solubility conditions of CaCO3 and CaSO4 were applied to study RO performances with and without colloid *** experiments,the membrane permeate fluxes were continuously ***,studies of chemical composition,structure,and morphology of the materials deposited on the membrane surface were conducted using energy dispersive microanalysis(EDS)X-ray diffraction and scanning electronic microscopy(SEM).Results show that in conditions of calcium carbonate oversaturation there is a reduction in the permeate flow of 11.2%due to fouling of the membrane by the precipitation of this *** in the same conditions of calcium sulphate oversaturation the reduction of the flow is 5%,so we can conclude that in conditions of oversaturation of both salts,calcium carbonate produces a greater fouling of the membrane that in its view causes greater decrease in the flow of *** this based on the results of the test with both salts in oversaturated *** in the formation of calcite and gypsum crystals onto the membranes as XRD analyses *** presence of colloidal silica in those conditions intensifies strongly the fouling,leading until to 24.1%of permeate flux decrease.

关键词： Reverse osmosis Desalination Fouling Seawater Scaling

Internet of things:Conceptual network structure,main challenges and future directions

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Digital Communications and Networks 2023年第3期9卷 677-687页

作者： leonardo B.Furstenau Yan Pablo Reckziegel Rodrigues Michele Kremer Sott Pedro leivas Michael S.Dohan josé Ricardo lópez-Robles Manuel J.Cobo Nicola luigi Bragazzi Kim-Kwang Raymond Choo Department of Industrial Engineering Federal University of Rio Grande do SulPorto Alegre 90035-190Brazil University of Santa Cruz do Sul Av.Independencia2293Santa Cruz do SulRSBrazil Business School Unisinos UniversityAv.Dr.Nilo Peçanha 160091330-002Porto AlegreBrazil lakehead University Thunder BayONP7B 5E1Canada Academic Unit of Accounting and Management Autonomous University of ZacatecasCalle Comercio y Administracion S/NFracc.ProgresoC.P.98066ZacatecasZacatecasMexico Department of Computer Science and Artificial Intelligence University of Granada18071 GranadaSpain York University ONM3J 1P3Canada Department of Information Systems and Cyber Security University of Texas at San AntonioSan AntonioTX78249USA

Internet of Things(IoT)is a key technology trend that supports our digitalized society in applications such as smart countries and smart *** this study,we investigate the existing strategic themes,thematic evolution structure,key challenges,and potential research opportunities associated with the *** this study,we conduct a Bibliometric Performance and Network Analysis(BPNA),supplemented by an exhaustive Systematic literature Review(SlR).Specifically,in BPNA,the software SciMAT is used to analyze 14,385 documents and 30,381 keywords in the Web of Science(WoS)database,which was released between 2002 and *** results reveal that 31 clusters are classified according to their importance and development,and the conceptual structures of key clusters are presented,along with their performance analysis and the relationship with other *** thematic evolution structure describes the important cluster(s)over *** the SlR,23 documents are *** SlR reveals key challenges and limitations associated with the *** expect the results will form the basis of future research and guide decision-making in the IoT and other supporting industries.

关键词： Internet of things Strategic intelligence Industry 4.0 SciMAT Bibliometric analysis Science mapping

Exploring seven hundred years of transhumance, climate dynamic, fire and human activity through a historical mountain pass in central Spain

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Journal of Mountain Science 2016年第7期13卷 1139-1153页

作者： josé antonio lópez-SáEZ Francisca AlBA-SáNCHEZ Sandra ROBlES-lópez Sebastián PéREZ-DíAZ Daniel ABEl-SCHAAD Silvia SABARIEGO-RUIZ Arthur GlAIS Research Group Archaeobiology Institute of History CSIC Dept.Botany Faculty of Sciences University of Granada Dept.of Plant Biology Faculty of Biology Complutense University lETG CAEN GEOPHEN-UMR 6554 CNRS University of Caen-Normandie

A high-altitude peat sequence from the heart of the Spanish Central System(Gredos range) was analysed through a multi-proxy approach to determine the sensitivity of high-mountain habitats to climate, fire and land use changes during the last seven hundred years, providing valuable insight into our understanding of the vegetation history and environmental changes in a mountain pass close to a traditional route of transhumance. The pollen data indicate that the vegetation was dominated by shrublands and grasslands with scattered pines in high-mountain areas, while in the valleys cereals, chestnut and olive trees were cultivated. Strong declines of high-mountain pines percentages are recorded at 1540, 1675, 1765, 1835 and 1925 cal AD, which may be related to increasing grazing activities and/or the occurrence of anthropogenic fires. The practice of mountain summer farming and transhumance deeply changed and redesigned the landscape of the high altitudes in central Spain(Gredos range) since the Middle Ages, although its dynamics was influenced in some way by climate variability of the past seven centuries.

关键词：气候变化西班牙人类活动火灾历史游牧山口高山地区

Atmospheric-pressure dielectric barrier discharge generation by a full-bridge flying capacitor multilevel inverter

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Plasma Science and Technology 2017年第7期19卷 40-49页

作者： Rosendo PE?A-EGUIlUZ josé A lópez-FERNáNDEZ antonio MERCADO-CABRERA Bethsabet JARAMIllO-SIERRA Régulo lópez-CAllEJAS Benjamín RODRíGUEZ-MéNDEZ Raúl VAlENCIA-AlVARADO Allan A FlORES-FUENTES Arturo E MU?OZ-CASTRO Plasma Physics laboratory Instituto Nacional de Investigaciones Nucleares Tecnológico de Estudios Superiores de Tianguistenco Universidad Autónoma del Estado de México

A new configuration of a resonant full-bridge flying capacitor multicell inverter has been designed and constructed with the aim of achieving an extended output voltage frequency range with low harmonic distortion and reduced semiconductor commutation losses. This configuration was tested as a power supply for two different coaxial dielectric barrier discharge reactors, one of them employed for electric characterization and the other one for inorganic compound elimination in an aqueous solution. Two different gas mixtures, 90% Ar-10% 02 and 80% Ar-20% 02, were individually supplied during the experiments; the results showed a high- efficiency removal of meta-cresol （m-cresol） to the order of 98%, which was obtained by adding more oxygen to the plasma gas mixture.

关键词： plasma applications water pollution plasma sources power supply circuits electricdischarges