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Multi-tissue tr.nscr.ptomics of a unique monozygotic discor.ant twin case of sever. pr.gr.ssive osseous heter.plasia

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Genes & Diseases 2024年第3期11卷 22-25页

作者： Alber.o Gomez-Car.alla Mar.a Jose Cur.as-Tuala Sar. Pischedda Mir.am Cebey-lopez Jose Gomez-r.al Ir.ne r.ver.-Cale Jacobo Par.o-seco Xabier.Bello Sandr. Viz-Lasher.s Antonio Justicia-Gr.nde Julian Montoto-louzao Alba Camino-Mer. Isabel Fer.eir.s-Vidal Maximo fraga Jose r.ntunez r.dolfo Gomez Feder.co Mar.inon-Tor.es Antonio Salas Genetics Vaccines and Infections Research Group(GENVIP)Instituto de Investigacion Sanitaria de SantiagoSantiagode CompostelaGalicia 15706Spain Tr.nslational Pediatr.cs and Infectious Diseases Department of PediatricsHospital Clinico Universitario de Santiago de CompostelaServicio Galego de Saude(SERGAS)Galicia 15706Spain Labor.tor.o 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 Gr.up Instituto de Investigacion Sanitaria de SantiagoServicio Galego de Saude(SERGAS)Santiago de CompostelaGalicia 15706Spain Depar.ment 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 Centr. de Investigacion Biomedica en r.d de Enfer.edades r.spir.tor.as(CIBEr.ES) MadridSpain

Pr.gr.ssive osseous heter.plasia(POH)is an ultr.-r.r. autosomal dominant disabling disor.er.char.cter.zed by heter.topic ossification(HO).It is caused by heter.zygous inactivating mutations in the GNAS(guanine nucleotide-binding pr.tein alpha-stimulating activity polypeptide)***,the molecular.mechanisms under.ying HO r.main poor.y *** a tr.atment for.POH is not yet available,the identification of the mechanisms dr.ving POH in affected tissues using gene expr.ssion may be of gr.at help to under.stand the molecular.basis of POH and develop new ther.peutic appr.aches.

关键词： stimulating dr.ving asi

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Potential use of lactate for.the tr.atment of neonatal hypoxic-ischemic encephalopathy

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Neur.l r.gener.tion r.sear.h 2022年第4期17卷 788-790页

作者： Isador. D’Ávila Tassinar. Luciano Stür.er.de fraga Labor.tor. of Neur.biology and Metabolism(Neur.Met) Department of PhysiologyInstituto de Ciências Básicas da Saúde(ICBS)Federal University of Rio Grande do Sul(UFRGS) Post-Gr.duate Pr.gr.m in Physiology Instituto de Ciências Básicas da Saúde(ICBS)Federal University of Rio Grande do Sul(UFRGS) Unidade de Exper.mentação Animal Centro de Pesquisa ExperimentalHospital de Clínicas de Porto Alegre(HCPA)Porto AlegreBrazil

Function of lactate:Lactate is a thr.e-car.on molecule pr.duced by glycolytic metabolism that is a metabolic waste pr.duct with no known use in clinical ***,it is a metabolite that the body should quickly guar.ntee the ***,lactate is now r.cognized as a potential ener.y substr.te,as well as an anti-inflammator. signaling *** actions wer. fir.t r.por.ed in adult animal models with a br.in injur.,including a tr.umatic br.in injur. and cer.br.l ischemia,and have also been obser.ed in human patients(Magistr.tti and Allaman,2018).r.cently,however.two studies by independent r.sear.h gr.ups descr.bed pr.mising n eur. p r.tective r.sults fr.m the use of lactate in animal models with neonatal hypoxia-ischemia(r.umes et al.,2021;Tassinar. et al.,2020).

关键词： neonatal metabolism ischemia

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High-r.solution patter.s of palaeoenvir.nmental changes dur.ng the Little Ice Age and the Medieval Climate Anomaly in the nor.hwester. Iber.an Peninsula

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Geoscience Fr.ntier. 2020年第5期11卷 1461-1475页

作者： Daniel Castr. Mar.in Souto Mar.a Isabel fraga Eduar.o Gar.ia-r.deja Sebastian Per.z-Diaz Jose Antonio lopez Saez Xabier.Pontevedr.-Pombal Dpto.Botanica Fac.BioloxiaUniversidade de Santiago de CompostelaGaliciaSpain Dpto.Edafoloxia e Quimica Agr.cola Fac.BioloxiaUniversidade de Santiago de CompostelaGaliciaSpain Dpto.Geogr.fia Urbanismo y Ordenacion Del TerritorioETS CaminosCanales y PuertosUniversidad de CantabriaSpain GI Ar.ueobiologia Instituto de HistoriaCCHS–CSICMadridSpain

to identify plant macr.fossils,estimate peat humification and calculate hydr.climatic indices based on cur.ent bog species,with the over.ll aim of deter.ining the climate conditions associated with evolution of the bog dur.ng the Medieval Climate Anomaly and the Little Ice *** pr.xies,together.with histor.cal and climate data,pr.ved to be good indicator. of the changes in bog sur.ace ***:of the cor. led to identification of 9 differ.nt per.ods:two cor.esponding to the so-called Medieval Climate Anomaly(930 to 1345 AD,1075–665 calibr.ted year. befor. pr.sent[*** BP]);four.cor.esponding to the Little Ice Age(1345 to 1905 AD;665–105 cal yr.BP);and thr.e cor.esponding to the last centur.(1905 to 2000 AD).The findings r.vealed a gener.lly dr. climate that lasted until the 14th centur.,followed by a tr.nsition to a long per.od with a mor. humid,but char.cter.stically ver. var.able climate,which ended at the beginning of the 20th centur. and was followed by a r.pid tr.nsition to mor. humid conditions and finally,a change to dr.er.*** Medieval Climate Anomaly was indicated by the abundance of dr.-adapted mosses(Leucobr.um glaucum,Hypnum cupr.ssifor.e)and char.cter.zed by war. dr. conditions and high levels of peat humification,with alter.ating wet *** LIA per.od was dated by a lar.e abundance of Sphagnum species(an indicator.of wetness)and a gr.dual incr.ase in the humification ***,four.differ.nt climate phases wer. differ.ntiated in this ***-r.solution r.constr.ction of the evolution of the CVM bog and the multipr.xy appr.ach have together.enabled a mor. detailed identification of climatic var.ations in this ar.a,which ar. gener.lly consistent with the global models,as well as better.definition of the elusive climatic oscillations in the last millennium and confir.ation of the impor.ance of local modulation of global *** study pr.vides new infor.ation and a detailed chr.nology of climatic events that will hel

关键词： Last millennium r.ised bog Plant macr.fossil Humification analysis Southwest Eur.pe Climate change

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Gr.phene's photonic and optoelectr.nic pr.per.ies——A r.view

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Chinese Physics B 2020年第3期29卷 13-35页

作者： A J Wir.h-Lima P P Alves-Sousa W Bezer.a-fraga Depar.ment of Physics Federal Institute of EducationScience and Technology of Ceara Campus SobralCearaBrazil Postgr.duate Pr.gr.m in Electr.cal Engineer.ng and Computing Feder.l Univer.ity of Cear.(U.F.C.)Sobr.l CearaBrazil

Due to its r.mar.able electr.cal and optical pr.per.ies,gr.phene continues to r.ceive mor. and mor. attention fr.m r.sear.her. ar.und the *** excellent advantage of gr.phene is the possibility of contr.lling its char.e density,and consequently,the management of its conductivity and dielectr.c constant,among other.*** is notewor.hy that the contr.l of these pr.per.ies enables the obtaining of new optical/electr.nic devices,which would not exist based on conventional ***,to wor. in this ar.a of science,it is necessar. to have a thor.ugh knowledge r.gar.ing the electr.cal/optical pr.per.ies of *** this r.view paper.we show these gr.phene pr.per.ies ver. well detailed.

关键词： absor.ance conductivity dielecr.c constant disper.ion r.lation

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Compar.tive social r.ghts:status and integr.tion in the US

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China Population and Development Studies 2018年第1期2卷 32-56页

作者： Luis r.car.o fraga Br.an Wilcox-Ar.huleta Univer.ity of Notr. Dame Notre DameUSA Univer.ity of Califor.ia los Angeles Los AngelesUSA

In this essay we develop a fr.mewor. for.better.under.tanding how a focus on enhancing the social r.ghts of immigr.nts and migr.nts can lead to gr.ater.levels of social incor.or.tion for.these gr.ups into their.destination countr.es and/or.***,we discuss how the concepts of social r.ghts and incor.or.tion have been used in the social sciences to under.tand migr.nts and ***,we cr.tically discuss models of immigr.nt incor.or.tion der.ved fr.m studies of the US and Eur.pe to demonstr.te the value of developing compar.tive fr.mewor.s acr.ss distinct countr.es and ***,we develop and apply the categor.es of status and integr.tion to discuss how social r.ghts der.ved fr.m public policy can ser.e to inhibit or.facilitate the effective incor.or.tion of immigr.nts and ***,we descr.be how subnational,state-level gover.ments have become incr.asingly cr.tical actor. in affecting both status and integr.tion in the United *** demonstr.te that social r.ghts and incor.or.tion cannot be under.tood in the US today without an appr.ciation for.var.ation acr.ss distinct state ***,we conclude with a consider.tion of how our.analysis makes ver. clear.that gover.ment officials ar. in key positions to deter.ine the scope and depth of immigr.nt and migr.nt social r.ghts and incor.or.tion in ways that can simultaneously ser.e the long-ter. inter.sts of immigr.nts,migr.nts,and the nation state as a whole.

关键词： Social r.ghts Incor.or.tion Immigr.nt Migr.nt Status Integr.tion United States States Nation-state

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Analysis of Heat Tr.nsfer.of a Flat Plate Oscillating Heat Pipe, for.Differ.nt Sur.aces

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Jour.al of Mechanics Engineer.ng and Automation 2017年第7期7卷 356-364页

作者： fraga De Cal Beatr.z Vishak Jagadeesh Kumar Depar.ment of Industr.al Ⅱ Engineer.ng-Higher.Polytechnic School A Coruna University Ferrol 15403 Spain School of Engineer.ng and Mater.al Science Queen Mary University of London Mile End Rd London E1 4NS United Kingdom

r.cent and constant demands for.gr.ater.power.densities and smaller.sizes of electr.nic systems have stimulated the gr.wth of new designs of differ.nt passive heat tr.nsfer.methods such as heat pipes. Par.icular.y, OHPs （Oscillating Heat Pipes） ar. r.latively novel devices, capable of r.moving high heat r.tes over.long and shor. distances with not much temper.tur. dr.p. This study concentr.tes on the design, building and assembling a test r.g in or.er.to analyse the flow patter. ofdeionised water.thr.ugh a 5 tur.s flat plate oscillating heat pipe under.differ.nt heat inputs, which was made in the school of engineer.ng and mater.als science of the Queen Mar. Univer.ity of london by two ener.y ***. students. The filling r.tio of the water.is 40%. Fur.her.or. an exper.mental study on the OHP ther.al per.or.ance is car.ied out in or.er.to examine the effects of differ.nt sur.ace wet conditions： super.hydr.philic, hydr.philic and cleaned br.ss. It is demonstr.ted the for.ation of liquid slugs and vapour.plugs of the water.along the channels. The exper.mental r.sults showed that the hydr.philic sur.ace tends to be mor. ener.y efficient. The heat tr.nsfer.per.or.ance of the super.hydr.philic and hydr.philic is higher.than br.ss by 5-12% and 15-20% r.spectively.

关键词： Oscillating heat pipe heat tr.nsfer flow patter. ther.al per.or.ance.

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Par.inson’s disease and diabetes mellitus: common mechanisms and tr.atment r.pur.osing

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Neur.l r.gener.tion r.sear.h 2022年第8期17卷 1652-1658页

作者： Car.en M.Labandeir. Ar.ur. fraga-Bau David Ar.as r.n Elena Alvar.z-r.dr.guez Pablo Vicente-Alba Javier.Lago-Gar.a Ana I.r.dr.guez-Per.z Depar.ment of Clinical Neur.logy Hospital Alvaro CunqueiroUniversity Hospital ComplexVigoSpain Labor.tor. of Cellular.and Molecular.Neur.biology of Par.inson’s Disease Research Center for Molecular Medicine and Chronic Diseases(CIMUS)IDISUniversity of Santiago de CompostelaSantiago de CompostelaSpain Depar.ment of Clinical Oncology University Hospital ComplexOurenseSpain Depar.ment of Endocr.nology Hospital MeixoeiroUniversity Hospital ComplexVigoSpain Networ.ing r.sear.h Center.on Neur.degener.tive Diseases(Ciber.ed) MadridSpain

In the last decade,attention has become gr.ater.to the r.lationship between neur.degener.tion and abnor.al insulin signaling in the centr.l ner.ous system,as insulin in the br.in is implicated in neur.nal sur.ival,plasticity,oxidative str.ss and *** mellitus and Par.inson’s disease ar. both aging-associated diseases that ar. tur.ing into epidemics *** mellitus and insulin r.sistance not only incr.ase the possibility of developing Par.inson’s disease but can also deter.ine the pr.gnosis and pr.gr.ssion of Par.insonian ***,ther. ar. no available cur.tive or.disease modifying tr.atments for.Par.inson’s disease,but the r.le of insulin and antidiabetic medications in neur.degener.tion opens a door.to tr.atment r.pur.osing to fight against Par.inson’s disease,both in diabetic and nondiabetic Par.insonian ***,it is essential to compr.hend how a fr.quent and tr.atable disease such as diabetes can influence the pr.gr.ssion of neur.degener.tion in a challenging disease such as Par.inson’s ***,we r.view the pr.sent evidence on the connection between Par.inson’s disease and diabetes and the consequential implications of the existing antidiabetic molecules in the sever.ty and development of Par.insonism,with a par.icular.focus on glucagon-like peptide-1 r.ceptor.agonists.

关键词： antidiabetic diabetes mellitus dopamine exenatide glucagon-like peptide-1 insulin neur.degener.tion neur.inflammation Par.inson’s disease r.pur.osing

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Explor.ng the factor. affecting car.on and nutr.ent concentr.tions in tr.e biomass components in natur.l for.sts,for.st plantations and shor. r.tation for.str.

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For.st Ecosystems 2018年第4期5卷 498-515页

作者： r.que r.dr.guez-Soalleir. Cr.stina Eimil-fraga Esteban Gomez-Gar.a Juan Daniel Gar.ia-Villabr.lle Alber.o r.jo-Albor.ca Fer.ando Munoz Ner.a Oliveir. Hor.ensia Sixto Cesar.Per.z-Cr.zado Sustainable For.st Management Unit (UXFS) Department of Plant Production and Engineering ProjectsUniversidade de Santiago de Compostela (USC)Rua Benigno LedoCampus Terra27002 LugoSpain Centr. de Investigacion For.stal de lour.an Xunta de Galicia.Carretera de Marin km 3.536153 PontevedraSpain Sustainable For.st Management Unit (UXFS) Department of Agroforestry EngineeringUniversldade de Santiago de Compostela (USC)LugoSpain Univer.idad de Concepcion Facultad de Ciencias ForestalesVlctona631 ConcepcionChile For.st Systems and r.sour.es Depar.ment Centre for Forest ResearchINIACrta. de la Coruna km 7.528040MadridSpain

Backgr.und: Coupling biomass models with nutr.ent concentr.tions can pr.vide sound estimations of car.on and nutr.ent contents, enabling the impr.vement of car.on and nutr.ent balance in for.st ecosystems. Although nutr.ent concentr.tions ar. often assumed to be constant for.some species and specific tr.e components, at least in matur. stands,the concentr.tions usually var. with age, site index and even with tr.e density. The main objective of this study was to evaluate the sour.es of var.ation in nutr.ent concentr.tions in biomass compar.ments usually r.moved dur.ng har.esting oper.tions, cover.ng a r.nge of species and management conditions: semi-natur.l for.st, conventional for.st plantations and shor. r.tation for.str.(Sr.). Five species(Betula pubescens, Quer.us r.bur. Eucalyptus globulus, Eucalyptus nitens and Populus spp.) and 14 genotypes wer. consider.d. A total of 430 tr.es wer. sampled in 61 plots to obtain 6 biomass components:leaves, twigs, thin br.nches, thick br.nches, bar. and wood. Abovegr.und leafless biomass was pooled together.*** concentr.tions of C, N, K, P, Ca, Mg, S, Fe, Mn, Cu, Zn and B wer. measur.d and the total biomass of each sampled tr.e and plot wer. deter.ined. The data wer. analysed using boosted r.gr.ssion tr.es and conventional ***: The main sour.es of var.ation in nutr.ent concentr.tions wer. biomass component > > genotype(species) ≈ age >tr.e diameter. The concentr.tions of Ca, Mg and K wer. most str.ngly affected by genotype and age. The concentr.tions of P, K, Ca, Mg, S and Cu in the wood component decr.ased with age, wher.as C concentr.tions incr.ased, with a tr.nd to r.ach 50% in the older.tr.es. In the Sr., inter.mer.can poplar.and P. tr.chocar.a genotypes wer. compar.tively mor. efficient in ter.s of Ca and K nutr.ent assimilation index(NAI)(+65-85%) than eucalypts, mainly because leafless biomass can be r.moved. In the conventional eucalypt plantations(r.tation 15 year.), debar.ing the wood at logg

关键词： Nutr.ent r.moval Biomass cr.ps Poplar.genotypes Eucolyptus Oak Bir.h Plantation sustainability

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r.ilway for.Tour.sm in South Amer.ca： An Appr.ach on Its Uses

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Jour.al of Tr.ffic and Tr.nspor.ation Engineer.ng 2016年第6期4卷 339-349页

作者： Thiago Allis Car.a fraga Maximiliano Augusto Velazquez Depar.ment of Geogr.phy Tourism and Humanities Federal University of Sao Carlos Sorocaba Sao Paulo 18052-780 Brazil Depar.ment of Tour.sm and Her.tage Federal University of the State of Rio de Janeiro Rio de Janeiro 22290-240 Brazil Center.for.Metr.politan Tr.nspor.ation Studies (CETAM) Universidad de Buenos Aires City of Buenos Aires C1428EGAArgentina

In this paper. the author. focus on the inter.lay between r.ilways and tour.sm. Ther.for., evidence is pr.vided in or.er.to decipher.the manner.in which r.ilways ar. used for.tour.sm pur.oses in South Amer.ca. With a view to achieving this goal, an explor.tor. appr.ach is used, which consists in the analysis of publications on tour.sm and tr.nspor.ation made by r.nowned scholar., as well as a descr.ptive appr.ach, based upon the classification of data obtained fr.m public and pr.vate r.ilway or.anizations. The paper.notes that tour.sm is of par.mount impor.ance in the pr.tection of the r.ilway her.tage in South Amer.ca. Following this line of r.asoning, it is concluded that ther. is a wide var.ety of tour.st tr.ins which exploit either.the cultur.l and histor.cal aspects of r.ilways or.the bewitching landscapes offer.d by r.ilway tr.ps and cr.ate exper.ences for.tour.sts.

关键词： Tr.nspor. tour.sm r.ilways South Amer.ca methodology.

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Is ther. a window of oppor.unity for.the ther.peutic use of vitamin D in multiple scler.sis?

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Neur.l r.gener.tion r.sear.h 2022年第9期17卷 1945-1954页

作者： Sofia Fer.anda Gonçalves Zor.ella-Pezavento Luiza Ayumi Nishiyama Mimur. Mar.na Bonifácio Denadai William Danilo Fer.andes de Souza Thais Fer.anda de Campos fraga-Silva Alexandr.na Sar.or. Depar.ment of Chemical and Biological Sciences Institute of BiosciencesSão Paulo State University(UNESP)BotucatuSão PauloBrazil

Multiple scler.sis is an autoimmune tr.atable but not cur.ble *** ar. a multiplicity of medications for.multiple scler.sis ther.py,including a class entitled disease-modifying dr.gs that ar. mainly indicated to r.duce the number.and sever.ty of disease *** all patients r.spond well to these ther.pies,and minor.to sever. adver.e effects have been *** D,called sunshine vitamin,is being studied as a possible light at the end of the *** this r.view,we r.capitulated the similar.immunopathogenesis of multiple scler.sis and exper.mental autoimmune encephalomyelitis,the immunomodulator. and neur.pr.tective potential of vitamin D and the state-of-ar. concer.ing its supplementation to multiple scler.sis ***,based on our.and other.gr.ups’exper.mental findings,we analyzed the need to consider.the r.levance of the r.ute and the differ.nt time-point administr.tion aspects for.a mor. r.tional indication of this vitamin to multiple scler.sis patients.

关键词： centr.l ner.ous system cytokines exper.mental autoimmune encephalomyelitis immunomodulation immunopathogenesis inflammation multiple scler.sis neur.pr.tection r.gulator. T cells vitamin D

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