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Clustering of voltage-gated ion channels as an evolutionary trigger of myelin formation

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Neural Regeneration Research 2024年第8期19卷 1631-1632页

作者： Henrike Ohm Simone rey Christian Klambt Institut fur Neuro-und Verhaltensbiologie MunsterGermany

Neurons carry apical dendrites that perceive information and a basal axon that transmits the computed information towards its to *** axon originates at the axon hillock which is followed by the axon initial ***,action potentials are initiated that are based on millisecond long openings of specific voltagegated sodium and potassium channels that are conserved in all parahoxozoa(Placozoa,Cnidaria,Bilateria)(Li et al.,2015).

关键词： initiated carry potassium

Insights on ZEB1-AS1:emerging role from cancer to neurodegeneration

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Neural Regeneration Research 2024年第6期19卷 1187-1188页

作者： Stephana Carelli Federica rey Erika Maghraby Cristina Cereda Pediatric Research Center“Romeo ed Enrica Invernizzi” Department of Biomedical and Clinical SciencesUniversity of MilanMilanItaly Center of Functional Genomics and Rare Diseases Department of PediatricsBuzzi Children’s HospitalMilanItaly Department of Biology and Biotechnology“L.Spallanzani” University of PaviaPaviaItaly

Implications for lncRNAs in the central nervous system:Transcriptional dysregulation is a key contributor to the pathogenesis of a wide range of diseases and long non-coding RNAs(lncRNAs)are highly expressed in the nervous ***,amongst the over 50,000 lncRNAs expressed in the human genome,more than 40%are specifically expressed in the brain where their roles in brain development,neuron functions,maintenance.

关键词： ZEB1 diseases pathogenesis

Beyond wrecking a wall:revisiting the concept of blood–brain barrier breakdown in ischemic stroke

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Neural Regeneration Research 2025年第7期20卷 1944-1956页

作者： Julia Castillo-González Elena González-rey Institute of Parasitology and Biomedicine Lopez-Neyra(IPBLN) CSICPT SaludGranadaSpain

The blood–brain barrier constitutes a dynamic and interactive boundary separating the central nervous system and the peripheral *** tightly modulates the ion transport and nutrient influx,while restricting the entry of harmful factors,and selectively limiting the migration of immune cells,thereby maintaining brain *** the well-established association between blood–brain barrier disruption and most neurodegenerative/neuroinflammatory diseases,much remains unknown about the factors influencing its physiology and the mechanisms underlying its ***,the role of blood–brain barrier breakdown in the translational failure underlying therapies for brain disorders is just starting to be *** review aims to revisit this concept of“blood–brain barrier breakdown,”delving into the most controversial aspects,prevalent challenges,and knowledge gaps concerning the lack of blood–brain barrier *** moving beyond the oversimplistic dichotomy of an“open”/“bad”or a“closed”/“good”barrier,our objective is to provide a more comprehensive insight into blood–brain barrier dynamics,to identify novel targets and/or therapeutic approaches aimed at mitigating blood–brain barrier ***,in this review,we advocate for considering the diverse time-and location-dependent alterations in the blood–brain barrier,which go beyond tight-junction disruption or brain endothelial cell breakdown,illustrated through the dynamics of ischemic stroke as a case *** this exploration,we seek to underscore the complexity of blood–brain barrier dysfunction and its implications for the pathogenesis and therapy of brain diseases.

关键词： blood–brain barrier disruption drug delivery ischemic stroke neuroinflammation tight-junctions

Rare dentin defects:Understanding the pathophysiological mechanisms of COLXVA1 mutations

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Genes & Diseases 2024年第5期11卷 1-5页

作者： Isaac Maximiliano Bugueno Tristan rey Alexandra Jimenez-Armijo Marzena Kawczynski Naji Kharouf Marie-Cecile Maniere Yann Herault Agnes Bloch-Zupan Virginie HaushalterLaugel Institute of Genetics and Molecular and Cellular Biology(IGBMC) CNRS-UMR7104INSERM U1258Universitede StrasbourgIllkirch 67400France Faculty of Dentistry Universitede StrasbourgStrasbourg 67000France Institute of Advanced Studies(USIAS) Universitede StrasbourgStrasbourg 67045France Reference Center for Rare Oral and Dental Diseases(CRMR-O-Rares) Oral Medicine and Surgery DepartmentHopitaux Universitaires de Strasbourg(HUS)Strasbourg 67000France Rare Diseases Health Network“TETE COU”&European Reference Network ERN CRANIO Strasbourg 67000France “Institut de Genetique Medicale d’Alsace” Laboratory of Genetic DiagnosticHopitaux Universitaires de Strasbourg(HUS)Strasbourg 67000France Biomaterials and Bioengineering Laboratory Inserm UMR_S 1121Strasbourg 67000France Orofacial Development and Regeneration Faculty of MedicineInstitute of Oral BiologyCentre of Dental MedicineUniversity of ZurichZurich 8032Switzerland

Dentin is a mineralized tissue with a chemical composition similar to bone but with a higher mineralized density and *** constitutes the central structure of the tooth between the internal pulp and external enamel toward the oral cavity or cementum toward the underlying *** dentin defects occur in a variety of rare genetic *** can manifest as“isolated”occurrences such as in dentinogenesis imperfecta(DI)or dentin dysplasia(DD)or can be associated with other symptoms in diseases such as osteogenesis imperfecta,Goldblatt syndrome,microcephalic osteodysplastic primordial dwarfism type Ⅱ,among others.

关键词： diseases den rigidity

Experimental test of a building thermal system for preventive maintenance based on thermoeconomic analysis

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Energy Storage and Saving 2024年第1期3卷 42-51页

作者： I.Prol-Godoy A.Picallo-Perez J.M.Sala-Lizarraga J.rey-Martínez Research group ENEDI Department of Thermal EngineeringUniversity of the Basque Country(UPV/EHU)Bilbao48013Spain Research Group Termotecnia University of ValladolidValladolid47002Spain

An analysis of the sectorial structure of energy consumption shows that residential and tertiary sector buildings are the third-highest consumers,responsible for 29.5%of a city’s final energy *** Building Quality Control Laboratory of the Basque Government aims to promote quality,innovation,and sustainability in *** accomplish this goal,it has constructed an experimental facility with different energy generation technologies and a very versatile control system for testing different energy systems and operation *** this study,we tested a facility for supplying domestic hot water and heating for a multi-family house by means of a condensing boiler and an aerothermal heat pump(together with the corresponding control).This installation could reproduce the thermal demands required to be satisfied by the generation equipment through a programmed operation of the installation based on real ***,this installation was analyzed using thermoeconomics(TE)to solve problems unable to be solved using traditional energy analyses based on the First Law of *** problems include:(1)Determining the costs of the products of the installation based on physical criteria,(2)detecting the places where losses actually occur,evaluating their costs,and proposing cost-effective improvements,and(3)diagnosing issues in the *** a result,this paper suggests a solution to the preventive maintenance problems confronting the technical maintenance personnel for thermal installations in buildings by applying TE knowledge and using real data collected from sensors.

关键词： Experimental essay Energy savings in thermal systems Thermoeconomics Thermoeconomic costs Diagnosis

Characterization of the molecular dysfunctions occurring in Aicardi-Goutieres syndrome patients with mutations in ADAR1

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Genes & Diseases 2024年第3期11卷 116-119页

作者： Sofan Al wardat Loredana Frasinlli Elisa Orecchini Federica rey Silvia Anna Ciafre Silvia Galardi Jessica Garau Stella Gagliardi Simona Orcesi Davide Tonduti Stephana Carelli Cristina Cereda Ernesto Picardi Alessandro Michienzi Department of Biomedicine and Prevention University of Rome Tor VergataRome 00133Italy Pediatric Clinical Research Center"Romeo ed Enrica Invernizzi" Department of Biomedical and Clinical SciencesUniversity of MilanoMilano 20157Italy Center of Functional Genomics and Rare Diseases Department of PediatricsBuzzi Children's HospitalMilano20154Italy Department of Child Neurology and Psychiatry IRccs Mondino FoundationPavia 27100Italy Unit of Pediatric Neurology C.O.A.L.A(Center for Diagnosis and Treatment of Leukodystrophies)Buzzi Children's HospitalMilano 20154Italy Department of Biosciences Biotechnologies and EnvironmentUniversityof Bari"AldoMoro"Bari 70125Italy Institute of Biomembranes Bioenergetics and Molecular Biotechnologies(IBIOM)National Research Council(CNR)Bari 70126Italy Biostructures and Biosystems National Institute(INBB) Rome00136Italy Institute of Biochemistry and Cell Biology IBBC-CNRCampus Adriano Buzzati TraversoRome 00015Italy

Aicardi-Goutieres syndrome(AGS)is a systemic inflammatory disorder caused by mutations in any one of the nine different genes,whose deficiency provokes a type I(interferon)IFN response probably central to pathogenesis.^(1) ADAR1,one of the genes mutated in AGS(AGS6),encodes for an enzyme that belongs to the ADAR family(ADAR1,ADAR2,and ADAR3)that catalyzes the conversion of adenosine to inosine within double-stranded RNAs(dsRNAs)(RNA editing A-to-1).

关键词： ADAR1 interferon res

Quality-adjusted life years and surgical waiting list:Systematic review of the literature

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World Journal of Gastrointestinal Surgery 2024年第4期16卷 1155-1164页

作者： Roberto de la Plaza Llamas Lorena Ortega Azor Marina Hernández Yuste Ludovica Gorini Raquel Aránzazu Latorre-Fragua Daniel Alejandro Díaz Candelas Farah Al Shwely Abduljabar Ignacio Antonio Gemio del rey Department of General and Digestive Surgery Hospital Universitario de GuadalajaraGuadalajara 19002Spain Department of Surgery Medical and Social SciencesFaculty of Medicine and Health SciencesUniversity of AlcaláAlcaláde Henares 28871MadridSpain

BACKGROUND The quality-adjusted life year(QALY)is a metric that is increasingly used today in the field of health economics to evaluate the value of different medical treatments and *** waiting lists(SWLs)represent a pressing problem in public *** QALY measure has rarely been used in the context of *** would be interesting to know how many QALYs are lost by patients on *** To investigate the relationship between QALYs and SWLs in a systematic review of the scientific *** The study was conducted in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses *** unlimited search was carried out in PubMed,updated on January 19,*** on the following variables were investigated and analyzed:Specialty,country of study,procedure under study,scale used to measure QALYs,the use of a theoretical or real-life model,objectives of the study and items measured,the economic value assigned to the QALY in the country in question,and the results and conclusions *** Forty-eight articles were selected for the *** data were found regarding QALYs lost on *** specialties in which QALYs were studied the most in relation to the waiting list were urology and general surgery,with 15 articles *** country in which the most studies of QALYs were carried out was the United States(n=21),followed by the United Kingdom(n=9)and Canada(n=7).The most studied procedure was organ transplantation(n=39),including 15 kidney,14 liver,5 heart,4 lung,and 1 ***(n=4),cataract surgery(n=2),bariatric surgery(n=1),mosaicplasty(n=1),and septoplasty(n=1)completed the surgical interventions ***-nine of the models used were theoretical(the most frequently applied being the Markov model,n=34),and nine were *** survey used to measure quality of life in 11 articles was the European Quality of Life-5 dimensions,but in 32 articles the survey was not specified.T

关键词： Quality-adjusted life year Waitlist Quality of life Surgery Systematic review

Promotion of structural plasticity in area V2 of visual cortex prevents against object recognition memory deficits in aging and Alzheimer's disease rodents

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Neural Regeneration Research 2024年第8期19卷 1835-1841页

作者： Irene Navarro-Lobato Mariam Masmudi-Martín Manuel F.López-Aranda Juan F.López-Téllez Gloria Delgado Pablo Granados-Durán Celia Gaona-Romero Marta Carretero-rey Sinforiano Posadas María E.Quiros-Ortega Zafar U.Khan Laboratory of Neurobiology Centro de Investigaciones Médico Sanitarias(CIMES)University of MalagaMalagaSpain Department of Medicine Faculty of MedicineUniversity of MalagaMalagaSpain Centro de Investigación Biomédica en Red de Enfermedades Neurodegenerativas(CIBERNED) Institute of Health Carlos IIIMadridSpain the Donders Institute for Brain Cognition and Behaviour Radboud UniversityNijmegenThe Netherlands the Brain Metastasis Group National Cancer Research Centre(CNIO)MadridSpain the Department of Neurobiology University of California-Los AngelesLos AngelesCAUSA

Memory deficit,which is often associated with aging and many psychiatric,neurological,and neurodegenerative diseases,has been a challenging issue for *** till now,all potential drug candidates have failed to produce satisfa ctory ***,in the search for a solution,we found that a treatment with the gene corresponding to the RGS14414protein in visual area V2,a brain area connected with brain circuits of the ventral stream and the medial temporal lobe,which is crucial for object recognition memory(ORM),can induce enhancement of *** this study,we demonstrated that the same treatment with RGS14414in visual area V2,which is relatively unaffected in neurodegenerative diseases such as Alzheimer s disease,produced longlasting enhancement of ORM in young animals and prevent ORM deficits in rodent models of aging and Alzheimer’s ***,we found that the prevention of memory deficits was mediated through the upregulation of neuronal arbo rization and spine density,as well as an increase in brain-derived neurotrophic factor(BDNF).A knockdown of BDNF gene in RGS14414-treated aging rats and Alzheimer s disease model mice caused complete loss in the upregulation of neuronal structural plasticity and in the prevention of ORM *** findings suggest that BDNF-mediated neuronal structural plasticity in area V2 is crucial in the prevention of memory deficits in RGS14414-treated rodent models of aging and Alzheimer’s ***,our findings of RGS14414gene-mediated activation of neuronal circuits in visual area V2 have therapeutic relevance in the treatment of memory deficits.

关键词： behavioral performance brain-derived neurotrophic factor cognitive dysfunction episodic memory memory circuit activation memory deficits memory enhancement object recognition memory prevention of memory loss regulator of G protein signaling

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深海之王

商业周刊（中文版） 2024年第9期 54-63页

作者： Kit Chellek Olivia Solon Jonathan Browning Ricardo rey(插图) 杨飞(译) 不详

多年来,世界上最厉害的深海沉船猎人一直在与各国政府及竞争对手争夺海底的财富。他把自己的身份隐藏得很好,一直到现在。冰岛海岸警卫队的官员们在控制室的显示屏上注视着这艘船——在广阔的蓝色海图上,它只是一个孤点。2017年4月,这... 详细信息

多年来,世界上最厉害的深海沉船猎人一直在与各国政府及竞争对手争夺海底的财富。他把自己的身份隐藏得很好,一直到现在。冰岛海岸警卫队的官员们在控制室的显示屏上注视着这艘船——在广阔的蓝色海图上,它只是一个孤点。2017年4月,这个小点在离岸120英里(约193公里)的地方盘旋了好几天,任何理智的水手都不会在当时那种恶劣的天气里闲逛。接近飓风等级的狂风呼啸着穿越凶涌的大海;天空中雨雪交加。

关键词：海岸警卫队控制室显示屏竞争对手