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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

Clues from a missense mutation of the adenosine A1 receptor gene associated with early-onset Parkinson’s disease

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Neural Regeneration Research 2023年第10期18卷 2204-2205页

作者： Sergi Ferré Leonardo pardo Francisco Ciruela Integrative Neurobiology Section National Institute on Drug AbuseIntramural Research ProgramNational Institutes of HealthBaltimoreMDUSA Laboratory of Computational Medicine Biostatistics UnitFaculty of MedicineUniversitat Autònoma BarcelonaBellaterraBarcelonaSpain Pharmacology Unit Department of Pathology and Experimental TherapeuticsSchool of Medicine and Health SciencesInstitute of NeurosciencesUniversity of Barcelona.Neuropharmacology&Pain GroupNeuroscience ProgramBellvitge Institute for Biomedical ResearchL’Hospitalet de LlobregatSpain

Parkinson’s disease (P D) is a com plex neu rodegenerative disorder for which ra re and common genetic variants contribute to disease risk,onset,and *** genetic contribution to PD can be classified mainly in,first,rare DNA variants that are highly penetrant and therefore causal,which are typically associated with monogenic PD;and second,more common risk polymorphic variants,which individually exert a small increase in the risk of the disease,which are usually identified in the most prevalent and apparently sporadic PD (Blauwendraat et al.,2020).

关键词： typically degenerative

Tourism versus nature conservation: reconciliation of common interests and objectives——an analysis through Picos de Europa National Park

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Journal of Mountain Science 2018年第11期15卷 2505-2516页

作者： LóPEZ Iván pardo Mercedes Department of Psychology and Sociology University of Zaragoza Department of Social Analysis University Carlos Ⅲ of Madrid

Nature tourism and particularly tourism in national parks have acquired significant importance in contemporary societies. Post-Fordist consumers have reevaluated the meanings of ‘nature' and ‘natural spaces' and now avoid standardization to seek singularity. Tourism in national parks is a consequence of this tendency and has both positive and negative aspects. The purpose of this sociological research is to describe the most relevant conflicts in the Picos de Europa National Park(Spain) involving the park's conservation, local economic development,and tourism. Seven in-depth interviews and three focus groups were addressed to key local *** this research were identified three chief areas of existing or potential inter-related conflicts and the main actors interacting with them. The first is on population, particularly, the negative consequences of depopulations on the local socio-economic development and the environment. A second source of conflicts identified is caused by the difficult conciliation between commercial exploitation and conservation of the protected natural area. More precisely, this specific form of tourism positively contributes to the economy of local communities whilst problems can arise for the conservation goals of National Parks. Thirdly, in this research is also analyzed the institutional governance and the inter and intra-governmental conflicts as well as with the Park's management body. These findings provide important information for the improved management of tourism and conflicting interests in natural parks.

关键词： National parks Environmental conservation Nature tourism Mountain traditional economy Picos de Europa Spain

Effect of successive sprays on liquid distribution in fluidized beds

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Particuology 2021年第1期19卷 17-24页

作者： Cedric Briens Liliana pardo Reyes Franco Berruti Jennifer McMillan Institute for Chemicals and Fuels from Alternative Resources Western University22312 Wonderland Rd.IldertonONN0M 2A0Canada Edmonton Research Centre Syncrude Canada Ltd942117th AveEdmontonABT6N 1H4 Canada

In processes such as Fluid Coking^(™),agglomerate formation should be minimized since it reduces the yield of valuable products,and degrades operability because of the fouling of *** experimental model,consisting of an aqueous solution of gum arabic with a dye,has been successfully developed to simulate the formation of agglomerates in the Fluid Coking^(™)process,where bitumen is sprayed into a fluidized bed of coke particles The particles wetted by a spray could be predicted by assuming that all the particles in the wake of bubbles formed from the tip of the spray jet have been wetted by the injected *** transfer of liquid from particles wetted with the spray to dry bed particles was relatively ineffective,as the number of wet particles increased by only 50%.With successive liquid injections,the proportion of the liquid trapped in agglomerates increases in latter injections:large agglomerates from earlier injections accumulate above the grid and are carried by gas bubbles into the spray jet cavity,where they seed fresh agglomerates.

关键词： Fluidized bed Liquid injection Agglomerate formation Fluid Coker

Glyco-sphingo biology: a novel perspective for potential new treatments in Huntington's disease

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Neural Regeneration Research 2017年第9期12卷 1439-1440页

作者： Alba Di pardo Vittorio Maglione Centre for Neurogenetics and Rare Diseases IRCCS Neuromed

Huntington＇s disease （HD） is the most common dominantly inherited neurodegenerative disorder, mainly characterized by the progressive striatal and cortical neurodegeneration and as- sociated motor, cognitive and behavioural disturbances （Zuccato et al., 2010）. The disease-causing mutation is an expansion of a GAG trinucleotide repeat （〉 36 repeats） encoding a polygluta- mine stretch in the N-terminal region ofhuntingtin （Htt）（Zuc- cato et al., 2010）, a ubiquitous protein whose function is still unclear （Zuccato et al., 2010）. Expansion of the polyQ stretch endows mutant Htt （mHtt） with toxic properties, and results in the development of a broad array of undesirable effects in both neuronal and non-neuronal cells （Zuccato et al., 2010）. Among all cellular dysfunctions and biochemical imbalances classically associated with HD, perturbed metabolism of （glyco） sphingolipids appears to play a crucial role in the pathogenesis of the disease. Over the last years, we and other have extensively contributed to these findings （Desplats et al., 2007;

关键词： Glyco-sphingo biology a novel perspective for potential new treatments in Huntington’s disease HD

On Second Order Degree of Graphs

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Acta Mathematica Sinica,English Series 2012年第1期28卷 171-182页

作者： Gabriela ARAUJO-pardo Camino BALBUENA Mika OLSEN Pilar VALENCIA Instituto de Matemáticas rea de la Investigación CientíficaUniversidad Nacional Autónoma de MxicoCircuito ExteriorCiudad UniversitariaCoyoacan04510 MxicoD.F.Mxico Departament de Matemàtica Aplicada III Universitat Politècnica de CatalunyaC/Jordi Girona 1-3(Edifici C2Despatx 302)08034-BarcelonaSpain Departamento de Matemáticas Aplicadas y Sistemas Universidad Autónoma MetropolitanaCalle Artificios 402o pisoCol.Hidalgo Delegación lvaro Obregón01120 Mxico D.F.Mxico

Given a vertex v of a graph G the second order degree of v denoted as d2（v） is defined as the number of vertices at distance 2 from v. In this paper we address the following question： What axe the sufficient conditions for a graph to have a vertex v such that d2（v） ≥ d（v）, where d（v） denotes the degree of v？ Among other results, every graph of minimum degree exactly 2, except four graphs, is shown to have a vertex of second order degree as large as its own degree. Moreover, every K4^--free graph or every maximal planar graph is shown to have a vertex v such that d2（v） ≥ d（v）. Other sufficient conditions on graphs for guaranteeing this property axe also proved.

关键词： Second order degree K4^-free graph planar graphs

Impact of dietary L-arginine supply during early gestation on myofiber development in newborn pigs exposed to intra-uterine crowding

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Journal of Animal Science and Biotechnology 2017年第4期8卷 945-956页

作者： Johannes Gulmann Madsen Camilo pardo Michael Kreuzer Giuseppe Bee Agroscope Posieux la tioleyre 4 1725 Posieux Switzerland ETH Zurich Institute of Agricultural Sciences Universitsstrasse 2 8092 ZurichSwitzerland

Background: Intra-uterine crowding(IUC) observed in hyperprolific sows impairs myofiber hyperplasia and overall fetal growth. Arginine supplementation(ARG) in gestation diets has been shown to positively affect litter and muscle development. The study objective was to assess whether the effect of ARG on offspring characteristics, with special emphasis on myofiber hyperplasia, differs under IUC conditions from these responses,because in that situation growth retardation is particularly prevalent due to reduced fetal nutrient *** oviduct ligation(OL) was used as a model for an uncrowded and hyperprolificacy(IN) as a model for a crowded intra-uterine ***: Five OL and five IN sows were fed a diet supplemented daily with either 43 g L-alanine(Ctrl) or 25 g L-arginine from d 14 to 28 of gestation in a cross-over design(two periods: 5^( th) and 6^( th) parity). At farrowing, two male and two female offspring, respectively, with a low and intermediate birth weight(BtW) were selected. After euthanization, the Semitendinosus muscle(STM) was removed and weighed, and the light and dark portions(STM_d and STM_l) were prepared for myofiber histochemistry using ATPase staining and the entire STM for gene expression analysis of myogenesis-related genes using RT-qPCR. In addition, various organs were removed and weighed. Data were analyzed using the MIXED model in ***: No effect of either IUC or dietary treatment was found in litter characteristics. Offspring of ARG sows displayed a greater muscle area in STM(P < 0.01) as a result of the greater myofiber hyperplasia(P < 0.01). The increase was more distinct in the STM_l(P < 0.05) than in the STM_d(P = 0.131). Offspring of OL sows were heavier at birth(P < 0.01), had a heavier STM(P < 0.05), liver(P < 0.01) and kidney(P < 0.05), but when expressed relative to birth weight, these differences were absent. In addition, IUC had an effect(P < 0.05) on the expression of one of the myogenesis-related

关键词： Dietary supplement Early gestation Intra-uterine growth restriction Myofiber hyperplasia Neonate Sow prolificacy

Calcium signaling primes RNA interference during viral infection

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Molecular Plant 2021年第10期14卷 1621-1622页

作者： Jesus Sanchez pardo Echevarria Bruno Pok Man Ngou The Sainsbury Laboratory University of East AngliaNorwich Research ParkNorwich NR47UHUK

Plants resist viral infection through multiple *** include physical barriers,activation of NUCLEOTIDE-BINDING LEUGINE-RICH REPEAT PROTEINS(NLRs),autophagy,biosyn-thesis of defense-related phytohormones,and RNA interference(RNAi).RNAI suppresses the expression of viral RNA and is conserved across several eukaryotic *** infection,RNA-DEPENDENT RNA POLYMERASES(RDRs)amplify viral RNA fragments into double-stranded RNAs(dsRNAs).

关键词： infection mechanisms viral

Phages and Nanotechnology:New Insights against Multidrug-Resistant Bacteria

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BioDesign Research 2023年第1期5卷 12-24页

作者： Marco pardo-Freire Pilar Domingo-Calap Institute for Integrative Systems Biology I2SysBioUniversitat de València-CSIC46980 PaternaSpain.

Bacterial infections are a major threat to the human healthcare system worldwide,as antibiotics are becoming less effective due to the emergence of multidrug-resistant ***,there is a need to explore nontraditional antimicrobial alternatives to support rapid interventions and combat the spread of pathogenic *** nonantibiotic approaches are being developed,many of them at the interface of physics,nanotechnology,and *** physical factors(e.g.,pressure,temperature,and ultraviolet light)are typically used in the sterilization process,nanoparticles and phages(bacterial viruses)are also applied to combat pathogenic ***,phage-based therapies are rising due to the unparalleled specificity and high bactericidal activity of *** the success of phages mostly as compassionate use in clinical cases,some drawbacks need to be addressed,mainly related to their stability,bioavailability,and systemic *** phages with nanoparticles can improve their performance in ***,the combination of nanotechnology and phages might provide tools for the rapid and accurate detection of bacteria in biological samples(diagnosis and typing),and the development of antimicrobials that combine the selectivity of phages with the efficacy of targeted therapy,such as photothermal ablation or photodynamic *** this review,we aim to provide an overview of how phage-based nanotechnology represents a step forward in the fight against multidrug-resistant bacteria.

关键词： diagnosis mostly ultraviolet