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The emerging role of autophagic-lysosomal dysfunction in Gaucher disease and Parkinson's disease

Neural Regeneration Research 2017年第3期12卷 380-384页

作者： Kerri J.Kinghorn Amir m.Asghari jorge Iván Castillo-Quan Institute of Healthy Ageing and Department of Genetics Evolution and EnvironmentUniversity College LondonLondonUK Institute of Neurology University College LondonLondonUK Department of Basic and Clinical Neuroscience King's College LondonMaurice Wohl Clinical Neuroscience InstituteLondonUK London Central&West Unscheduled Care Collaborative St.Charles Centre for Health and WellbeingExmoor Street StCharles HospitalLondonUK Research Division Joslin Diabetes CenterBostonMAUSA Department of Genetics and Harvard Stem Cell Institute Harvard Medical SchoolBostonMAUSA

Gaucher disease（GD）,the commonest lysosomal storage disorder,results from the lack or functional deficiency of glucocerebrosidase（GCase） secondary to mutations in the GBA1 *** is an established association between GBA1 mutations and Parkinson＇s disease（PD）,and indeed GBA1 mutations are now considered to be the greatest genetic risk factor for *** lysosomal-autophagic degradation of cellular proteins,including α-synuclein（α-syn）,is implicated in the pathogenesis of PD,and there is increasing evidence for this also in GD and *** we have recently shown in a Drosophila model lacking neuronal GCase,that there are clear lysosomal-autophagic defects in association with synaptic loss and *** addition,we demonstrated alterations in mechanistic target of rapamycin complex 1（mTORC1） signaling and functional rescue of the lifespan,locomotor defects and hypersensitivity to oxidative stress on treatment of GCase-deficient flies with the mT OR inhibitor ***,a number of other recent studies have shown autophagy-lysosomal system（ALS） dysfunction,with specific defects in both chaperone-mediated autophagy（CmA）,as well as macroautophagy,in GD and GBA1-PD model *** we discuss the possible therapeutic benefits of inhibiting mT OR using drugs such as rapamycin to reverse the autophagy defects in GD and PD.

关键词： Gaucher disease Parkinson's disease Drosophila autophagy lysosome glucocerebrosidase GBA

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Preparation of Rod-like Aluminum Doped Zinc Oxide Powders by Sol-gel Technique Using metal Chlorides and Acetylacetone Precursors

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Journal of Wuhan University of Technology(materials Science) 2018年第6期33卷 1293-1297页

作者： KESHTKAR Javad VARGAS GARCIA jorge Roberto GALAVIZ PEREZ jorge mARTINEZ TRINIDAD José Escuela Superior de Ingenieria mecánica y Eléctrica Instituto Politécnico NacionalMéxico07738CDMXMéxico Escuela Superior de Ingenieria Quimica e Industrias Extractivas Instituto Politécnico NacionalMéxico07738CDMXMéxico Universidad Juárez Autònoma de Tabasco DivisiònAcadémica Multidisciplinaria de Jalpa de MéndezRibera AltaJalpa de MéndezTabasco86205México

Al-doped ZnO(AZO) powders were prepared by using metal chloride precursors and the sol-gel technique. IR peaks observed at 1590 cm-1 and 1620 cm-1indicated the formation of metal chelate as a consequence of the addition of acetylacetone to the metal chloride solution. TG-DSC analysis of the AZO gels confirmed the formation of metal chelate as evidenced by the development of several weight loss peaks accompanied by the introduction of new endothermic peaks. The resulting AZO gels were annealed at 500, 600, and 800 ℃ to study the effect of annealing temperature. XRD and SEm results showed that crystallization of AZO gels takes place around 600 ℃. Hexagonal wurtzite structure was identified as the main phase for all the samples. In addition, small shift of the XRD(002) peak coupled with XPS results from the AZO powders confirmed the successful doping of the ZnO powders. micron sized rod-like AZO powders were uniform in dimension and morphology and remained stable even at 800 ℃.

关键词： aluminum doped ZnO rod-like metal chloride sol gel acetylacetone

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Shear strength behavior of geotextile/geomembrane interfaces

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Journal of Rock mechanics and Geotechnical Engineering 2015年第6期7卷 638-645页

作者： Belén m.Bacas jorge Caizal Heinz Konietzky Terrasolum S.L.Geotechnical Engineering Technology Development Center of University of Cantabria(CDTUC) School of Civil Engineering University of Cantabria Geotechnical Institute TU Bergakademie Freiberg

This paper aims to study the shear interaction mechanism of one of the critical geosynthetic interfaces,the geotextile/geomembrane, typically used for lined containment facilities such as landfills. A largedirect shear machine is used to carry out 90 geosynthetic interface tests. The test results show a strainsoftening behavior with a very small dilatancy （〈0.5 mm） and nonlinear failure envelopes at a normalstress range of 25e450 kPa. The influences of the micro-level structure of these geosynthetics on themacro-level interface shear behavior are discussed in detail. This study has generated several practicalrecommendations to help professionals to choose what materials are more adequate. From the threegeotextiles tested, the thermally bonded monofilament exhibits the best interface shear strength underhigh normal stress. For low normal stress, however, needle-punched monofilaments are *** the regular textured geomembranes tested, the space between the asperities is an important *** closer these asperities are, the better the result achieves. For the irregular textured geomembranestested, the nonwoven geotextiles made of monofilaments produce the largest interface shear strength.

关键词： Geotextiles Geomembranes Landfills Fiber length Roughness Shear strength Friction angle

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Repression of the Auxin Response Pathway Increases Arabidopsis Susceptibility to Necrotrophic Fungi

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molecular Plant 2008年第3期1卷 496-509页

作者： Francisco Llorente Paul m usksktt Andrea Sanchez-Vallet Gemma Lopez Brisa Ramos Clara Sanchez-Rodriguez Lucia Jorda Jane Parker Antonio molina Centro de Biotecnologia y Genbmica de Plantas (CBGP) Departamento de Biotecnologia-UPM ETS Ingenieros Agrbnomos Avda Complutense E-28040Madrid Spain Department of Plant-microbe Interactions Max Planck Institute for Plant Breeding Research Carl-von-Linne Weg 10 50829 Cologne Germany

In plants, resistance to necrotrophic pathogens depends on the interplay between different hormone systems, such as those regulated by salicylic acid （SA）, jasmonic acid （JA）, ethylene, and abscisic acid. Repression of auxin signaling by the SA pathway was recently shown to contribute to antibacterial resistance. Here, we demonstrate that Arabidopsis auxin signaling mutants axrl, axr2, and axr6 that have defects in the auxin-stimulated SCF （Skpl-Cullin- F-box） ubiquitination pathway exhibit increased susceptibility to the necrotrophic fungi Plectosphaerella cucumerina and Botrytis cinerea. Also, stabilization of the auxin transcriptional repressor AXR3 that is normally targeted for removal by the SCF-ubiquitin/proteasome machinery occurs upon P. cucumerina infection. Pharmacological inhibition of auxin transport or proteasome function each compromise necrotroph resistance of wild-type plants to a similar extent as in non-treated auxin response mutants. These results suggest that auxin signaling is important for resistance to the necrotro- phic fungi P. cucumerina and B. cinerea. SGTlb （one of two Arabidopsis SGT1 genes encoding HSP90/HSC70 co-chaperones） promotes the functions of SCF E3-ubiquitin ligase complexes in auxin and JA responses and resistance conditioned by certain Resistance （R） genes to biotrophic pathogens. We find that sgtlb mutants are as resistant to P. cucumerina as wild-type plants. Conversely, auxin/SCF signaling mutants are uncompromised in RPP4-triggered resistance to the obligate biotrophic oomycete, Hyaloperonospora parasitica. Thus, the predominant action of SGTlb in R gene-conditioned resis- tance to oomycetes appears to be at a site other than assisting SCF E3-ubiquitin ligases. However, genetic additivity of sgtlb axrl double mutants in susceptibility to H. parasitica suggests that SCF-mediated ubiquitination contributes to lim- iting biotrophic pathogen colonization once plant-pathogen compatibility is established.

关键词： plant defense innate immunity necrotrophic fungi auxin signaling proteasome.

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Key innovations in ruminant evolution: a paleontological perspective

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Integrative Zoology 2014年第4期9卷 412-433页

作者： Daniel DemIGUEL Beatriz AZANZA jorge mORALES Catalan Institute of Paleontology miquel Crusafont Autonomus University of BarcelonaBarcelonaSpain Department of Earth Sciences University of ZaragozaUniversity Research Institute in Environmental SciencesZaragozaSpain Department of Paleobiology National Museum of Natural SciencesMadridSpain

Key innovations are newly acquired structures that permit the performance of a new function and open new adaptive zones,and are,therefore,of paramount significance for understanding the history of the Ruminantia,particularly its diversification through the *** we review and discuss what is known about these evolutionary novelties,with special emphasis on the appearance and evolution of cranial appendages and high-crowned(or hypsodont)*** appendages probably favored the diversification of pecorans by being structures strongly related to sexual selection,whereas the acquisition of hypsodont teeth could have expanded potential dietary breadth and allowed species to extend diets into the grazing range without eliminating browsing as a potential *** analyzed in conjunction with patterns of faunal diversity and in the context of climatic changes,it seems that the overall view that ruminant cranial appendages and hypsodonty may have started responding to increased patterns of seasonality and the opening-up of ecosystems is roughly ***stead,they occurred through several distinct pulses and varied widely among *** review is,to our knowledge,the first to highlight that the evolution of these innovations has been far from constant and uniform through ***,we identify that both a first attempt to increase hypsodonty starting in the early miocene and a first evolution of antlers and pronghorns were interrupted as a consequence of wide climatic fluctuations in the early-middle miocene transition.

关键词： Artiodactyla climate change cranial appendages diversity hypsodonty

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Prevalence of type 2 diabetes mellitus in the pediatric population of a third-level care hospital in mexico City in 2013 and 2018

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World Journal of Diabetes 2023年第3期14卷 290-298页

作者： jorge mario molina-Díaz Blanca Estela Vargas-Terrez Patricia Guadalupe medina-Bravo Antonia martínez-Ambrosio América Liliana miranda-Lora miguel Klünder-Klünder Department of Child Endocrinology Children’s Hospital Federico GomezAutonomous University of MexicoMexico City 06250Mexico Faculty of Business La Salle UniversityMexico City 06250Mexico Department of Endocrinology Children’s Hospital Federico GomezMexico City 06720Mexico Department of Nursing and Endocrinology Children’s Hospital Federico GómezMexico City 06720Mexico Epidemiological Research Unit in Endocrinology and Nutrition Children’s Hospital Federico GomezMexico City 06720Mexico Division of Research Children’s Hospital Federico GomezMexico City 06720Mexico

BACKGROUND Diabetes mellitus type 2(T2Dm)is a state of hyperglycemia due to a defect in the secretion and/or action of insulin,and it represents the most common form of diabetes *** mexico,10.3%of the adult population have been diagnosed with T2Dm and it is expanding to the pediatric *** To evaluate and compare the prevalence of T2Dm in the pediatric population at the Children’s Hospital,“Hospital Infantil de méxico Federico Gómez(HImFG)”,at two time periods:2013 vs *** A comparison of two cross-sectional studies was done(2013 and 2018).The study population was comprised of children and adolescents 8-17 years old,from the Diabetes Clinic at the aforementioned institution.A comprehensive interrogation regarding family history and perinatal antecedents was *** blood work after 12 h of fasting was obtained to determine serum levels of glucose,glycated hemoglobin,lipid profile,C-peptide,and *** data were analysed using the statistical software package SPSS v.23.0.A P value ofm 2013,and 104 from *** were age differences noted between the two periods with younger patients presenting T2Dm in ***,T2Dm predominated in the male sex in 55.36%in 2013 vs 32.7%in *** increased prevalence of T2Dm was noted from 2013 to 2018(20.2%vs 33.0%,respectively),which was a statistically significant 12.8%increase(Pmore aggressive in the 2018 group with the presence of a higher proportion of obesity,insulin resistance,and adverse lipid *** The prevalence of T2Dm at the HImFG institution from 2013 to 2018 increased by 12.8%(20.2%vs 33.0%,respectively).The study results demonstrate the need for vigilance in T2Dm trends,and to strengthen programs of healthy nutrition and physical activity as well as early detection and risk factors for obesity,data on insulin resistance,and metabo

关键词： Diabetes Children adolescents Epidemiology Etiology Risk factors and complications

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The analysis of composite laminated beams using a 2D interpolating meshless technique

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Acta mechanica Sinica 2018年第1期34卷 99-116页

作者： S.H.m.Sadek J.Belinha m.P.L.Parente R.m.Natal jorge J.m.A.César de Sá A.J.m.Ferreira Department of mechanical Engineering Faculty of EngineeringUniversity of Porto Institute of mechanical Engineering and Industrial management University of Porto

Laminated composite materials are widely implemented in several engineering constructions. For its relative light weight, these materials are suitable for aerospace, military, marine, and automotive structural applications. To obtain safe and economical structures, the modelling analysis accuracy is highly relevant. Since meshless methods in the recent years achieved a remarkable progress in computational mechanics, the present work uses one of the most flexible and stable interpolation meshless technique available in the literature—the Radial Point Interpolation method(RPIm).Here, a 2 D approach is considered to numerically analyse composite laminated beams. Both the meshless formulation and the equilibrium equations ruling the studied physical phenomenon are presented with detail. Several benchmark beam examples are studied and the results are compared with exact solutions available in the literature and the results obtained from a commercial finite element software. The results show the efficiency and accuracy of the proposed numeric technique.

关键词： Radial point interpolators RPIm Laminated beam meshless method

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On the possibility that PbZrO_(3) not be antiferroelectric

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npj Computational materials 2021年第1期7卷 1812-1821页

作者： Hugo Aramberri Claudio Cazorla massimiliano Stengel jorge Íñiguez materials Research and Technology Department Luxembourg Institute of Science and Technology5 Avenue des Hauts-FourneauxL-4362 Esch/AlzetteLuxembourg Departament de Física Universitat Politècnica de CatalunyaCampus Nord B4-B5Barcelona 08034Spain Institut de Ciència de materials de Barcelona(ICmAB-CSIC) Campus UAB08193 BellaterraSpain ICREA-InstitucióCatalana de Recerca i Estudis Avançats 08010 BarcelonaSpain Department of Physics and materials Science University of Luxembourg41 Rue du BrillBelvaux L-4422Luxembourg

Lead zirconate(PbZrO_(3))is considered the prototypical antiferroelectric material with an antipolar ground ***,several experimental and theoretical works hint at a partially polar behaviour in this compound,indicating that the polarization may not be completely *** this work,we propose a simple ferrielectric structure for lead ***-principles calculations reveal this state to be more stable than the commonly accepted antiferroelectric phase at low temperatures,possibly up to room temperature,suggesting that PbZrO_(3)may not be antiferroelectric at ambient *** discuss the implications of our discovery,how it can be reconciled with experimental observations and how the ferrielectric phase could be obtained in practice.

关键词： ferroelectric ambient phase

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A new physical simulation tool to predict the interface of dissimilar aluminum to steel welds performed by friction melt bonding

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Journal of materials Science & Technology 2019年第9期35卷 2048-2057页

作者： T.Sapanathan N. Jimenez-mena I. Sabirov m.A. monclus J.m. molina-Aldaregui P. Xia L. Zhao A. Simar Institute of mechanics Materials and Civil EngineeringUCLouvain1348 Louvain-la-NeuveBelgium ImDEA materials Institute Calle Eric Kandel 228906 GetafeMadridSpain Universidad Politicnica de madrid E.T.S. de Ingenieros de Caminos28040 MadridSpain

Optimization of the intermetallic layer thickness and the suppression of interfacial defects are key elements to improve the load bearing capacity of dissimilar joints. However, till date we do not have a systematic tool to investigate the dissimilar joints and the intermetallic properties produced by a welding condition. Friction melt Bonding (FmB) is a recently developed technique for joining dissimilar metals that also does not exempt to these challenges. The FmB of DP980 and A16061-T6 was investigated using a new physical simulation tool, based on Gleeble thermo-mechanical simulator, to understand the effect of individual parameter on the intermetallic formation. The proposed method demonstrates its capability in reproducing the intermetallic characteristics, including the thickness of intermetallic bonding layer, the morphology and texture of its constituents (Fe2AU and Fe4Ali3), as well as their nanohardness and reduced modulus. The advantages of physical simulation tool can enable novel developing routes for the development of dissimilar metal joining processes and facilitate to reach the requiring load bearing capacity of the joints.

关键词： Physical simulation Interface Intermetallics Nanoindentation Joining

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Analysis of the testimonial evidence of Portugal's first major forensic case:part Ⅱ

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Forensic Sciences Research 2020年第4期5卷 266-285页

作者： Ricardo jorge Dinis-Oliveira Department of Public Health and Forensic Sciences and Medical EducationFaculty of MedicineUniversity of PortoPortoPortugal IINFACTS-Institute of Research and Advanced Training in Health Sciences and Technologies Department of SciencesUniversity Institute of Health Sciences(IUCS)Advanced Polytechnic and University Cooperative(CESPU)CRLGandraPortugal UCIBIO-REQUImTE Laboratory of Toxicology

The crime possibly perpetrated by a doctor named Vicente Urbino de Freitas in 1890 is one of the most famous cases of poisoning,and it had echoes in the Portuguese and foreign press for several *** prestigious doctor was convicted of the fatal poisoning of his *** also attempted the homicide of two nieces and their mother-in-law,who only escaped because they obstinately refused to comply with the"therapeutics"prescribed by the family *** motive of the crime should have been Vicente Urbino de Freitas'ambition to receive the family inheritance of his wife,the daughter of the well-known mer-chant Jose Antonio Sampaio of Flores Street in *** Urbino de Freitas was con-victed but doubt about his guilt persists for more than a *** second work aimed to collect and analyse all the relevant and contradictory testimonial evidence of the prosecu-tion and defence *** case represents an odd historical record obtained through more than 12years of research on the first major significant Portuguese forensic *** and unprecedented testimonial evidence and photographs were obtained from different countries and then repaired,since these also provide an important historical record of the medical photography.

关键词： History of legal medicine forensic sciences forensic toxicology Vicente Urbino de Freitas mário Guilherme Augusto de Sampaio testimonial evidence Flores Street

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