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Two novel mutations in theGAN gene causing giant axonal neuropathy

World Journal of Pediatrics 2018年第3期14卷 298-304页

作者： Monica Irad Normendez-Martínez Lucero Monterde-Cruz Roberto Martínez Magdalena Marquez-Harper Nayelli Esquitin-Garduno Margarita Valdes-Flores leonora Casas-Avila valeria ponce de leon-suarez Viktor Javier Romero-Díaz Alberto Hidalgo-Bravo Medical Specialties Hospital Boulevard Milenio 130San Carlos la Roncha37660 LeonGuanajuatoMexico department of Gene tics National Institute of R ehabilitationAvenue Mexico-Xochimilco 28914389 Me xico CityMexico department ofHis tology School ofMedicineAutonomous University oft heS tate ofN uevo LeonMonterreyMexico

Background Giant axonal neuropathy (GAN) is a rare neurodegenerative disease transmitted in an autosomal recessive mode. This disorder presents motor and sensitive symptoms with an onset in early childhood. Progressive neurodegeneration makes the patients wheelchair dependent by the end of the second decade of life. Affected individuals do not survive beyond the third decade of life. Molecular analysis has identified mutations in the gene GAN in patients with this disorder. This gene produces a protein called gigaxonin which is presumably involved in protein degradation via the ubiquitin-proteasome system. However, the underlying molecular mechanism is not clearly understood yet. Methods Here we present the fi rst patient from Mexico with clinical data suggesting GAN. Sequencing of the GAN gene was carried out. Changes in the nucleotide sequence were investigated for their possible impact on protein function and structure using the publicly available prediction tools PolyPhen-2 and PANTHER. Results The patient is a compound heterozygous carrying two novel mutations in the GAN gene. The sequence analy-sis revealed two missense mutations in the Kelch repeats domain. In one allele, a C>T transition was found in exon 9 at the nucleotide position 55393 (g.55393C>T). In the other allele, a transversion G>T in exon 11 at the nucleotide position 67471 (g.67471G>T) was observed. Both of the bioinformatic tools predicted that these amino acid substitutions would have a negative impact on gigaxonin's function. Conclusion This work provides useful information for health professionals and expands the spectrum of disease-causing mutations in the GAN gene and it is the first documented case in Mexican population.

关键词： GAN Genetics Giant axonal neuropathy Incapacity Neurodegenerative

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Current Progress and Future Perspectives of Electrolytes for Rechargeable Aluminum-Ion Batteries

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Energy & Environmental Materials 2023年第1期6卷 25-42页

作者： Dongwei Ma Du Yuan Carlos ponce de leon Zheng Jiang Xin Xia Jiahong Pan MOE Key Laboratory of Resources and Environmental Systems Optimization College of Environmental Science and EngineeringNorth China Electric Power UniversityBeijing 102206China College of Materials Science and Engineering Changsha University of Science and TechnologyChangsha 410004China Electrochemical Engineering Department of Engineering and Physical SciencesUniversity of SouthamptonSouthampton UK Beijing Key Laboratory of Novel Thin Film Solar Cells North China Electric Power UniversityBeijing 102206China

Aluminum-ion batteries(AIBs)with Al metal anode are attracting increasing research interest on account of their high safety,low cost,large volumetric energy density(≈8046 mA h cm−3),and environmental ***,the reversible Al electrostripping/deposition is achieved with the rapid development of room temperature ionic liquids,and rapid progress has been made in fabricating high-performance and durable AIBs during the past *** review provides an integrated comprehension of the evolution of AIBs and highlights the development of various non-aqueous and aqueous electrolytes including high-temperature molten salts,room temperature ionic liquids,and gel–polymer *** critical issues on the interplay of electrolytes are outlined in terms of the voltage window span,the effective ion species during charge storage(Al 3+or Al x Cl?y)and their underlying charge transfer(e.g.,interfacial transfer and diffusion),and the solid electrolyte interface formation and its *** the critical insight,future perspectives on how to practically design feasible AIBs are given.

关键词： aluminum-ion batteries electrochemical energy storage electrolytes ionic liquids solid electrolyte interface

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Pressure drop analysis on the positive half-cell of a cerium redox flow battery using computational fluid dynamics: mathematical and modelling aspects of porous media

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Frontiers of Chemical Science and Engineering 2021年第2期15卷 399-409,F0004页

作者： Fernando F.Rivera Berenice Miranda-Alcantara German Orozco Carlos ponce de leon Luis F.Arenas National Council of Science and Technology(CONACYT) Mexico City 03940Mexico Center of Research and Technological development in Electrochemistry(CIdeTEQ) Queretaro 76703Mexico Electrochemical Engineering Laboratory Energy Technology Research GroupFaculty of Engineering and Physical SciencesUniversity of SouthamptonSouthampton SO171BJUK

description of electrolyte fluid dynamics in the electrode compartments by mathematical models can be a powerful tool in the development of redox flow batteries(RFBs)and other electrochemical *** order to determine their predictive capability,turbulent Reynolds-averaged Navier-Stokes(RANS)and free flow plus porous media(Brinkman)models were applied to compute local fluid velocities taking place in a rectangular channel electrochemical flow cell used as the positive half-cell of a cerium-based RFB for laboratory *** different platinized titanium electrodes were considered,a plate plus a turbulence promoter and an expanded metal *** pressure drop was validated against experimental data obtained with typical cerium *** was found that the pressure drop values were better described by the RANS approach,whereas the validity of Brinkman equations was strongly dependent on porosity and permeability values of the porous media.

关键词： CFD simulation porous media porous electrode pressure drop redox flow battery

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Dysregulation of the PD-1/PD-L1 pathway contributes to the pathogenesis of celiac disease

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Cellular & Molecular Immunology 2019年第9期16卷 777-779页

作者： Candelaria ponce de leon Miguel Angel Lopez-Casado Pedro Lorite Teresa Palomeque Maria Isabel Torres department of Experimental Biology University of JaenJaenSpain department of Pediatrics Gastroenterology Hospital Virgen de las NievesGranadaSpain

In this study,we focus on the alteration of the programmed cell death 1(PD-1)/PD-L1 pathway in celiac *** discuss the diverse roles of the PD-1 pathway in regulating immune responses and how this knowledge can improve celiac disease(CD)*** PD-1 and PD-L1 levels in the serum and in intestinal biopsies of CD patients may be relevant to the determination of a possible correlation between markers of the autoimmune response,inflammation,and disease activity.

关键词： celiac pathogenesis inflammation

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RANS simulations of the U and V grooves effect in the subcritical flow over four rotated circular cylinders

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Journal of Hydrodynamics 2015年第4期27卷 569-578页

作者： ALONZO-GARCIA A. GUTIERREZ-TORRES C.del C. JIMENEZ BERNAL J.A. MOLLINEDO-ponce de leon H.R. MARTINEZ-deLGADILLO S.A. BARBOSA-SALDANA J.G. Instituto Politécnico Nacional SEPI ESIME ZacatencoLABINTHAP U.P.Adolfo López Mateos Edif.5 3er.PisoMéxicoD.F.México Instituto Politécnico Nacional UPIITAAv.IPN 2580C.P.07340MéxicoD.F.México Universidad Autónoma Metropolitana-Azcapotzalco Depto.Ciencias BásicasAv.San Pablo 180Azcapotzalco C.P 07740MéxicoD.F.México

This paper presents a CFD study about the effect of the V and U grooves in the flow over four cylinders in diamond shape configuration at subcritical flow conditions（Re= 41 000）. The k- ε Realizable turbulence model was implemented to fully structured hexahedral grids with near-wall refinements. Results showed that the numerical model was able to reproduce the impinging flow pattern and the repulsive forces present in the lateral cylinders of the smooth cylinder array. As a consequence of the flow alignment induced by the grooves, a jet-flow is formed between the lateral cylinders, which could cause an important vortex induced vibration effect especially in the rear cylinder. The magnitudes of the shear stresses at the valleys and peaks for the V grooved cylinders were lower than those of the U grooved cylinders, but the separation points were delayed due the U grooves presence. It is discussed the presence of a blowing effect caused by counter-rotating eddies located near the grooves peaks that cause a decrease of the shear stresses in the valleys, and promote them at the peaks.

关键词： RANS model grooved cylinder four cylinders subcritical flow impinging flow

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Does parity worsen diabetes-related chronic complications in women with type 1 diabetes?

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World Journal of Diabetes 2016年第12期7卷 252-259页

作者： Marilia Brito Gomes Carlos Antonio Negrato Ana Almeida Antonio ponce de leon department of Internal Medicine Diabetes Unit State University of Rio de Janeiro Rio de Janeiro 20550-900 Brazil Bauru’s Diabetics Association Bauru SPaulo 17012-433 Brazil department of Epidemiology State University of Rio de Janeiro Rio de Janeiro 20550-900 Brazil

AIM: To determine the relationship between parity, glycemic control, cardiovascular risk factors and diabetesrelated chronic complications in women with type 1 ***: This was a multicenter cross-sectional study conducted between december 2008 and december 2010 in 28 public clinics in 20 cities from the 4 Brazilian geographic regions. Data were obtained from 1532 female patients, 59.2% Caucasians, and aged 25.2 ± 10.6 years. Diabetes duration was of 11.5 ± 8.2 years. Patient's information was obtained through a questionnaire and a chart review. Parity was stratified in five groups: Group 0(nulliparous), group 1(1 pregnancy), group 2(2 pregnancies), group 3(3 pregnancies), group 4(≥ 4 pregnancies). Test for trend and multivariate random intercept logistic and linear regression models were used to evaluate the effect of parity upon glycemic control, cardiovascular risk factors and diabetes-related complications. RESULTS: Parity was not related with glycemic control and nephropathy. Moreover, the effect of parity upon hypertension, retinopathy and macrovascular disease did not persist after adjustments for demographic and clinical variables in multivariate analysis. For retinopathy, the duration of diabetes and hypertension were the most important independent variables and for macrovascular disease, these variables were age and hypertension. Overweight or obesity was noted in a total of 538 patients(35.1%). A linear association was found between the frequency of overweight or obesity and parity(P = 0.004). Using a random intercept multivariate linear regression model with body mass index(BMI) as dependent variable a borderline effect for parity(P = 0.06) was noted after adjustment for clinical and demographic data. The observed variability of BMI was not attributable to differences between ***: Our results suggest that parity has a borderline effect on body mass index but does not have an important effect upon hypertension and micro or macrova

关键词： Type 1 diabetes Parity Glycemic control Cardiovascular risk factors Diabetes-related chronic complications

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Effect of paricalcitol and enalapril on renal inflammation/oxidative stress in atherosclerosis

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World Journal of Biological Chemistry 2015年第3期6卷 240-248页

作者： Kazim Husain Edu suarez Angel Isidro Wilfredo Hernandez leon Ferder department of Physiology Pharmacology and Toxicology Ponce School of Medicine and Health Sciences department of Biochemistry Ponce School of Medicine and Health Sciences

AIM: To investigate the protective effect of paricalcitol and enalapril on renal inflammation and oxidative stress in Apo E-knock out mice. METHODS: Animals treated for 4 mo as group(1) Apo E-knock out plus vehicle, group(2) Apo E-knock out plus paricalcitol(200 ng thrice a week),(3) Apo Eknock out plus enalapril(30 mg/L),(4) Apo E-knock out plus paricalcitol plus enalapril and(5) normal. Blood pressure(BP) was recorded using tail cuff method. The kidneys were isolated for biochemical assays using spectrophotometer and Western blot analyses. RESULTS: Apo E-deficient mice developed high BP(127 ± 3 mm Hg) and it was ameliorated by enalapril and enalapril plus paricalcitol treatments but not with paricalcitol alone. Renal malondialdehyde concentrations, p22 phox, manganese-superoxide dismutase, inducible nitric oxide synthase(NOS), monocyte chemoattractant protein-1, tumor necrosis factor-alpha and transforming growth factor-β1 levels significantly elevated but reduced glutathione, Cu Zn-SOD and e NOS levels significantly depleted in Apo E-knock out animals compared to normal. Administration of paricalcitol, enalapril and combined together ameliorated the renal inflammation and oxidative stress in Apo E-knock out animals. CONCLUSION: Paricalcitol and enalapril combo treatment ameliorates renal inflammation as well as oxidative stress in atherosclerotic animals.

关键词： Atherosclerosis Enalapril Paricalcitol Renal inflammation Oxidative stress

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Evaluating the role of fire over a decade in a tropical mountainous forestgrassland mosaic

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Journal of Mountain Science 2024年第12期21卷 4191-4207页

作者： Juliana KUCHENBECKER Yumi OKI Flávio CAMAROTA Frederico S.NEVES Diego R.MACEDO Bárbara SILVA FERREIRA Ramiro AGUILAR Lorena ASHWORTH Ezequiel FABIANO Bárbara DIAS ARAÚJO Amanda ponce de leon Bruna RIBEIRO SILVA Thamyris BRAGIONI Fernando FIGUEIREDO GOULART JoséEugênio CÔRTES FIGUEIRA Geraldo WILSON FERNANdeS departamento de Genética Ecologia e EvoluçãoInstituto de Ciências BiológicasUniversidade Federal de Minas Gerais-UFMGBelo HorizonteMG 31270-901Brazil Programa de Pós-graduação Biodiversidade e Uso de Recursos Naturais Universidade Estadual de Montes Claros-UNIMONTESMontes ClarosMG 39401-089Brazil departamento de Geografia Instituto de GeociênciasUniversidade Federal de Minas Gerais-UFMGBelo HorizonteMG 31270-901Brazil Instituto Multidisciplinario de Biologia Vegetal CONICET-Universidad Nacional de CórdobaX5000JJCCórdobaArgentina department of Wildlife Management and Ecotourism University of NamibiaKatima MuliloKatima Mulilo 20001Namíbia departamento de Botânica Instituto de Ciências BiológicasUniversidade Federal de Minas Gerais-UFMGBelo HorizonteMG 31270-901Brazil Knowledge Center for Biodiversity Belo HorizonteMG 31270-901Brazil

Forest-grassland mosaics comprise a major component of tropical landscapes,hosting invaluable biodiversity and providing essential ecosystem services to hundreds of millions of people *** open biomes often benefit from disturbance,forests can particularly be susceptible to structural changes resulting from such *** we evaluate the influence of fire on the structure and landscape properties within natural forest islands immersed in a matrix of megadiverse montane *** conducted this study in 15 forest islands located in southeastern Brazil,assessing its fire frequency,intensity,and post-fire time over an eleven-year period from January 2012 to december *** results show that fire frequency is linked to soil characteristics and the percentage of herbaceous cover within the forest *** also found that the post-fire time is related to the percentage cover of the forest islands’associated herbs and ***,neither fire frequency,intensity,nor post-fire time was connected to significant changes in plant species richness,abundance,or in the upper vegetation strata(tree species richness and abundance,and canopy cover)in the interior of the forest ***,these fire-related variables did not result in temporal changes in the forest island’s canopy variation or landscape *** results underscore a low fire frequency and intensity within our study area,potentially explaining the limited fire-associated impact,and primarily on the lower vegetation *** acknowledging the relative stability of these forest islands under current fire regimes,we suggest further studies that can experimentally manipulate not only fire but also other anthropic disturbances for understanding the temporal dynamics of the forest islands and,consequently,their *** perspective is indispensable for comprehensively understanding the ecological consequences of anthropogenic disturbances in natural forest islands.

关键词： Campo Rupestre Fire Ecology Forest islands Mountain ecosystems Remote Sensing Serra do Cipó

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First report of L1014F kdr mutation in Culex quinquefasciatus in Mexico

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Insect Science 2016年第6期23卷 829-834页

作者： Gustavo ponce Iram P. Sanchez Selene M. Garcia Jose M. Torrado Saul Lozano-Fuentes Beatriz Lopez-Monroy Adriana E. Flores Facultad de Ciencias Biologicas Universidad Autonoma de Nuevo Leon A v. Universidad sin Cd. Universitaria San Nicolas de los GarzaN.L. 66455 Mexico department of Microbiology Immunology and Pathology Colorado State University Fort Collins CO USA

The L1014F mutation in the voltage-sodium channel gene has been associated with resistance to DDT and pyrethroids in various arthropod species including mosquitoes. We determined the frequency of the L1014F kdr mutation in 16 field populations of Culex quinquefasciatus from Northeastern Mexico collected between 2008 and 2013. The L1014F was present in all populations analyzed with the lowest frequency （3.33%） corresponding to the population from Monclova collected in 2012, and the highest frequency （63.63%） from the Monterrey population collected in 2012. The presence of a kdr mutation in populations of Cx. quinquefasciatus from northeastern Mexico provides evidence ofpyrethroid resistance. This requires a special attention, considering that pyrethroid-based insecticides are commonly used in vector-control campaigns, especially against Aedes aegypti （L.）.

关键词： Culex quinquefasciatus insecticide resistance kdr L 1014F mutation

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Impact of payment source, referral site, and place of residence on outcomes after allogeneic transplantation in Mexico

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World Journal of Transplantation 2024年第2期14卷 76-87页

作者： Andrés Gómez-de León Yesica A López-Mora valeria García-Zárate Ana Varela-Constantino Sergio U Villegas-de leon Xitlaly J González-Leal Raúl del Toro-Mijares Anna C Rodríguez-Zúñiga Juan F Barrios-Ruiz Victor Mingura-Ledezma Perla R Colunga-Pedraza Olga G Cantú-Rodríguez César H Gutiérrez-Aguirre Luz Tarín-Arzaga Elías E González-López David Gómez-Almaguer Hematology Service Universidad Autónoma de Nuevo LeónFacultad de Medicina y Hospital Universitario Dr.JoséEleuterio GonzálezMonterrey 64460Nuevo LeónMexico Hemato-logy Service Clínica Gómez AlmaguerMonterrey 64710Nuevo LeónMexico Hematology Service Instituto Tecnológico de Estudios Superiores de MonterreyTec SaludEscuela de Medicina Ignacio SantosMonterrey 64710Nuevo LeónMexico

BACKGROUND The impact of social determinants of health in allogeneic transplant recipients in low-and middle-income countries is poorly *** observational study analyzes the impact of place of residence,referring institution,and transplant cost coverage(out-of-pocket vs government-funded vs private insurance)on outcomes after allogeneic hematopoietic stem cell transplantation(alloHSCT)in two of Mexico's largest public and private *** To evaluate the impact of social determinants of health and their relationship with outcomes among allogeneic transplant recipients in *** In this retrospective cohort study,we included adolescents and adults≥16 years who received a matched sibling or haploidentical transplant from *** were selected without regard to their diagnosis and were sourced from both a private clinic and a public University Hospital in *** payment groups were compared:Out-of-pocket(OOP),private insurance,and a federal Universal healthcare program“Seguro Popular”.Outcomes were compared between referred and institution-diagnosed patients,and between residents of Nuevo leon and *** outcomes included overall survival(OS),categorized by residence,referral,and payment *** outcomes encompassed early mortality,event-free-survival,graft-versus-host-relapse-free survival,and non-relapse-mortality(NRM).Statistical analyses employed appropriate tests,Kaplan-Meier method,and Cox proportional hazard regression *** software included SPSS and R with tidycmprsk *** Our primary outcome was overall *** included 287 patients,n=164 who lived out of state(57.1%),and n=129 referred from another institution(44.9%).The most frequent payment source was OOP(n=139,48.4%),followed by private insurance(n=75,26.1%)and universal coverage(n=73,25.4%).No differences in OS,event-free-survival,NRM,or graft-versus-host-relapse-free survival were observed for patients dia

关键词： Hematopoietic cell transplant Social determinants of health Geography Haploidentical Out-of-pocket Financial toxicity Survival Health services and outcomes Hematopoietic malignancy Aplastic anemia

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