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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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More Accurate Climate Models for Better Prediction—The Case of the Precis Model in Burkina Faso,Ghana and Mali

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地学前缘 2009年第S1期16卷 101-102页

作者： Placide Beyon Hien monica Idinoba Department of Environment and Sustainable Development University of Ouagadougou Centre for International Forestry Research

West Africa is a region where not only the climate has seen some of the most dramatic changes but also climate models carry the greatest level of uncertainty. Designing an accurate climate model can be *** many cases,while the models may be relatively accurate at the global level,they show important discrepancies compared to reality when applied at a smaller *** the correct climate

关键词： rain West Africa climate rainfall

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Organic acids production from lactic acid bacteria:A preservation approach

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Food Bioscience 2022年第2期46卷 959-974页

作者： Sneh Punia Bangar Shweta Suri monica Trif Fatih Ozogul Department of Food Nutrition and Packaging SciencesClemson UniversityClemson29631USA Department of Food Engineering National Institute of Food Technology Entrepreneurship and ManagementSonipatHaryana131028India Centre for Innovative Process Engineering(CENTIV)GmbH 28857SykeGermany CENCIRA Agrofood Research and Innovation Centre 400650Cluj-NapocaRomania Cukurova University Faculty of FisheriesDepartment of Seafood Processing Technology01330AdanaTurkey

The lactic acid bacteria(LAB)are a group of gram-positive bacteria capable of converting carbohydrate substrates into organic acids and producing a wide range of *** acids,including propionic,formic,acetic acid,and lactic acid,create an unfavorable environment for the growth of spoilage and pathogenic ***,they are employed as monomers or starting ingredients for food-based supplements,biomaterials,and biodegradable *** acids from LAB have the potential to prevent spoilage and improve food taste that enhances consumer acceptance and *** acids can be useful as starting materials in multiple applications,including food,chemical,cosmetic,pharmaceutical,and beverage industries owing to their various functional ***,the use of LAB for organic acids production can replace petroleum-based processes due to their ability to utilize inexpensive and renewable *** article aims to provide the potential of LAB as a biological catalyst for producing different organic ***,the current review will give a critical snapshot of the strategies to improve microbial organic acid production and potential application of organic acids and discuss new challenges and opportunities for developing novel economically effective and sustainable nutritional sources and techniques.

关键词： Lactic acid bacteria Organic acids Synthesis Mechanism Applications

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Effective Opinion Spam Detection: A Study on Review Metadata Versus Content

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Journal of Data and Information Science 2020年第2期5卷 76-110页

作者： Ajay Rastogi monica Mehrotra Syed Shafat Ali Department of Computer Science Jamia Millia IslamiaNew DelhiIndia

Purpose:This paper aims to analyze the effectiveness of two major types of features—metadata-based(behavioral)and content-based(textual)—in opinion spam ***/methodology/approach:Based on spam-detection perspectives,our approach works in three settings:review-centric(spam detection),reviewer-centric(spammer detection)and product-centric(spam-targeted product detection).Besides this,to negate any kind of classifier-bias,we employ four classifiers to get a better and unbiased reflection of the obtained *** addition,we have proposed a new set of features which are compared against some well-known related *** experiments performed on two real-world datasets show the effectiveness of different features in opinion spam ***:Our findings indicate that behavioral features are more efficient as well as effective than the textual to detect opinion spam across all three *** addition,models trained on hybrid features produce results quite similar to those trained on behavioral features than on the textual,further establishing the superiority of behavioral features as dominating indicators of opinion *** features used in this work provide improvement over existing features utilized in other related ***,the computation time analysis for feature extraction phase shows the better cost efficiency of behavioral features over the *** limitations:The analyses conducted in this paper are solely limited to two wellknown datasets,viz.,Yelp Zip and Yelp NYC of *** implications:The results obtained in this paper can be used to improve the detection of opinion spam,wherein the researchers may work on improving and developing feature engineering and selection techniques focused more on metadata ***/value:To the best of our knowledge,this study is the first of its kind which considers three perspectives(review,reviewer and product-centric)and four classifiers to analyze the effecti

关键词： Opinion spam Behavioral features Textual features Review spammers Spam-targeted products

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An innovative bio-engineering retaining structure for supporting unstable soil

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Journal of Rock Mechanics and Geotechnical Engineering 2017年第2期9卷 247-259页

作者： Gianluca Bella monica Barbero Fabrizio Barpi Mauro Borri-Brunetto Daniele Peila DISEG Politecnico di TorinoCorso Duca degli Abruzzi 24Torino10129Italy DIATI Politecnico di TorinoCorso Duca degli Abruzzi 24Torino10129Italy

The paper presents a new prefabricated bio-engineering structure for the support of unstable *** prefabricated structure is made of a steel frame which is completely filled with soil and a face made of tree trunks among which scions or autochthonous bushes are *** to the difficulties in interpreting the complex interaction between soil and structure during the installation and lifetime,an in situ test was carried out in order to evaluate the state of stress in the steel frame and to understand the global behavior of the structure under service *** the basis of the obtained results,a procedure for checking the structure safety was proposed and *** easy design method was developed during the ***,the use of this type of prefabricated structure shows several advantages,such as good performances in terms of stabilizing effects,and easy assembly and transport.

关键词： Bio-engineering Test site Landslide Live cribwall Unstable soil

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Canopy Roughness:A New Phenotypic Trait to Estimate Aboveground Biomass from Unmanned Aerial System

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Plant Phenomics 2020年第1期2卷 419-428页

作者： monica Herrero-Huerta Alexander Bucksch Eetu Puttonen Katy M.Rainey Department of Agronomy Purdue UniversityWest LafayetteINUSA Department of Cartographic and Land Engineering Higher Polytechnic School of AvilaUniversity of SalamancaAvilaSpain Institute for Plant Sciences College of AgriculturePurdue UniversityWest LafayetteINUSA Department of Plant Biology University of GeorgiaAthensGAUSA Warnell School of Forestry and Natural Resources University of GeorgiaAthensGAUSA Institute of Bioinformatics University of GeorgiaAthensGAUSA Finnish Geospatial Research Institute National Land Survey of FinlandMasalaFinland

Cost-effective phenotyping methods are urgently needed to advance crop genetics in order to meet the food,fuel,and fiber demands of the coming ***,characterizing plot level traits in fields is of particular *** developments in highresolution imaging sensors for UAS(unmanned aerial systems)focused on collecting detailed phenotypic measurements are a potential *** introduce canopy roughness as a new plant plot-level *** tested its usability with soybean by optical data collected from UAS to estimate *** validate canopy roughness on a panel of 108 soybean[Glycine max(L.)Merr.]recombinant inbred lines in a multienvironment trial during the R^(2) growth stage.A senseFly eBee UAS platform obtained aerial images with a senseFly *** digital *** a structure from motion(SfM)technique,we reconstructed 3D point clouds of the soybean experiment.A novel pipeline for feature extraction was developed to compute canopy roughness from point *** used regression analysis to correlate canopy roughness with field-measured aboveground biomass(AGB)with a leave-one-out ***,our models achieved a coefficient of determination(R^(2))greater than 0.5 in all ***,we found that canopy roughness has the ability to discern AGB variations among different *** test trials demonstrate the potential of canopy roughness as a reliable trait for high-throughput phenotyping to estimate *** such,canopy roughness provides practical information to breeders in order to select phenotypes on the basis of UAS data.

关键词： soybean Biomass roughness

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Do subtropical grasslands recover spontaneously after afforestation?

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Journal of Plant Ecology 2019年第2期12卷 228-234页

作者： Fábio Piccin Torchelsen monica Cadenazzi Gerhard Ernst Overbeck Departamento de Botânica Instituto de BiociênciasUniversidade Federal do Rio Grande do Sul-UFRGSAv.Bento Gonçalves 9500-Bloco IV-Prédio 43433Porto AlegreRSCEP 91501-970Brazil Facultad de Agronomía Universidad de La Republica-UdelaRPaysandúUruguay

Aims South American Pampa grasslands are habitats of great conservation interest,with a distinct and rich flora,but have been intensely con-verted to other land uses,including tree *** neces-sity for restoration grows,no information on restoration potential of grasslands after afforestation ***,we aim at analyzing composition and structure of grassland vegetation with a history of eucalyptus plantations in order to assess recovery potential of these *** hypothesized that areas with history of eucalyptus would differ from reference grasslands with no history of land-use change in terms of floristic and functional composition and would present lower species *** Our study region comprised four sites in the southeastern part of the Pampa biome,in the coastal plain in the extreme south of Brazil,always with sites with long(50 years)history of eucalyptus plantation and reference *** sampled vegetation at post-eucalyptus sites(with and without resprouting)10 years after clear-clutting and in natural *** analyzed data by analysis of variance and ordination techniques,considering compositional parameters and life forms,and indicator species *** Findings Species richness and vegetation cover were higher in reference grassland than in resprout areas but did not differ from post-eucalyp-tus *** species cover was significantly higher in areas with afforestation *** terms of total composition,natural grasslands differed significantly from areas with past plantation *** species analyses revealed considerable differences between grass-land *** conclusion,vegetation development led to grassland communities that are still quite distinct from reference ***,the specificity of grassland management has a high importance in defining vegetation trajectories in time and importance for grassland recovery,and restoration needs to be addressed in more studies.

关键词： Campos Sulinos conservation Pampa restoration southern Brazil vegetation management

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Effects of obstructive sleep apnea and its treatment over the erectile function： a systematic review

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Asian Journal of Andrology 2017年第3期19卷 303-310页

作者： Felix Campos-Juanatey Marcos Fernandez-Barriales monica Gonzalez Jose A Portillo-Martin Urology Department Marques de Valdecilla University Hospital Santander 39008 Spain Institute of Investigation Marques de Valdecilla Santander 39011 Spain Oral and Maxillofacial Surgery Department Araba University Hospital Araba 01004 Spain Sleep and Ventilation Unit Marques de Valdecilla University Hospital Santander 39008 Spain Medical and Surgical Sciences Department School of Medicine University of Cantabria Santander 39011 Spain.

Erectile dysfunction （ED） is considered a condition with a broad range of etiologies. Obstructive sleep apnea （OSA） syndrome is one of the lesser studied risk factors for ED. We intend to summarize the current evidence on the relationship between OSA and sexual impairment, focusing on the results in terms of erectile function of the different therapies offered to OSA patients. A systematic review was conducted, selecting articles related to the physiology of OSA and ED, and to the treatments of OSA syndrome and their reported outcomes in erectile and sexual function. Higher prevalences of ED in the OSA groups have been published. However, whether this effect on the erectile function occurs in the entire range of OSA severities remains unclear. Several hypotheses were proposed to explain the physiology of this association. Continuous Positive Airway Pressure as a treatment for OSA patients with ED has achieved a significative improvement in the sexual parameters in most of the studies. Phosphodiesterase type 5 inhibitors （iPDE5） on demand are useful as a treatment for ED in this subgroup of patients, with high satisfaction rates. The surgical treatment for the OSA evidenced benefits over the erectile function, and the effect on the sexual satisfaction of the therapy using Mandibular Advancement Devices is still undefined.

关键词： continuous positive airway pressure erectile dysfunction obstructive sleep apnea operative surgical procedures phosphodiesterase type 5 inhibitors

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Loss of largest and oldest individuals of the Montpellier snake correlates with recent warming in the southeastern Iberian Peninsula

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Current Zoology 2017年第6期63卷 607-613页

作者： Cosme LOPEZ-CALDERON monica FERICHE Esmeralda ALAMINOS Juan M. PLEGUEZUELOS Department of Zoology Faculty of Biology University of Seville C/Reina Mercedes Seville E-41012 Spain Department of Zoology Faculty of Sciences University of Granada Granada E-18071 Spain

The effects of climate change on organisms are now being extensively studied in many different taxa. However, the variation in body size, usually shrinkage in response to increasing temperature, has received little attention regarding to reptiles. During past periods of global warming, many organisms shrank in size, and current evidence and experiments manipulating temperature have shown a biomass decrease in some organisms with increasing temperatures. Here we test whether the body size of the Montpellier snake Malpolon rnonspessulanus from the southeastern Iberian Peninsula is changing and correlated with the increasing temperature in this region during a 39- year period （1976-2014）. We measured the snout-vent length （SVL） of vouchers in scientific collections to check for trends in adult body size at the population level in relation with temperature, while controlling for the age of the individuals （estimated by skeletochronology, n =141）. Given the great ontogenetic variation in body size of the study species, we categorized age in 3 classes：＂young adults＂（under 5 years old）, ＂intermediate adults＂（from 5 to 7 years old）, and ＂old adults＂（from 8 to 14 years old）. By means of linear mixed models, we found a negative relationship between SVL of ＂old adults＂ and average annual temperature in the region during the lifetime of each individual. Our results indicate that largest and oldest individuals of the Montpellier Snake, that is, males because of strong sexual size dimorphism in this species, disappeared from the study population, and suggest that it occurred in response to rising environmental temperature.

关键词： body shrinkage climate warming differential mortality Malpolon monspessulanus skeletochronology snakes Spain.

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Transcript Accumulation Dynamics of Phenylpropanoid Pathway Genes in the Maturing Xylem and Phloem of Picea abies during Latewood Formation

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Journal of Integrative Plant Biology 2011年第10期53卷 783-799页

作者： Giovanni Emiliani Maria Laura Traversi monica Anichini Guido Giachi Alessio Giovannelli Trees and Timber Institute-National Research Council Department of Chemistry "U.Schiff" University of Florence

In temperate regions, latewood is produced when cambial activity declines with the approach of autumnal dormancy. The understanding of the temporal （cambium activity vs dormancy） and spatial （phloem, cambial region, maturing xylem） regulation of key genes involved in the phenylpropanoid pathway during latewood formation represents a crucial step towards providing new insights into the molecular basis of xylogenesis. In this study, the temporal pattern of transcript accumulation of 12 phenylpropanoid genes （PAL1, C4H315, C4H4, 4CL3, 4CL4, HCT1, C3H3, CCoAOMT1, COMT2, COMT5, CCR2） was analyzed in maturing xylem and phloem of Picea abies during latewood formation. Quantitative reverse transcription-polymerase chain reaction analyses revealed a well-defined RNA accumulation pattern of genes involved in the phenylpropanoid pathway during latewood formation. Differences in the RNA accumulation patterns were detected between the different tissue types analyzed. The results obtained here demonstrated that the molecular processes involved in monolignol biosynthesis are not restricted to the cambial activity timeframe but continued after the end of cambium cell proliferation. Furthermore, since it has been shown that lignification of maturing xylem takes place in late autumn, we argue on the basis of our data that phloem could play a key role in the monoliqnol biosynthesis process.

关键词： cambial region gene expression latewood formation xylogenesis.

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