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The Study of Carbamoyl Phosphate Synthetase 1 Deficiency Sheds Light on the Mechanism for Switching On/Off the Urea Cycle

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Journal of genetics and genomics 2015年第5期42卷 249-260页

作者： Carmen Díez-Fernández jos. gallego Johannes H?berle Javier Cervera vicente Rubio Institute for Biomedicine of Valencia of the Spanish Research Council Medical School of the Catholic University of Valencia University Children's Hospital Zurich and Children's Research Center Centre for Biomedical Networking Research on Rare Diseases of the Carlos Ⅲ Health Institute

Carbamoyl phosphate synthetase i （CPS1） deficiency （CPS1D） is an inborn error of the urea cycle having autosomal （2q34） recessive inheritance that can cause hyperammonemia and neonatal death or mental retardation. We analyzed the effects on CPS1 activity, kinetic parameters and enzyme stability of missense mutations reported in patients with CPS1 deficiency that map in the 20-kDa C-terminal domain of the enzyme. This domain turns on or off the enzyme depending on whether the essential allosteric activator of CPS 1, N-acetyl- L-glutamate （NAg）, is bound or is not bound to it. To carry out the present studies, we exploited a novel system that allows the expression in vitro and the purification of human CPS1, thus permitting site-directed mutagenesis. These studies have clarified disease causation by individual mutations, identifying functionally important residues, and revealing that a number of mutations decrease the affinity of the enzyme for NAg. Patients with NAg affinity-decreasing mutations might benefit from NAg site saturation therapy with N-carbamyl-L- glutamate （a registered drug, the analog of NAg）. Our results, together with additional present and prior site-directed mutagenesis data for other residues mapping in this domain, suggest an NAg-triggered conformational change in the 134-~4 loop of the C-terminal domain of this enzyme. This change might be an early event in the NAg activation process. Molecular dynamics simulations that were restrained according to the observed effects of the mutations are consistent with this hypothesis, providing further backing for this structurally plausible signaling mechanism by which NAg could trigger urea cycle activation via CPS1.

关键词： Urea cycle diseases Inborn errors Hyperammonemia Site-directed mutagenesis Restrained molecular dynamics Allosteric regulation Carbamoyl phosphate synthetase 1 Enzyme

Do non-target species visit feeders and water troughs targeting small game?A study from farmland Spain using camera-trapping

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Integrative Zoology 2021年第2期16卷 226-239页

作者： jos.A.ARMENTEROS Jesús CARO Carlos SÁNCHEZ-gARCÍA Beatriz ARROYO jos.A.PÉREZ vicente RgAUDIOSO Emilio J.TIZADO Department of Animal Production Research Group on Game Species Breeding and ManagementUniversity of LeónLeónSpain Instituto de Investigación en Recursos Cinegéticos(IREC CSIC-UCLM-JCCM)Ciudad RealSpain Fundación Artemisan Ciudad RealSpain Department of Biodiversity and Environmental Management University of LeónPonferradaSpain

Provision of food and water is a widespread tool implemented around the world for the benefit of game and other wildlife,but factors affecting the use of food and water by non-target species are poorly *** evaluated visits to feeders and water troughs by non-game species using camera-traps in two separate areas of *** and water troughs were either“protected”(when surrounded by more than 50%of shrubs/forest)or“open”(in the opposite case).A total of 18948 photos from 5344 camera-trapping days depicted animals,and 75 species were *** and water troughs were visited by target species(partridges and lagomorphs,55.3%of visits)and non-target species(44.7%of visits).Among the latter,corvids were themost common(46.1%of visits),followed by rodents(26.8%),other birds(23.6%,mainly passerines),columbids(1.9%),and other species at minor *** highest proportion of visiting days to feeders and water troughs was from corvids(0.173)followed by otherbirds(0.109)and rodents(0.083);the lowest proportion was recorded for columbids(0.016).Use intensity and visit frequency of water troughs tripled that recorded in feeders,and visits to open feeders/troughs were approximately twice those to protected *** summary:feeders and water troughs targeting small game species are also used regularly by non-target ones;they should be set close to cover to optimize their use by non-target species that are not competitors of target species(though corvids may visit them);water availability should be prioritized where drought periods are expected.

关键词： camera-trapping feeding game species management watering wildlife

Plyometric exercise and bone health in children and adolescents: a systematic review

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World Journal of Pediatrics 2017年第2期13卷 112-121页

作者： Alejandro gómez-Bruton (A)ngel Matute-Llorente Alejandro gonzález-Agüero jos.A.Casajús germán vicente-Rodríguez growth ExerciseNutrition and Development(GENUD)Research GroupUniversity of ZaragozaZaragozaSpain Faculty of Health and Sport Sciences(FCSD) Department of Physiatry and NursingUniversity of ZaragozaRonda Misericordia 522001-HuescaSpain growth ExerciseNutrition and Development(GENUD)Research GroupUniversity of ZaragozaZaragozaSpain

Background:Many jumping interventions have been performed in children and adolescents in order to improve bone-related variables and thus,ensure a healthy bone development during these periods and later in *** systematic review aims to summarize and update present knowledge regarding the effects that jumping interventions may have on bone mass,structure and metabolism in order to ascertain the efficacy and durability (duration of the effects caused by the intervention) of the *** sources:Identification of studies was performed by searching in the database MEDLINE/PubMed and *** studies were identified by contacting clinical experts and searching bibliographies and *** terms included 'bone and bones','jump*','weight-bearing','resistance training' and 'school intervention'.The search was conducted up to October *** studies that had performed a specific jumping intervention in under 18-year olds and had measured bone mass were *** extraction of articles was done by 2 authors using predefined data ***:A total of 26 studies were included in this ***-four studies found positive results as subjects included in the intervention groups showed higher bone mineral density,bone mineral content and bone structure improvements than *** two studies found no effects on bone mass after a 10-week and 9-month ***,those studies that evaluated the durability of the effects found that some of the increases in the intervention groups were maintained after several ***:Jumping interventions during childhood and adolescence improve bone mineral content,density and structural properties without side *** type of interventions should be therefore implemented when possible in order to increase bone mass in early stages of life,which may have a direct preventive effect on bone diseases like osteoporosis later in fife.

关键词： adolescents bone mineral density intervention jumping osteoporosis

Evolution of wheat architecture,physiology,and metabolism during domestication and further cultivation:Lessons for crop improvement

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The Crop Journal 2023年第4期11卷 1080-1096页

作者： Arturs Katamadze Omar Vergara-Díaz Estefanía Uberegui Ander Yoldi-Achalandabaso jos. Luis Araus Rubén vicente Plant Ecophysiology and Metabolism group Instituto de Tecnologia Química e Biológica António XavierUniversidade Nova de LisboaP-2780 OeirasPortugal genetics and genomics of Plant Complex Traits Instituto de Tecnologia Química e Biológica António XavierUniversidade Nova de LisboaP-2780 OeirasPortugal FisioClimaCO_(2) group Department of Plant Biology and EcologyFaculty of Science and TechnologyUniversity of the Basque CountryE-48940 LeioaSpain Integrative Crop Ecophysiology group Section of Plant PhysiologyFaculty of BiologyUniversity of BarcelonaE-08028 Barcelona&AGROTECNIO-CERCA CenterE-25198 LleidaSpain

In recent decades,genetic advances in yield improvement in the major cereal crops,including wheat,has stagnated or proceeded at a slower rate than is required to meet future global food demand,particularly in the face of climate *** reverse this situation,and in view of the future climate scenario,there is a need to increase the genetic diversity of wheat to increase its productivity,quality,stability,and adaptation to local *** abundant genetic resources and literature are a basis for wheat ***,many species,such as wild relatives,landraces,and old cultivars have not been studied beyond their agronomic characteristics,highlighting the lack of understanding of the physiological and metabolic processes(and their integration) associated with higher productivity and resilience in limiting *** studies using wheat ancestors and modern cultivars may identify novel traits that have not previously been considered,or have been underestimated,during domestication and breeding,but that may contribute to future food *** review describes existing wheat genetic diversity and changes that occurred during domestication and breeding,and considers whether mining natural variation among wheat ancestors offers an opportunity to enhance wheat agronomic performance,spike architecture,canopy-and organ-level photosynthetic capacity,and responses to abiotic stress,as well as to develop new wheat hybrids.

关键词： Breeding Landraces Photosynthesis Wild relatives Yield

Detection and Delineating of Hydrocarbon Contaminants by Using Time and Frequency Analysis of ground Penetrating Radar

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Journal of geoscience and Environment Protection 2021年第12期9卷 35-56页

作者： jos. vicente Fuente Research & Develop Department Geozone Asesores SL Bétera Spain

This paper provides the results of using ground penetrating radar (gPR) method to detect hydrocarbon products (diesel and gasoline) in a controlled lab test. The work addresses the environmental problem generated by the uncontrolled leakage of hydrocarbon products and the subsequent contamination of plumes in the subsoil. Most of the research proposes the geophysical techniques to evaluate the plumes but some controversial were discussed on how it affected the electrical and dielectric response depending on the excitation of the non-invasive method. The present work focuses on a comparative analysis of some signal attributes of the GPR traces to determine under what premises the detection is properly done. These signal attributes were from the time and frequency domain as attenuation coefficient, instantaneous amplitude and frequency have been considered to analyze three different soil samples. The laboratory tests consist of buried liquid (total hydrocarbon of petroleum, so called TPH) bags in the soil sample boxes reveal the range of target detection and consistency of data on the controlled test regarding the dielectric soil characterization and the delimiting position and depth. Instantaneous amplitude and time-frequency shift are revealed as promising signal attributes to accurate detection of the TPH presence. Numerical simulation data were a

关键词： Time-Frequency Analysis Instantaneous Amplitude gPR Survey Hydrocar-bon Products

Hepatogenic differentiation of human mesenchymal stem cells from adipose tissue in comparison with bone marrow mesenchymal stem cells

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World Journal of gastroenterology 2006年第36期12卷 5834-5845页

作者： Raquel Taléns-Visconti Ana Bonora Ramiro Jover vicente Mirabet Francisco Carbonell jos. vicente Castell María jos. gómez-Lechón Unidad de Hepatología Experimental Centro de Investigación Hospital La Fe Valencia Spain Departamento de Bioquímica y Biología Molecular Universidad de Valencia Valencia Spain. Unidad de Hepatología Experimental Centro de Investigación Hospital La Fe Valencia Spain Centro de Transfusión de la Comunidad Valenciana Generalidad ValencianaValencia Spain Unidad de Hepatología Experimental Centro de Investigación Hospital La Fe Valencia.Spain. Departamento de Bioquímica y Biología Molecular Universidad de Valencia Valencia Spain Unidad de Hepatología Experi-mental Centro de Investigación Hospital La Fe Valencia Spain

AIM： To investigate and compare the hepatogenic transdifferentiation of adipose tissue-derived stem cells （ADSC） and bone marrow-derived mesenchymal stem cells （BMSC） in vitro. Transdifferentiation of BMSC into hepatic cells in vivo has been described. Adipose tissue represents an accessible source of ADSC, with similar characteristics to BMSC. METHODS： BMSCs were obtained from patients undergoing total hip arthroplasty and ADSC from human adipose tissue obtained from lipectomy. Cells were grown in medium containing 15% human serum. Cultures were serum deprived for 2 d before cultivating under similar pro-hepatogenic conditions to those of liver development using a 2-step protocol with sequential addition of growth factors, cytokines and hormones. Hepatic differentiation was RT-PCR-assessed and liver-marker genes were immunohistochemically ***： BMSC and ADSC exhibited a fibroblastic morphology that changed to a polygonal shape when cells differentiated. Expression of stem cell marker Thyl decreased in differentiated ADSC and BMSC. However, the expression of the hepatic markers, albumin and CYPs increased to a similar extent in differentiated BMSC and ADSC. Hepatic gene activation could be attributed to increased liver-enriched transcription factors （C/EBPβ and HNF4α）, as demonstrated by adenoviral expression ***： Mesenchymal stem cells can be induced to hepatogenic transdifferentiation in vitro. ADSCs have a similar hepatogenic differentiation potential to BMSC, but a longer culture period and higher proliferation capacity. Therefore, adipose tissue may be an ideal source of large amounts of autologous stem cells, and may become an alternative for hepatocyte regeneration, liver cell transplantation or preclinical drug testing.

关键词： Mesenchymal stem cells Bone marrow Adipose tissue Transdifferentiation Hepatic lineage Liver cell transplantation.

Clinicopathological alterations in wild mammals from the reservoir system of Trypanosoma cruzi:a scoping review

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Animal Diseases 2024年第1期4卷 48-58页

作者： Ricardo Sanchez Pérez jos.Manuel Aranda-Coello jos.Antonio De Fuentes vicente Oscar Rico Chavez Facultad de Estudios Superiores Cuautitlan Universidad Nacional Autonoma deMexicoCuautitlan lzcalliEstadodeMexicoMexico El Colegio de la Frontera Sur-Unidad Campeche CampecheCampecheMexico Instituto de Ciencias Biologicas-Universidad de Ciencias y Artes de Chiapas Tuxtla GutierrezChiapasMexico Facultad de Medicina Veterinaria y Zootecnia-Universidad Nacional Autonoma deMexico Ciudad deMexicoMexico

Trypanosoma cruzi is the etiologic agent of Chagas *** flagellated protozoan is transmitted to humans as well as different species of domestic and wild animals via vectors from the Reduviidae family(known as"kissing bugs").Despite the fact that hundreds of species of wild mammals are part of the reservoir system,the morphologi-cal changes and clinical manifestations resulting from the pathogenesis of the infection have been largely *** aim of this review is to systematically compile the available information regarding clinicopathological altera-tions in wild mammals due to natural infection by *** was obtained from six online bibliographic data search platforms,resulting in the identification of 29 publications that met the inclusion *** was the most common clinical manifestation,cardiac damage was the main finding at necropsy,and lymphoplas-macytic inflammation was the most frequent microscopic ***,regardless of its role as a reservoir,*** has the potential to affect the health status of wild mammals,a situation that highlights the need for further research to analyze,measure,and compare its effects at both the individual and population levels.

关键词： Trypanosoma cruzi Chagas disease American trypanosomiasis Clinicopathological alterations Natural infection Wild mammals

MMALE—A Methodology for Malware Analysis in Linux Environments

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Computers, Materials & Continua 2021年第5期67卷 1447-1469页

作者： jos.Javier de vicente Mohino Javier Bermejo Higuera Juan Ramón Bermejo Higuera Juan Antonio Sicilia Montalvo Manuel Sánchez Rubio jos.Javier Martínez Herraiz Escuela Superior de Ingeniería y Tecnología Universidad Internacional de La RiojaLogroño26006La RiojaSpain Departamento de Ciencias de la Computación de la Escuela Politécnica Superior Universidad de Alcaláde HenaresAlcaláde HenaresMadridSpain

In a computer environment,an operating system is prone to malware,and even the Linux operating system is not an *** recent years,malware has evolved,and attackers have become more qualified compared to a few years ***,Linux-based systems have become more attractive to cybercriminals because of the increasing use of the Linux operating system in web servers and Internet of Things(IoT)*** is the most employed OS,so most of the research efforts have been focused on its malware protection rather than on other operating *** a result,hundreds of research articles,documents,and methodologies dedicated to malware analysis have been ***,there has not been much literature concerning Linux security and protection from *** address all these new challenges,it is necessary to develop a methodology that can standardize the required steps to perform the malware analysis in depth.A systematic analysis process makes the difference between good and ordinary malware ***,a deep malware comprehension can yield a faster and much more efficient malware *** order to address all mentioned challenges,this article proposed a methodology for malware analysis in the Linux operating system,which is a traditionally overlooked field compared to the other operating *** proposed methodology is tested by a specific Linux malware,and the obtained test results have high effectiveness in malware detection.

关键词： Malware analysis methodology analysis Linux malware IoT malware

Coaches’ Speech in Regard to Score and Match Phase

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Journal of Sports Science 2021年第1期9卷 17-27页

作者： vicente Calpe-gómez jos.Francisco guzmán Department of Physical and Sport Education Faculty of Physical Activity and Sports SciencesUniversity of ValenciaSpain

The aim of this study was to analyse the rate and content of 10,430 messages,sent by 21 handball coaches in 21 matches,in relation to score and match *** situations were defined as regards score:large victory,tight game,and large defeat;and four phases for match phase:part 1 and 2 of first half,and part 1 and 2 of second *** rates were calculated by dividing the number of messages sent at each level of score and match phase by the elapsed *** calculate the content,messages were coded using Coach Analysis and Intervention System and percentages were calculated according to score and match ***-square analysis showed statistically significant differences for rate of messages by score(χ^(2)=211.06,df=2,siggge of content by score(χ^(2)=444.011,df=42,siggguidelines to increase the quality of coaches’speech.

关键词： Coaching situational variables communicational skills observational methodology