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A novel homozygous missense variant in ARSK causes MPS X,a new subtype of mucopolysaccharidosis

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Genes & Diseases 2024年第3期11卷 87-90页

作者： Miao Sun Cornelia K.Kaminsky Philip Deppe Mai-Brittllse Frederic M.Vaz barbara Plecko Torben Lubke Linda M.Randolph Division of Genomic Medicine Department of Pathology and Laboratory MedicineChildren's Hospital Los Angeles/Keck School of Medicine of USCLos AngelesCA 90027USA Department of Radiology Children's Hospital Los Angeles/Keck School of Medicine of USCLos AngelesCA 90027USA Department of Chemistry BiochemistryBielefeld UniversityBielefeld 33615Germany Amsterdam UMC Location University of Amsterdam Department of Clinical Chemistry and PediatricsLaboratory Genetic Metabolic DiseasesEmma Children's HospitalMeibergdreef 9Amsterdam 1100 DEthe Netherlands Amsterdam Gastroenterology Endocrinology Metabolism Inborn Errors of MetabolismAmsterdam 1105 BKthe Netherlands Core Facility Metabolomics Amsterdam UMC Location University of AmsterdamAmsterdam 1100DDthe Netherlands Department of Pediatrics Division of General PediatricsMedical Universityof GrazGraz8036Austria Division of Medical Genetics Department of PediatricsChildren's Hospital Los Angeles/Keck School of Medicine of USCLos AngelesCA 90027USA

Mucopolysaccharidoses(MPS)are a group of rare inborn errors of metabolism caused by defective lysosomal enzymes which prevent cells from degrading and recycling certain carbohydrates and fats,resulting in the storage of glycosaminoglycans in cells throughout the *** leads to multisystem abnormalities involving bone,connective tissues,brain,blood,spinal cord,skin,and other tissues.

关键词： metabolism doses polys

Modeling of recrystallization behaviour of AA6XXX aluminum alloy profile during extrusion process

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Transactions of Nonferrous Metals Society of China 2024年

作者： Marco Negozio Antonio Segatori Riccardo Pelaccia barbara Reggiani Sara Di Donato Lorenzo Donati DIN Department of Industrial Engineering – University of Bologna University of Parma-Department of Engineering and Architecture， Parco Area delle Scienze Hydro， Innovation and Technology InterMech-MO.RE – University of Modena and Reggio Emilia DISMI Department of Sciences and Methods for Engineering， University of Modena and Reggio Emilia

An innovative approach was introduced for the development of a AA6063 recrystallization model. This method incorporated a regression-based technique for the determination of material constants and introduced novel equations for assessing the grain size evolution. Calibration and validation of this methodology involved a combination of experimentally acquired microstructural data from the extrusion of three different AA6063 profiles and results from the simulation using the Qform Extrusion UK finite element code. The outcomes proved the agreement between experimental findings and numerical prediction of the microstructural evolution. The trend of the grain size variation based on different process parameters has been accurately simulated， both after dynamic and static recrystallization， with an error rate of less than 25% in almost the whole sampling computations.

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A brief history of the Compendium of Physical Activities

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Journal of Sport and Health Science 2024年第1期13卷 3-5页

作者： barbara E.Ainsworth Stephen D.Herrmann David R.Jacobs Jr. Melicia C.Whitt-Glover Catrine Tudor-Locke School of Exercise and Health Shanghai University of SportShanghai 200438China College of Health Solutions Arizona State UniversityPhoenixAZ 85003USA Kansas Center for Metabolism and Obesity Research University of Kansas Medical CenterKansas CityKS 66160USA Division of Epidemiology and Community Health University of MinnesotaMinneapolisMN 55455USA Gramercy Research Group KnightdaleNC 27545USA College of Health and Human Services University of North Carolina at CharlotteCharlotteNC 28223USA

The Compendium of Physical Activities(Compendium)was developed to address consistent assignment of physical activity(PA)intensity values used in PA epidemiology research of the association between PA and health outcomes.1The known protective effects of PA on incident health outcomes traces to the mid-1900s,with over 50 studies examining coronary heart disease(CHD)as the outcome of interest.

关键词： epidemiology Activities protective

DFT Studies of Electronic Properties and Effect of He and Xe Incorporation in Selected Ceramics

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Journal of Modern Physics 2024年第10期15卷 1485-1501页

作者： barbara Szpunar Jayangani I. Ranasinghe Jerzy A. Szpunar Department of Physics and Engineering Physics University of Saskatchewan Saskatoon Canada Department of Mechanical Engineering University of Saskatchewan Saskatoon Canada

The electronic properties of several prospective nuclear fuels are not yet well known. We used Quantum Espresso and EPW codes to evaluate the electron density of states, the electronic heat capacity coefficient, the electron-phonon coupling strength, the number of mobility electrons, and the electronic heat conductivity. The electronic properties for ThN, ThC and UN using a slightly different approach that were previously evaluated are discussed and the results are compared. We confirmed that while the electronic heat capacity coefficient is linearly dependent on the electron density of states at Fermi energy, such a simple relation could not be used to determine the difference in the electronic heat conductivity of investigated materials. The highest heat conductivity was registered in ThN. These metallic fuels also have high U/Th density, therefore are more economical since enrichment is expensive. Furthermore, it is important to examine swelling in these high-density fuels. We evaluated that UN had 42% more U atoms per unit volume than UO2 and a 55% higher volume increase when accommodating one Xe atom in one interstitial of a (2 × 2 × 2) supercell. However, for He, the volume increase was 27% lower in UN. Interestingly, even though the Th atom’s density in ThN and ThC was lower than that of U atoms in the UN compound, a similar trend of volume changes was found. We concluded, therefore, that when we consider swelling, the local structural symmetry (tetrahedral versus octahedral sites) is more important than the density of atoms. The 37 % greater of absolute value of the total energy increase due to incorporation of Xe in ThC versus ThN cannot be explained by the crystal structure since a ThC-Xe supercell has a higher lattice constant than a ThN-Xe corresponding supercell. Such results can only be explained by investigating electronic structure.

关键词： UN ThN ThC Thermal Conductivity Defects

High-quality assembled and annotated genomes of Nicotiana tabacum and Nicotiana benthamiana reveal chromosome evolution and changes in defense arsenals

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Molecular Plant 2024年第3期17卷 423-437页

作者： Jubin Wang Qingling Zhang Jeffrey Tung Xi Zhang Dan Liu Yingtian Deng Zhendong Tian Huilan Chen Taotao Wang Weixiao Yin Bo Lij Zhibing Lai Savithramma P.Dinesh-Kumar barbara Baker Feng Li National Key Laboratory for Germplasm Innovation and Utilization for Fruit and Vegetable Horticultural Crops College of Horticulture and Forestry SciencesHuazhong Agricultural UniversityWuhanHubei 430070China Plant Gene Expression Center Department of Plant and Microbial BiologyUniversity of CaliforniaBerkeleyBerkeleyCA 94706USA College of Plant Science and Technology Huazhong Agricultural UniversityWuhanHubei 430070China College of Life Science and Technology Huazhong Agricultural UniversityWuhanHubei 430070China Department of Plant Biology and The Genome Center College of Biological SciencesUniversity of CalforniaDavisDavisCA 95616USA The Key Laboratory of Horticultural Plant Genetic and Improvement of Jiangxi Province Institute of Biological ResourcesJiangxi Academy of SciencesNanchang 330299China institute of Vegetables and Flowers Jiangxi Academy of Agricultural SciencesNanchang 330200Chin Hubei Hongshan Laboratory WuhanHubei 430070China

Nicotiana tabacum and Nicotiana benthamiana are widely used models in plant biology ***,genomic studies of these species have *** we report the chromosome-level reference genome assemblies for *** and *** with an estimated 99.5%and 99.8%completeness,*** transcription start and termination site sequencing methods were developed and used for accurate gene annotation in *** analyses revealed evidence for the parental origins and chromosome structural changes,leading to hybrid genome formation of each ***,theantiviral silencinggenesRDR1,RDR6,DCL2,DCL3,andAGO2were lost from one or both subgenomes in ***,while both homeologs were kept in ***,the *** genome encodes fewer immune receptors and signaling components than that of *** find-ings uncover possible reasons underlying the hypersusceptible nature of *** developed the user-friendly Nicomics(http:/***/Nicomics/)web server to facilitate better use of Nicotiana genomic resources as well as gene structure and expression analyses.

关键词： Nicotiana tabacum Nicotiana benthamiana genomics evolution disease resistance NLR

Nutrient restriction-activated Fra-2 promotes tumor progression via IGF1R in miR-15a downmodulated pancreatic ductal adenocarcinoma

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Signal Transduction and Targeted Therapy 2024年第3期9卷 1132-1146页

作者： Gian Luca Rampioni Vinciguerra Marina Capece Luca Reggiani Bonetti Giovanni Nigita Federica Calore Sydney Rentsch Paolo Magistri Roberto Ballarin Fabrizio Di Benedetto Rosario Distefano Roberto Cirombella Andrea Vecchione barbara Belletti Gustavo Baldassarre Francesca Lovat Carlo M.Croce Department of Cancer Biology and Genetics and Comprehensive Cancer Center The Ohio State UniversityColumbus 43210 OHUSA Department of Clinical and Molecular Medicine Faculty of Medicine and PsychologySant’Andrea HospitalUniversity of Rome“Sapienza”Rome 00189Italy Department of Diagnostic Clinic and Public Health MedicineUniversity of Modena and Reggio EmiliaModena 41100Italy Hepato-pancreato-biliary Surgery and Liver Transplantation Unit University of Modena and Reggio EmiliaModena 41100Italy Division of Molecular Oncology Centro di Riferimento Oncologico di Aviano(CRO)Istituto di Ricovero e Cura a Carattere Scientifico(IRCCS)National Cancer InstituteAviano 33081Italy

Pancreatic ductal adenocarcinoma(PDAC)is a lethal disease,characterized by an intense desmoplastic reaction that compresses blood vessels and limits nutrient *** aggressiveness largely relies on its extraordinary capability to thrive and progress in a challenging tumor *** of the onco-suppressor miR-15a has been extensively documented in ***,we identified the transcription factor Fos-related antigen-2(Fra-2)as a miR-15a target mediating the adaptive mechanism of PDAC to nutrient *** report that the IGF1 signaling pathway was enhanced in nutrient deprived PDAC cells and that Fra-2 and IGF1R were significantly overexpressed in miR-15a downmodulated PDAC ***,we discovered that miR-15a repressed IGF1R expression via Fra-2 *** miR-15a-low context,IGF1R hyperactivated mTOR,modulated the autophagic flux and sustained PDAC growth in nutrient *** a genetic mouse model,Mir15aKO PDAC showed Fra-2 and Igf1r upregulation and mTOR activation in response to diet ***,nutrient restriction improved the efficacy of IGF1R inhibition in a Fra-2 dependent ***,our results point to a crucial role of Fra-2 in the cellular stress response due to nutrient restriction typical of pancreatic cancer and support IGF1R as a promising and vulnerable target in miR-15a downmodulated PDAC.

关键词： IGF1R Fra miR

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利用先进试验机优化自行车配件制造

现代制造 2024年第5期 40-41页

作者： barbara Schleper 不详

为了测试自行车零件的耐久性,自行车零部件生产厂SRAM公司对其生产的产品进行了疲劳性能测试。测试时的静态和动态性能试验使用的是Zwick-Roell性能试验机。SRAM公司位于德国Schweinfurth市的生产厂为全球自行车骑行爱好者研发了一种很... 详细信息

为了测试自行车零件的耐久性,自行车零部件生产厂SRAM公司对其生产的产品进行了疲劳性能测试。测试时的静态和动态性能试验使用的是Zwick-Roell性能试验机。SRAM公司位于德国Schweinfurth市的生产厂为全球自行车骑行爱好者研发了一种很受欢迎的自行车变速器和车圈。这一研发项目的目标是技术创新和坚固耐用的新产品。

关键词：动态性能试验技术创新试验机研发项目疲劳性能变速器自行车 SRAM

Older Adult Compendium of Physical Activities:Energy costs of human activities in adults aged 60 and older

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Journal of Sport and Health Science 2024年第1期13卷 13-17,F0003页

作者： Erik A.Willis Stephen D.Herrmann Mary Hastert Chelsea L.Kracht Tiago V.Barreira John M.Schuna Jr. Zhenghua Cai Minghui Quan Scott A.Conger Wendy J.Brown barbara E.Ainsworth Center for Health Promotion and Disease Prevention University of North Carolina at Chapel HillChapel HillNC 27599USA Kansas Center for Metabolism and Obesity Research The University of Kansas Medical CenterKansas CityKS 66160USA Division of Physical Activity and Weight Management Department of Internal MedicineUniversity of Kansas Medical CenterKansas CityKS 66160USA Clinical Science Division Pennington Biomedical Research CenterBaton RougeLA 70808USA Exercise Science Department Syracuse UniversitySyracuseNY 13244USA School of Exercise&Sport Science Oregon State UniversityCorvallisOR 97331USA School of Exercise and Health Shanghai University of SportShanghai 200438China Department of Kinesiology Boise State UniversityBoiseID 83725USA School of Human Movement and Nutrition Sciences The University of QueenslandSt.LuciaQLD 4072Australia Faculty of Health Sciences and Medicine Bond UniversityGold CoastQLD 4229Australia College of Health Solutions Arizona State UniversityPhoenixAZ 85003USA

Purpose:To describe the development of a Compendium for estimating the energy costs of activities in adults>60 years(OA Compendium).Methods:Physical activities(PAs)and their metabolic equivalent of task(MET)values were obtained from a systematic search of studies published in 4 sport and exercise databases(PubMed,Embase,SPORTDiscus(EBSCOhost),and Scopus)and a review of articles included in the 2011 Adult Compendium that measured PA in older *** values were computed as the oxygen cost(VO_(2),mL/kg/min)during PA divided by 2.7 m L/kg/min(MET_(60+))to account for the lower resting metabolic rate in older ***:We identified 68 articles and extracted energy expenditure data on 427 *** these,we derived 99 unique Specific Activity codes with corresponding MET_(60+)values for older *** developed a website to present the OA Compendium MET_(60+)values:https://***:The OA Compendium uses data collected from adults>60 years for more accurate estimation of the energy cost of PAs in older *** is an accessible resource that will allow researchers,educators,and practitioners to find MET_(60+)values for older adults for use in PA research and practice.

关键词： Energy expenditure Exercise MET Older adults

Bone-targeting engineered small extracellular vesicles carrying anti-miR-6359-CGGGAGC prevent valproic acid-induced bone loss

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Signal Transduction and Targeted Therapy 2024年第2期9卷 751-765页

作者： Xudong Xie Peng Cheng Liangcong Hu Wu Zhou Detai Zhang Samuel Knoedler Guodong Liu Yuan Xiong Hang Xue Yiqiang Hu barbara Kern Doha Obed Adriana CPanayi Lang Chen Chenchen Yan Ze Lin Guandong Dai Bobin Mi Yingze Zhang Guohui Liu Department of Orthopedics Union HospitalTongji Medical CollegeHuazhong University of Science and TechnologyWuhan 430022China Hubei Province Key Laboratory of Oral and Maxillofacial Development and Regeneration Wuhan 430022China Department of Clinical Laboratory Union HospitalTongji Medical CollegeHuazhong University of Science and TechnologyWuhanHubei 430022P.R.China Division of Plastic Surgery Brigham and Women’s HospitalHarvard Medical SchoolBostonMA 02152USA Department of Plastic Surgery and Hand Surgery Klinikum Rechts der IsarTechnical University of MunichMunichGermany Medical Center of Trauma and War Injuries Daping HospitalArmy Medical UniversityChonqing 400042China Department of Plastic Surgery Campus CharitéMitte|Campus Virchow-KlinikumCharité-Universitätsmedizin Berlincorporate member of Freie Universität BerlinHumboldt-Universität zu Berlin and Berlin Institute of HealthBerlinGermany Berlin Institute of Health Charité-Universitätsmedizin BerlinBerlinGermany Department of Plastic AestheticHand and Reconstructive SurgeryHannover Medical SchoolHannoverGermany Department of Hand Plastic and Reconstructive SurgeryMicrosurgeryBurn CenterBG Trauma Center LudwigshafenUniversity of HeidelbergLudwig-Guttmann-Strasse 1367071 Ludwigshafen/RhineGermany Pingshan District People’s Hospital of Shenzhen Pingshan General Hospital of Southern Medical UniversityShenzhenGuangdong 518118China Department of Orthopaedic Surgery The Third Hospital of Hebei Medical UniversityNO.139 Ziqiang RoadShijiazhuang 050051China

The clinical role and underlying mechanisms of valproic acid(VPA)on bone homeostasis remain ***,we confirmed that VPA treatment was associated with decreased bone mass and bone mineral density(BMD)in both patients and *** effect was attributed to VPA-induced elevation in osteoclast formation and *** RNA-sequencing,we observed a significant rise in precursor miR-6359 expression in VPA-treated osteoclast precursors in vitro,and further,a marked upregulation of mature miR-6359(miR-6359)in vivo was demonstrated using quantitative real-time PCR(qRT-PCR)and miR-6359 fluorescent in situ hybridization(miR-6359-FISH).Specifically,the miR-6359 was predominantly increased in osteoclast precursors and macrophages but not in neutrophils,T lymphocytes,monocytes and bone marrow-derived mesenchymal stem cells(BMSCs)following VPA stimulation,which influenced osteoclast differentiation and bone-resorptive ***,VPA-induced miR-6359 enrichment in osteoclast precursors enhanced reactive oxygen species(ROS)production by silencing the SIRT3 protein expression,followed by activation of the MAPK signaling pathway,which enhanced osteoclast formation and activity,thereby accelerating bone ***,there are no medications that can effectively treat VPA-induced bone ***,we constructed engineered small extracellular vesicles(E-sEVs)targeting osteoclast precursors in bone and naturally carrying anti-miR-6359 by introducing of EXOmotif(CGGGAGC)in the 3’-end of the anti-miR-6359 *** confirmed that the E-sEVs exhibited decent bone/osteoclast precursor targeting and exerted protective therapeutic effects on VPA-induced bone loss,but not on ovariectomy(OVX)and glucocorticoid-induced osteoporotic models,deepening our understanding of the underlying mechanism and treatment strategies for VPA-induced bone loss.

关键词： miR protective stimulation