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Neurologic orphan diseases:Emerging innovations and role for genetic treatments

World Journal of Experimental medicine 2023年第4期13卷 59-74页

作者： Ivelina P Kioutchoukova Devon T Foster Rajvi N Thakkar marco A Foreman brandon J Burgess Rebecca m Toms Eduardo E molina Valero brandon Lucke-Wold College of medicine University of FloridaGainesvilleFL 32611United States Florida International University Herbert Wertheim College of medicine Florida International University Herbert Wertheim College of MedicineMiamiFL 33199United States Department of Neurosurgery University of FloridaGainesvilleFL 32611United States

Orphan diseases are rare diseases that affect less than 200000 individuals within the United *** orphan diseases are of neurologic and genetic *** the current advances in technology,more funding has been devoted to developing therapeutic agents for patients with these *** our review,we highlight emerging options for patients with neurologic orphan diseases,specifically including diseases resulting in muscular deterioration,epilepsy,seizures,neurodegenerative movement disorders,inhibited cognitive development,neuron deterioration,and *** extensive literature review,gene therapy offers a promising route for the treatment of neurologic orphan *** use of clustered regularly interspaced palindromic repeats/Cas9 has demonstrated positive results in experiments investigating its role in several ***,the use of adeno-associated viral vectors has shown improvement in survival,motor function,and developmental milestones,while also demonstrating reversal of sensory ataxia and cardiomyopathy in Friedreich ataxia *** oligonucleotides have also been used in some neurologic orphan diseases with positive *** target of rapamycin inhibitors are currently being investigated and have reduced abnormal cell growth,proliferation,and *** innovations and the role of genetic treatments open a new window of opportunity for the treatment of neurologic orphan diseases.

关键词： Neurologic orphan diseases Gene therapy Clustered regularly interspaced palindromic repeats/Cas9 Antisense oligonucleotides Adeno-associated virus mTOR inhibitors

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Clinical classification of symptomatic heterotopic pancreas of the stomach and duodenum:A case series and systematic literature review

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World Journal of Gastroenterology 2022年第14期28卷 1455-1478页

作者： michael T Le Compte brandon mason Keenan J Robbins motoyo Yano Deyali Chatterjee Ryan C Fields Steven m Strasberg William G Hawkins Department of Surgical Oncology University of North CarolinaRaleighNC 27608United States Department of Radiology Stillwater Medical CenterStillwaterOK 74074United States Department of General Surgery Washington University St.LouisSt.LouisMO 63110-8109United States Department of Radiology Mayo ClinicPhoenixAZ 8505United States Department of Pathology and Immunology MD Anderson Cancer CenterHoustonTX 77030United States Department of Surgical Oncology Washington University School of MedicineSt.LouisMO 63110-8109United States Section of Hepatobiliary-Pancreatic and GI Surgery Washington University St.LouisSt.LouisMO 63110United States

BACKGROUND Heterotopic pancreas(HP)is an aberrant anatomic malformation that occurs most commonly in the upper gastrointestinal *** the majority of heterotopic pancreatic lesions are asymptomatic,many manifest severe clinical symptoms which require surgical or endoscopic *** of the clinical manifestations and symptoms of HP is limited due to the lack of large volume studies in the *** purpose of this study is to review symptomatic cases at a single center and compare these to a systematic review of the literature in order to characterize common clinical manifestations and treatment of this *** To classify the common clinical manifestations of heterotopic *** A retrospective review was conducted of pathologic samples containing heterotopic pancreas from *** was limited to HP of the upper gastrointestinal tract due to the frequency of presentation in this *** patients were identified from review of the medical records and clinical symptoms were *** were compared to a systematic review of the literature utilizing Pub med and Embase searches for papers pertaining to heterotopic *** describing symptomatic presentation of HP were selected for *** including demographics,symptoms,presentation and treatment were compiled and *** Twenty-nine patient were identified with HP at a single center,with six of these identified has having clinical *** manifestations included,gastrointestinal bleeding,gastric ulceration with/without perforation,pancreatitis,and gastric outlet *** review of the literature yielded 232 publications detailing symptomatic cases with only 20 studies describing ten or more *** and multi-patient studies were combined to form a cohort of 934 symptomatic *** majority of patients presented with abdominal pain(67%)combined with one of the following clinical catego

关键词： Heterotopic pancreas Ectopic pancreas Aberrant pancreas Pancreatic rest Groove pancreatitis

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Strategies to enhance monoclonal antibody uptake and distribution in solid tumors

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Cancer Biology & medicine 2021年第3期18卷 649-664页

作者： brandon m.Bordeau Joseph P.Balthasar Department of Pharmaceutical Science University at BuffaloBuffaloNY 14214USA

Despite the significant resources dedicated to the development of monoclonal antibody(m Ab)therapies for solid tumors,the clinical success,thus far,has been *** efficacy of m Ab in solid tumors likely relates to unique aspects of tumor *** tumors have an aberrant vasculature and a dense extracellular matrix that slow both the convective and diffusive transport of m Abs into and within *** m Abs that are directed against cellular antigens,high antigen expression and rapid antigen turnover can result in perivascular cells binding to and eliminating a significant amount of extravasated m Ab,limiting m Ab distribution to portions of the tumor that are distant from functional *** preclinical investigations have reported strategies to improve m Ab uptake and distribution;however,to our knowledge,none have translated into the ***,we provide an overview of several barriers in solid tumors that limit m Ab uptake and distribution and discuss approaches that have been utilized to overcome these barriers in preclinical studies.

关键词： Solid tumors antibody uptake and distribution monoclonal antibody antibody-drug conjugate

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Computational discovery of ultra-strong, stable, and lightweight refractory multi-principal element alloys. Part II: comprehensive ternary design and validation

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npj Computational materials 2023年第1期9卷 1469-1481页

作者： Kate L.m.Elder Joel Berry Aurélien Perron brandon Bocklund Jibril Shittu Connor J.Rietema Hunter B.Henderson Scott K.mcCall Joseph T.mcKeown materials Science Division Lawrence Livermore National LaboratoryLivermoreCA 94550USA.✉

Here the discovery of refractory multi-principal element alloys(mPEAs)with high-temperature strength and stability is pursued within a constrained and application-relevant design space.A comprehensive approach is developed and applied to explore all 165 ternary systems in the Al-Ce-Fe-Hf-mo-Nb-Ta-Ti-V-W-Zr family.A subset of ternary systems that contain large areas in composition–temperature space with high strength and robust BCC phase stability is *** sets of high-performing alloys are identified,each set optimized for one combination of phase constraint,optimization target,and temperature *** mechanical tests support the viability of the *** work highlights the importance of considering phase stability,exploring non-equiatomic regions of composition space,and applying application-relevant *** I and II provide three down-selection techniques for identifying high-performing BCC refractory mPEAs,design guidelines,and many candidates predicted to have BCC phase stability and strengths 2–3 times higher than any reported to date.

关键词： alloys element ternary

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CNN-BiLSTm-Attention model in Forecasting Wave Height over South-East China Seas

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Computers, materials & Continua 2022年第10期73卷 2151-2168页

作者： Lina Wang Xilin Deng Peng Ge Changming Dong brandon J.Bethel Leqing Yang Jinyue Xia School of Artificial Intelligence(School of Future Technology) Nanjing University of Information Science and Technology Nanjing210044China Southern marine Science and Engineering Guangdong Laboratory(Zhuhai) Zhuhai519080China School of marine Sciences Nanjing University of Information Science and TechnologyNanjing210044China International Business machines Corporation(IBm) New York10504USA

Though numerical wave models have been applied widely to significant wave height prediction,they consume massive computing memory and their accuracy needs to be further *** this paper,a two-dimensional(2D)significant wave height(SWH)prediction model is established for the South and East China *** proposed model is trained by Wave Watch III(WW3)reanalysis data based on a convolutional neural network,the bidirectional long short-term memory and the attention mechanism(CNNBiLSTm-Attention).It adopts the convolutional neural network to extract spatial features of original wave height to reduce the redundant information input into the BiLSTm ***,the BiLSTm model is applied to fully extract the features of the associated information of time series ***,the attention mechanism is used to assign probability weight to the output information of the BiLSTm layer units,and finally,a training model is *** to 24-h prediction experiments are conducted under normal and extreme conditions,*** the normal wave condition,for 3-,6-,12-and 24-h forecasting,the mean values of the correlation coefficients on the test set are 0.996,0.991,0.980,and 0.945,*** corresponding mean values of the root mean square errors are measured at 0.063 m,0.105 m,0.172 m,and 0.281 m,*** the typhoon-forced extreme condition,the model based on CNN-BiLSTm-Attention is trained by typhooninduced SWH extracted from the WW3 reanalysis *** 3-,6-,12-and 24-h forecasting,the mean values of correlation coefficients on the test set are respectively 0.993,0.983,0.958,and 0.921,and the averaged root mean square errors are 0.159 m,0.257 m,0.437 m,and 0.555 m,*** model performs better than that trained by all the WW3 reanalysis *** result suggests that the proposed algorithm can be applied to the 2D wave forecast with higher accuracy and efficiency.

关键词： Conv2D CNN-BiLSTm-Attention wave forecasting significant wave height typhoon

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Experimental evidence that soil heterogeneity enhances plant diversity during community assembly

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Journal of Plant Ecology 2014年第5期7卷 461-469页

作者： brandon m.Williams Gregory R.Houseman Department of Biological Sciences Wichita State University1845 FairmountWichitaKS 67260USA

Aims Environmental heterogeneity is a primary mechanism explain-ing species coexistence and extant patterns of *** strong theoretical support and ample observational evidence,few experimental studies in plant communities have been able to demonstrate a causal link between environmental heterogene-ity and plant *** lack of experimental evidence sug-gests that either fine-scale heterogeneity has weak effects on plant diversity or previous experiments have been unable to effectively manipulate ***,we utilize a unique soil manipu-lation to test whether fine-scale soil heterogeneity will increase plant richness through species sorting among experimental patch *** This experiment was conducted in the tallgrass prairie region of south-central Kansas,*** utilized the inherent variation found in the vertical soil profile,which varied in both biotic and abiotic characteristics,and redistributed these strata into either homoge-neous or heterogeneous spatial arrangements in 2.4×2.4 m *** the soil manipulation,34 native prairie species were sown into all *** conducted annual censuses at peak biomass to quantify species composition and plant density by species within the experimental *** Findings After 2 years,species richness was significantly higher in heteroge-neous relative to homogeneous plots and this pattern was independ-ent of total plant *** the heterogeneous plots,13 species had higher establishment in a specific patch type representing one of the three soil ***,no species had greater estab-lishment in the mixed stratum,which comprised the homogene-ous plots,relative to the heterogeneous *** species sorting patterns suggest that fine-scale heterogeneity creates opportunities for plant establishment due to niche differences,which translates into increased plant diversity at the plot *** richness was more strongly related to plant density among patch

关键词： environmental heterogeneity species richness species sorting community assembly niche theory neutral theory

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US-China Relations:Nationalism,the Trade War,and COVID-19

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Fudan Journal of the Humanities and Social Sciences 2021年第1期14卷 23-40页

作者： brandon m.Boylan Jerry mcBeath Bo Wang University of Alaska Fairbanks(UAF) 603 C/D Gruening Building1747 South Chandalar DriveFairbanksAK 99775USA Emeritus of Political Science University of Alaska Fairbanks(UAF)1777 Red Fox DriveFairbanksAK 99709USA School of International Relations China University of International Business and Economics(UIBE)Mail Box 100No.10 Huixin East StreetChangyang DistrictBeijing 100029People’s Republic of China

The trade war between the USA and China has shocked many across the world.A disruption to the interdependence of the two largest economies seemed ***,in an effort to thwart China’s economic practices and boost the US economy,President Trump’s administration levied tariffs on Chinese imports shortly after taking office,moving US foreign economic policy from liberalism,practiced for decades,to *** has retaliated,and the trade war continues *** conceptual insights from the nationalism literature,we explore the nationalist roots of the trade war from both the US and Chinese *** the USA,the Trump administration’s plan to achieve energy autonomy,decrease reliance on foreign resources,and reinvigorate the manufacturing sector has led to protectionist policies,the othering of China,and hence the trade *** reluctant to enter the conflict,China has rebuffed the USA,resisting and counterattacking US actions,owing to a long-felt sense of persecution in the global space and an eagerness to participate fully,and lead in some issue areas,in international *** conflict continues into the COVID-19 era,marked by US scapegoating of China and hits to economic *** both sides are convinced they have achieved their goals,or the USA undergoes an administration change,the conflict will likely continue.

关键词： US-China relations Nationalism International trade Natural resources Energy Political economy COVID-19

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Structural variation and parallel evolution of apomixis in citrus during domestication and diversification

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National Science Review 2022年第10期9卷 82-97页

作者： Nan Wang Xietian Song Junli Ye Siqi Zhang Zhen Cao Chenqiao Zhu Jianbing Hu Yin Zhou Yue Huang Shuo Cao Zhongjie Liu Xiaomeng Wu Lijun Chai Wenwu Guo Qiang Xu brandon S.Gaut Anna m.G.Koltunow Yongfeng Zhou Xiuxin Deng Key Laboratory of Horticultural Plant Biology (ministry of Education) Huazhong Agricultural University Shenzhen Branch Guangdong Laboratory of Lingnan Modern Agriculture Genome Analysis Laboratory of the Ministry of Agriculture and Rural Affairs Agricultural Genomics Institute at Shenzhen Chinese Academy of Agricultural Sciences Department of Ecology and Evolutionary Biology University of California Irvine Queensland Alliance for Agriculture and Food Innovation University of Queensland

Apomixis,or asexual seed formation,is prevalent in Citrinae via a mechanism termed nucellar or adventitious ***,multiple embryos of a maternal genotype form directly from nucellar cells in the ovule and can outcompete the developing zygotic embryo as they utilize the sexually derived endosperm for *** nucellar embryony enables the propagation of clonal plants of maternal genetic constitution,it is also a barrier to effective breeding through *** address the genetics and evolution of apomixis in Citrinae,a chromosome-level genome of the Hongkong kumquat(Fortunella hindsii) was assembled following a genome-wide variation map including structural variants(SVs) based on234 Citrinae *** map revealed that hybrid citrus cultivars shelter genome-wide deleterious mutations and SVs into heterozygous states free from recessive selection,which may explain the capability of nucellar embryony in most cultivars during Citrinae *** reve aled that parallel evolution may explain the repeated origin of apomixis in different genera of *** Fortu nella,we found that apomixis of some varieties originated via *** apomictic Fortunella,the locus associated with apomixis contains the FhRWP gene,encoding an RWP-RK domain-containing protein previously shown to be required for nucellar embryogenesis in *** found the heterozygous SV in the FhRWP and CitRWP promoters from apomictic Citrus and Fortunella,due to either two or three miniature inverted transposon element(mITE) insertions.A transcription factor,FhARID,encoding an AT-rich interaction domain-containing protein binds to the mITEs in the promoter of apomictic varieties,which facilitates induction of nucellar *** study provides evolutionary genomic and molecular insights into apomixis in Citrinae and has potential ramifications for citrus breeding.

关键词： genomics evolution population genetics apomixis

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High-Content Screening and Analysis of Stem Cell-Derived Neural Interfaces Using a Combinatorial Nanotechnology and machine Learning Approach

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研究(英文) 2023年第1期2022卷 153-167页

作者： Letao Yang Brian m.Conley Jinho Yoon Christopher Rathnam Thanapat Pongkulapa brandon Conklin Yannan Hou Ki-Bum Lee Department of Chemistry and Chemical Biology Rutgers UniversityThe State University of New JerseyPiscatawayNJ 08854USA

A systematic investigation of stem cell-derived neural interfaces can facilitate the discovery of the molecular mechanisms behind cell behavior in neurological disorders and accelerate the development of stem cell-based ***,high-throughput investigation of the cell-type-specific biophysical cues associated with stem cell-derived neural interfaces continues to be a significant obstacle to *** this end,we developed a combinatorial nanoarray-based method for high-throughput investigation of neural interface micro-/nanostructures(physical cues comprising geometrical,topographical,and mechanical aspects)and the effects of these complex physical cues on stem cell fate ***,by applying a machine learning(mL)-based analytical approach to a large number of stem cell-derived neural interfaces,we comprehensively mapped stem cell adhesion,differentiation,and proliferation,which allowed for the cell-type-specific design of biomaterials for neural interfacing,including both adult and human-induced pluripotent stem cells(hiPSCs)with varying genetic *** short,we successfully demonstrated how an innovative combinatorial nanoarray and mL-based platform technology can aid with the rational design of stem cell-derived neural interfaces,potentially facilitating precision,and personalized tissue engineering applications.

关键词： overcome neural rational

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Highly Aligned Ultra-Thick Gel-Based Cathodes Unlocking Ultra-High Energy Density Batteries

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Energy & Environmental materials 2022年第4期5卷 1332-1339页

作者： Shichun Yang Chaochao Zhou Qiong Wang Binbin Chen Yan Zhao Bin Guo Zhengjie Zhang Xinlei Gao Ridwanur Chowdhury Huizhi Wang Chao Lai Nigel P.brandon Billy Wu Xinhua Liu School of Transportation Science and Engineering Beihang UniversityBeijing 100191China School of Chemistry and materials Science Jiangsu Normal UniversityXuzhou 221116China Imperial College London Sough Kensington CampusLondon SW72AZUK

Increasing electrode thickness can substantially enhance the specific energy of lithium-ion batteries;however,ionic transport,electronic conductivity,and ink rheology are current barriers to ***,a novel approach using a mixed xanthan gum and locust bean gum binder to construct ultrathick electrodes is proposed to address above *** combining aqueous binder with single-walled carbon nanotubes(SWCNT),active material(LiNi_(0.8)Co_(0.1)mn_(0.1)O_(2)) and subsequent vacuum freeze-drying,highly aligned,and low-tortuosity structures with a porosity of ca.50%can be achieved with an average pore size of 10μm,whereby the gum binder-SWCNT-NmC811 forms vertical structures supported by tissue-like binder/SWCNT networks allowing for excellent electronic conducting phase *** a result,ultra-thick electrodes with a mass loading of about 511 mg cm^(−2) and 99.5 wt%active materials have been demonstrated with a remarkable areal capacity of 79.3 mAh cm^(−2),which is the highest value reported so *** represents a>25×improvement compared with conventional electrodes with an areal capacity of about 3 mAh cm^(−2).This route also can be expanded to other electrode materials,such as LiFePO_(4) and Li_(4)Ti_(5)O_(12),and thus opens the possibility for low-cost and sustainable ultra-thick electrodes with increased specific energy for future lithium-ion batteries.

关键词： binder gel-based cathodes high areal capacity lithium-ion battery ultrathick electrodes

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