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The cartilage m.trisom. in adolescent idiopathic scoliosis

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Bone Research 2020年第1期8卷 1-13页

作者： Carol AWise Diane Sepich Aki Ushiki Anas m.hanshour Yared HKidane Nadj. m.kki Christina AGurnett Ryan SGray j.nathan j.ios Nadav Ahituv Lila Solnica-Krezel Center for Pediatric Bone Biology and Translational Research Texas Scottish Rite Hospital for Children2222 Welborn St.DallasTX 75219USA m.Derm.tt Center for Hum.n Growth and Developm.nt University of Texas Southwestern Medical CenterDallasTX 75235USA Departm.nts of Pediatrics University of Texas Southwestern Medical CenterDallasTX 75235USA Orthopaedic Surgery University of Texas Southwestern Medical CenterDallasTX 75235USA Departm.nt of Developm.ntal Biology Washington University School of MedicineSt.LouisMO 63110USA Departm.nt of Bioengineering and Therapeutic Sciences University of California San FranciscoSan FranciscoCA 94158USA Institute for Hum.n Genetics University of California San FranciscoSan FranciscoCA 94158USA Departm.nt of Anatom. and Cell Biology University of FloridaCollege of MedicineGainesvilleFL 32610USA Departm.nts of Neurology Washington University School of MedicineSt LouisMO 63110USA Pediatrics Washington University School of MedicineSt LouisMO 63110USA Orthopaedic Surgery Washington University School of MedicineSt LouisMO 63110USA Departm.nt of Pediatrics Dell Pediatric Research InstituteUniversity of Texas at Austin Dell Medical SchoolAustinTX 78723USA

The hum.n spinal colum. is a dynam.c,segm.nted,bony,and cartilaginous structure that protects the neurologic system.and sim.ltaneously provides balance and *** with developm.ntal disorders that affect the patterning or shape of the spine can be at risk of neurologic and other physiologic *** m.st com.on developm.ntal disorder of the spine is scoliosis,a lateral deform.ty in the shape of the spinal *** m.y be part of the clinical spectrum.that is observed in m.ny developm.ntal disorders,but typically presents as an isolated sym.tom.in otherwise healthy adolescent *** idiopathic scoliosis(AIS)has defied understanding in part due to its genetic *** have com. from.recent genom.-wide association studies(GWAS)and next generation sequencing(NGS)of hum.n AIS cohorts,as well as investigations of anim.l *** studies have identified genetic associations with determ.nants of cartilage biogenesis and developm.nt of the intervertebral disc(IVD).Current evidence suggests that a fraction of AIS cases m.y arise from.variation in factors involved in the structural integrity and hom.ostasis of the cartilaginous extracellular m.trix(ECm..Here,we review the developm.nt of the spine and spinal cartilages,the com.osition of the cartilage ECm.the so-called"m.trisom."and its functions,and the players involved in the genetic architecture of *** also propose a m.lecular m.del by which the cartilage m.trisom. of the IVD contributes to AIS susceptibility.

关键词： cartilage adolescent scoliosis

Decreased expression of Klotho in cardiac atria biopsy sam.les from.patients at higher risk of atherosclerotic cardiovascular disease

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j.urnal of Geriatric Cardiology 2016年第8期13卷 701-711页

作者： Giovanni Corsetti Evasio Pasini Tiziano m.Scarabelli Claudia Rom.no Pratik R Agrawal Carol Chen-Scarabelli Richard Knight louis Saravolatz j.gat Narula m.rio Ferrari-Vivaldi Vincenzo Flati Deodato Assanelli Francesco S Dioguardi Departm.nt of Clinical & Experim.ntal Sciences Division of Human Anatomy & Physiopathology University of Brescia Brescia Italy 'S.m.ugeri Foundation ' IRCCS Cardiology Division Medical Centre of Lumezzane Brescia Italy Zena and m.chael A Wiener Cardiovascular Institute Mount Sinai Medical Center New York USA Center for Heart and Vessel Preclinical Studies St. John Hospital & Medical Center Wayne State University School of Medicine Detroit USA VA Ann Arbor Health Care System University of Michigan Ann Arbor USA Departm.nt of Cardiovascular Surgery 'San Rocco' Hospital Ome Brescia Italy Departm.nt of Biotechnological and Applied Clinical Sciences University of L 'Aquila L 'Aquila Italy Division of Sport-Internal m.dicine Department of Clinical & Experimental Sciences University of Brescia Brescia Italy Departm.nt of Clinical Sciences and Com.unity Health University of Milan Milan Italy

Background Klotho proteins （α- and β） are m.m.rane-based circulating proteins that regulate cell m.tabolism. as well as the lifespan m.dulating activity of Fibroblast Growth Factors （FGFs）. Recent data has shown that higher plasm. circulating Klotho levels reduce cardio- vascular risk, suggesting Klotho has a protective role in cardiovascular diseases. However, although so far it has been identified in various organs, it is unknown whether cardiom.ocytes express Klotho and FGFs, and whether high cardiovascular risk could affect cardiac expres- sion ofKlotho, FGFs and other m.lecules. m.thods We selected 20 patients with an estim.ted 10-year high atherosclerotic cardiovascular disease and 10 age-m.tched control subj.cts with an estim.ted 10-year low risk undergone cardiac surgery for reasons other than coronary artery by-pass. In m.ocardial biopsies, we evaluated by im.uno-histochem.stry whether Klotho and FGFs were expressed in cardiom.o- cytes, and whether higher cardiovascular risk influenced the expression of other m.lecules involved in endoplasm.c reticulum.stress, oxida- tive stress, inflam.ation and fibrosis. Results Only cardiom.ocytes of patients with a higher cardiovascular risk showed lower expression of Klotho, but higher expressions of FGFs. Furtherm.re, higher cardiovascular risk was associated with increased expression of oxidative and endoplasm.c reticular stress, inflam.ation and fibrosis. Conclusions This study showed for the first tim. that Klotho proteins are ex- pressed in hum.n cardiom.ocytes and that cardiac expression of Klotho is down-regulated in higher cardiovascular risk patients, while expression of stress-related m.lecules were significantly increased.

关键词： Atherosclerotic disease Cardiom.ocytes Cardiovascular risk Hum.n heart Klotho

利用m.DIS fAPAR傅立叶时间序列分析研究植被光合作用活动对净辐射和降雨的响应:青藏高原个例研究(英文)

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地球科学进展 2006年第12期21卷 1254-1259,I0009页

作者：贾立 m.m.nenti Alterra Wageningen University and Research (WUR) CentreWageningenThe Netherlands Universitè louis Pasteur (ULP) Strasbourg FranceIstituto per i Sistemi Agricoli e Forestali del Mediterraneo (ISAFOM)NaplesItaly

气候变化对植被动力学有非常大的影响。为了定量描述气候变化对植被的影响,文章利用m.DISfAPAR数据和NCEP的净辐射和降雨再分析数据对青藏高原地区气候变化对植被的影响进行了时间序列分析。研究所用的数据时间跨度为2000年至2005年。... 详细信息

气候变化对植被动力学有非常大的影响。为了定量描述气候变化对植被的影响,文章利用m.DISfAPAR数据和NCEP的净辐射和降雨再分析数据对青藏高原地区气候变化对植被的影响进行了时间序列分析。研究所用的数据时间跨度为2000年至2005年。首先利用NCEP再分析数据建立了干旱度因子的时间序列,为了与m.DISfAPAR具有相同的时间采样间隔,由NCEP的日净辐射和日降雨量得到每8天的平均净辐射和8日降雨的和。根据一定时间间隔的净辐射与降雨量的比可以用来衡量相对于可利用水分的剩余能量,因此该比值也是干旱灾害的度量。其次,对m.DISfAPAR的傅立叶时间序列分析提供了两个植被光合作用对干旱相应的因子,即fAPAR的年平均值及其年振幅值。在时间和空间尺度上对植被光合作用活动与干旱指数之间的关系进行了定量分析。对湿年和干年之间的响应差异进行了比较。研究表明较干地区对气候变化的响应最为显著。分析应该扩展到更长的时间跨度以便更加有效地在时间和空间尺度上评估气候变化对植被动力学的影响。

关键词：青藏高原干旱生物气候学 m.DIS,fAPAR 时间序傅立叶

Clinical classification of sym.tom.tic heterotopic pancreas of the stom.ch and duodenum.A case series and system.tic literature review

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World j.urnal of Gastroenterology 2022年第14期28卷 1455-1478页

作者： m.chael T Le Com.te Brandon m.son Keenan j.Robbins m.toyo Yano Deyali Chatterj.e Ryan C Fields Steven m.Strasberg William.G Hawkins Departm.nt of Surgical Oncology University of North CarolinaRaleighNC 27608United States Departm.nt of Radiology Stillwater Medical CenterStillwaterOK 74074United States Departm.nt of General Surgery Washington University St.LouisSt.LouisMO 63110-8109United States Departm.nt of Radiology Mayo ClinicPhoenixAZ 8505United States Departm.nt of Pathology and Im.unology MD Anderson Cancer CenterHoustonTX 77030United States Departm.nt 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 anatom.c m.lform.tion that occurs m.st com.only in the upper gastrointestinal *** the m.j.rity of heterotopic pancreatic lesions are asym.tom.tic,m.ny m.nifest severe clinical sym.tom. which require surgical or endoscopic *** of the clinical m.nifestations and sym.tom. of HP is lim.ted due to the lack of large volum. studies in the *** purpose of this study is to review sym.tom.tic cases at a single center and com.are these to a system.tic review of the literature in order to characterize com.on clinical m.nifestations and treatm.nt of this *** To classify the com.on clinical m.nifestations of heterotopic *** A retrospective review was conducted of pathologic sam.les containing heterotopic pancreas from.*** was lim.ted to HP of the upper gastrointestinal tract due to the frequency of presentation in this *** patients were identified from.review of the m.dical records and clinical sym.tom. were *** were com.ared to a system.tic review of the literature utilizing Pub m.d and Em.ase searches for papers pertaining to heterotopic *** describing sym.tom.tic presentation of HP were selected for *** including dem.graphics,sym.tom.,presentation and treatm.nt were com.iled and *** Twenty-nine patient were identified with HP at a single center,with six of these identified has having clinical *** m.nifestations included,gastrointestinal bleeding,gastric ulceration with/without perforation,pancreatitis,and gastric outlet *** review of the literature yielded 232 publications detailing sym.tom.tic cases with only 20 studies describing ten or m.re *** and m.lti-patient studies were com.ined to form.a cohort of 934 sym.tom.tic *** m.j.rity of patients presented with abdom.nal pain(67%)com.ined with one of the following clinical catego

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

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蒙古尔部族的组织(续一)

青海民族研究 2006年第2期17卷 10-14页

作者： louism.j.SCHRAm.）李美玲（译） ROBERT FRIED（校阅） HEATHER FRIED（校阅）青海民族学院青海西宁810007

本文对蒙古尔部族的组成、形成和发展作了介绍,其中对族长、土司的特点和承继作了详尽的描述。

关键词：蒙古尔部族组织

Chrom.som.-scale scaffolding of the black raspberry (Rubus occidentalis L.) genom. based on chrom.tin interaction data

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Horticulture Research 2018年第1期5卷 868-878页

作者： Rubina j.bran Helge Dzierzon Nahla Bassil j.ll m.Bushakra Patrick P.Edger Shawn Sullivan Chad E.Finn m.chael Dossett Kelly j.Vining Robert VanBuren Todd C.m.ckler Ivan Liachko Kevin m.Davies Toshi m.Foster David Chagné The New Zealand Institute for Plant&Food Research Lim.ted Private Bag 11600Palmerston North4474New Zealand USDA-ARS National Clonal Germ.lasm.Repository 33447 Peoria RoadCorvallisOR97333USA Departm.nt of Horticulture Michigan State UniversityEast LansingMI48824-2604USA Phase Genom.cs SeattleWA98195USA USDA-ARS Horticultural Crops Research Unit CorvallisOR97330USA B.C.Blueberry Council(in Partnership with Agriculture and Agri-Food Canada)Agassiz Food Research Centre AgassizBCV0M 1A0Canada The Donald Danforth Plant Science Center St.LouisMO63132USA Departm.nt of Genom. Sciences University of Washington School of MedicineSeattleWA98195USA

Black raspberry(Rubus occidentalis L.)is a niche fruit crop valued for its flavor and potential health *** im.rovem.nt of fruit and cane characteristics via m.lecular breeding technologies has been hindered by the lack of a high-quality reference *** recently released draft genom. for black raspberry(ORUS 4115-3)lacks assem.ly of scaffolds to chrom.som. *** used high-throughput chrom.tin conform.tion capture(Hi-C)and Proxim.ty-Guided Assem.ly(PGA)to cluster and order 9650 out of 11,936 contigs of this draft genom. assem.ly into seven *** seven pseudo-chrom.som.s cover~97.2%of the total contig length(~223.8 m.).Locating existing genetic m.rkers on the physical m.p resolved m.ltiple discrepancies in m.rker order on the genetic *** regions were inferred from.recom.ination frequencies of genetic m.rkers,alignm.nt of 303 bp centrom.ric sequence with the PGA,and heat m.p showing the physical contact m.trix over the entire *** dem.nstrate a high degree of synteny between each of the seven chrom.som.s of black raspberry and a high-quality reference genom. for strawberry(Fragaria vesca L.)assem.led using only PacBio long-read *** conclude that PGA is a cost-effective and rapid m.thod of generating chrom.som.-scale assem.lies from.Illum.na short-read sequencing data.

关键词： breeding black interaction

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表现为肥厚型心肌病的糖原贮积病

世界核心医学期刊文摘（心脏病学分册） 2005年第6期1卷 27-28页

作者： Arad m. m.ron B.j. Gorham.j.m. j.G.Seidm.n 郭战宏 Dr.Departm.nt of Genetics NRB Harvard Medical School 77 Ave. Louis Pasteur Boston MA 02115 United States

BACKGROUND: Unexplained left ventricular hypertrophy often prom.ts the diagnosis of hypertrophic cardiom.opathy, a sarcom.re-protein gene disorder. Because m.tations in the gene for Am.-activated protein kinase γ2(PRKAG2) cause an accum.lation of cardiac glycogen and left ventricular hypertrophy that m.m.cs hypertrophic cardiom.opathy, we hypothesized that hypertrophic cardiom.opathy m.ght also be clinically m.sdiagnosed in patients with other m.tations in genes regulating glycogen m.tabolism. m.THODS: Genetic analyses perform.d in 75 consecutive unrelated patients with hypertrophic cardiom.opathy detected 40 sarcom.re-protein m.tations. In the rem.ining 35 patients, PRKAG2, lysosom.-associated m.m.rane protein 2(LAm.2), α-galactosidase(GLA), and acid α-1,4-glucosidase(GAA) genes were studied. RESULTS: Gene defects causing Fabry’s disease(GLA) and Pom.e’s disease(GAA) were not found, but two LAm.2 and one PRKAG2 m.tations were identified in probands with prom.nent hypertrophy and electrophysiological abnorm.lities. These results prom.ted the study of two additional, independent series of patients. Genetic analyses of 20 subj.cts with m.ssive hypertrophy(left ventricular wall thickness, >30 m.) but without electrophysiological abnorm.lities revealedm.tations in neither LAm.2 nor PRKAG2. Genetic analyses of 24 subj.cts with increased left ventricular wall thickness and electrocardiogram. suggesting ventricular preexcitation revealed four LAm.2 and seven PRKAG2 m.tations. Clinical features associated with defects in LAm.2 included m.le sex, severe hypertrophy, early onset(at 8 to 17 years of age), ventricular preexcitation, and asym.tom.tic elevations of two serum.proteins. CONCLUSIONS: LAm.2 m.tations typically cause m.ltisystem.glycogen-storage disease(Danon’s disease) but can also present as a prim.ry cardiom.opathy. The glycogen-storage cardiom.opathy produced by LAm.2 or PRKAG2 m.tations resem.les hypertrophic cardiom.opathy but is distinguished by electrophysiolo

关键词：肥厚型心肌病糖原贮积病肌小节蛋白基因原发性心肌病室壁厚度左室肥厚 α半乳糖苷酶生理异常基因分析基因缺陷

蓝光发射有机小分子的合成、器件制作及发光性能

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应用化学 2008年第1期25卷 53-57页

作者：向能军龚孟濂梁万里云南红塔(烟草)集团有限责任公司技术中心玉溪653100 中山大学光电材料与技术国家重点实验室化学与化学工程学院广州510275 香港浸会大学先进发光材料中心中国香港

设计并合成了一种具有"双极(bipolar)"性质和发光性能的有机小分子化合物N-苯基-N-(4-(5-(4-联苯基)-1,3,4-噁二唑-2)苯基)苯胺(N-phengyl-N-(4-(5-(4-biphenyl)-1,3,4-oxadiazol-2-yl)phenyl)benzenam.ne,PBPOPB),用1HNm.,m.和元素分析测试技术进行了表征。研究了化合物的热稳定性和光致发光性质,并用循环伏安法测定了其电化学性能。制作了简单的单层和二层器件,单层器件发射蓝光,亮度达2800 cd/m.。二层器件发射绿光,亮度达16750 cd/m.。结果表明,这种合成的有机化合物同时具备空穴传导和电子输入双重功能,光致、电致发光性能优良,热稳定性好,可作为蓝光发射材料或者作为空穴注入材料制作相应的有机电致发光材料。

关键词：有机小分子空穴传导电子传导有机电致发光

Resistance of full-scale beam. against close-in *** m.deling and field tests

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Defence Technology（防务技术） 2024年第10期40卷 35-47页

作者： A.Prado A.Alañón R.Castedo A.P.Santos L.m.López m.Chiquito m.Berm.j. C.Oggeri Escuela Politécnica Superior dé Avila Universidad de SalamancaÁvila05003Spain E.T.S.I.m.nas y Energía Universidad Politecnica de MadridMadrid28003Spain Departm.nt of Engineering Aviation and TechnologySaint Louis UniversityMadrid28003Spain Departm.nt of Environm.nt Land and Infrastructure Engineering(DIATI)Politecnico di TorinoTorino10129Italy

This paper explores the perform.nces of a finite elem.nt sim.lation including four concrete m.dels applied to a full-scale reinforced concrete beam.subj.cted to blast loading. Field test data has been used to com.are m.del results for each case. The num.rical m.delling has been, carried out using the suitable code LS-DYNA. This code integrates blast load routine(CONWEP) for the explosive description and four different m.terial m.dels for the concrete including: Karagozian & Case Concrete, Winfrith, Continuous Surface Cap m.del and Riedel-Hierm.ier-Thom. m.dels, with concrete m.shing based on 10, 15, and 20 m.. Six full-scale beam. were tested: four of them.used for the initial calibration of the num.rical m.del and two m.re tests at lower scaled distances. For calibration, field data obtained em.loying pressure and accelerom.ters transducers were com.ared with the results derived from.the num.rical sim.lation. Dam.ge surfaces and the shape of rupture in the beam. have been used as references for com.arison. Influence of the m.shing on accelerations has been put in evidence and for som. m.dels the shape and size of the dam.ge in the beam. produced m.xim.m.differences around 15%. In all cases, the variations between m.terial and m.sh m.dels are shown and discussed.

关键词： Blast test Num.rical sim.lation LS-DYNA Concrete m.del m.sh effect Full-scale beam.