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Tsc2 mutation induces renal tubular cell nonautonomous disease

Genes & Diseases 2022年第1期9卷 187-200页

作者： Prashant Kumar Fahad Zadjali Ying Yao Daniel Johnson Brian Siroky Aristotelis Astrinidis peter vogel Kenneth W.Gross John J.Bissler Department of Pediatrics University of Tennessee Health Science Center and Le Bonheur Children's HospitalMemphisTN 38103USA Children's Foundation Research Institute(CFRI) Le Bonheur Children's HospitalMemphisTN 38105USA Department of Clinical Biochemistry College of Medicine&Health SciencesSultan Qaboos UniversityMuscat 123Oman Molecular Bioinformatics Center University of Tennessee Health Science CenterMemphisTN 38163USA Department of Veterinary Pathology St.Jude Children's Research HospitalMemphisTN 38105USA Department of Molecular and Cellular Biology Roswell Park Comprehensive Cancer CenterBuffaloNY 14263USA Pediatric Medicine Department St.Jude Children's Research HospitalMemphisTN 38105USA

TSC renal cystic disease is poorly understood and has no approved *** a new principal cell-targeted murine model of Tsc cystic disease,the renal cystic epithelium is mostly composed of type A intercalated cells with an intact Tsc2 gene confirmed by sequencing,although these cells exhibit a Tsc-mutant disease *** used a newly derived targeted murine model in lineage tracing and extracellular vesicle(EV)characterization experiments and a cell culture model in EV characterization and cellular induction experiments to understand TSC *** lineage tracing experiments,we found principal cells undergo clonal expansion but contribute very few cells to the *** determined that cystic kidneys contain more interstitial EVs than noncystic kidneys,excrete fewer EVs in urine,and contain EVs in cyst ***,the loss of Tsc2 gene in EV-producing cells greatly changes the effect of EVs on renal tubular epithelium,such that the epithelium develops increased secretory and proliferative pathway *** demonstate that the mTORC1 pathway activity is independent form the EV production,and that the EV effects for a single cell line can vary *** cystogenesis involves significant contribution from genetically intact cells conscripted to the mutant phenotype by mutant cell derived EVs.

关键词： Cell nonautonomous trait Polycystic kidney disease Renalcystogenesis Tuberous sclerosis complex

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High-throughput screening of mouse gene knockouts identifies established and novel skeletal phenotypes

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Bone Research 2014年第3期2卷 152-181页

作者： Robert Brommage Jeff Liu Gwenn M Hansen Laura L Kirkpatrick David G Potter Arthur T Ss Brian Zambrowicz David R Powell peter vogel Lexicon Pharmaceuticals The Woodlands TX USA

Screening gene function in vivo is a powerful approach to discover novel drug targets. We present high-throughput screening （HTS） data for 3 762 distinct global gene knockout （KO） mouse lines with viable adult homozygous mice generated using either gene-trap or homologous recombination technologies. Bone mass was determined from DEXA scans of male and female mice at 14 weeks of age and by microCT analyses of bones from male mice at 16 weeks of age. Wild-type （WT） cagemates/littermates were examined for each gene KO. Lethality was observed in an additional 850 KO lines. Since primary HTS are susceptible to false positive findings, additional cohorts of mice from KO lines with intriguing HTS bone data were examined. Aging, ovariectomy, histomorphometry and bone strength studies were performed and possible non-skeletal phenotypes were explored. Together, these screens identified multiple genes affecting bone mass： 23 previously reported genes （Calcr, Cebpb, Crtap, Dcstamp, Dkkl, Duoxa2, Enppl, Fgf23, Kissl/Kisslr, Kl （Klotho）, Lrp5, Mstn, Neol, Npr2, Ostml, Postn, Sfrp4, S1c30a5, Sic39a13, Sost, Sumf1, Src, Wnt10b）, five novel genes extensively characterized （Cldn18, Fam20c, Lrrkl, Sgpll, Wnt16）, five novel genes with preliminary characterization （Agpat2, RassfS, Slc10a7, Stc26a7, Slc30a10） and three novel undisclosed genes coding for potential osteoporosis drug targets.

关键词： KO High-throughput screening of mouse gene knockouts identifies established and novel skeletal phenotypes BMD HTS DEXA gene

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New tight junction protein 2 variant causing progressive familial intrahepatic cholestasis type 4 in adults: A case report

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World Journal of Gastroenterology 2020年第5期26卷 550-561页

作者： Chun-Shan Wei Naja Becher Jenny Blechingberg Friis peter Ott Ida vogel Henning Grønbæk Department of Clinical Medicine-Department of Hepatology and Gastroenterology Aarhus University HospitalAarhus N DK-8200Denmark Department of Hepatology Shenzhen Traditional Chinese Medicine HospitalThe Fourth Clinical Medical College of Guangzhou University of Chinese MedicineShenzhen 518033Guangdong ProvinceChina Department of Clinical Genetics Aarhus University HospitalAarhus N DK-8200Denmark

BACKGROUND Progressive familial intrahepatic cholestasis(PFIC)encompasses a group of autosomal recessive disorders with high morbidity and *** in the gene encoding tight junction protein-2(TJP2)have been linked to PFIC type 4(PFIC4),which predominantly presents in ***,there are only limited data from adults with TJP2-related *** report a family with an autosomal recessive disorder with a novel variant in the TJP2 gene in adults with very variable expression of *** SUMMARY The index patient presented at 19 years old with liver cirrhosis and variceal bleeding and was treated with endoscopic banding and *** 2018,he developed primary liver cancer that was treated with radiofrequency ablation followed by liver transplantation in *** testing revealed a novel homozygous TJP2 variant causing PFIC4(TJP2([NM_004817.3]:c.[3334C>T];[3334C>T])).The consanguineous family consists of the father and mother(both heterozygous)and their 12 children,of which five carry the variant in a homozygous state;however,these five siblings have highly variable expression of *** homozygous brothers had cirrhosis and portal hypertension at diagnosis at the ages of 19 and *** other homozygous brothers,age 23 and 19,and the homozygous sister,age 21,have elevated liver enzymes but presently no cirrhosis,which may suggest an age-dependent *** addition,five sisters had severe and mild intrahepatic cholestasis of pregnancy and carry the TJP2 variant in a homozygous and heterozygous state,*** This novel TJP2 variant is associated with PFIC4 causing severe liver disease with cirrhosis and primary liver cancer in adolescents/adults.

关键词： Progressive familial intrahepatic cholestasis Tight junction protein 2 Genetic variants Liver cirrhosis Liver cancer Case report

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Challenges for Software Engineering in Automation

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Journal of Software Engineering and Applications 2014年第5期7卷 440-451页

作者： Birgit vogel-Heuser Christian Diedrich Alexander Fay Sabine Jeschke Stefan Kowalewski Martin Wollschlaeger peter Gohner Institute of Automation and Information Systems Technische Universitat MünchenMünchenGermany Institute for Automation and Communication Otto von Guericke University MagdeburgMagdeburgGermany Institute of Automation Technology Helmut-Schmidt-UniversityHamburgGermany Institute of Information Management in Mechanical Engineering RWTH Aachen UniversityAachenGermany Computer Science 11—Embedded Software Laboratory RWTH Aachen UniversityAachenGermany Institute for Applied Computer Science Dresden University of TechnologyDresdenGermany Institute of Industrial Automation and Software Engineering University of StuttgartStuttgartGermany

This paper gives an introduction to the essential challenges of software engineering and requirements that software has to fulfill in the domain of automation. Besides, the functional characteristics, specific constraints and circumstances are considered for deriving requirements concerning usability, the technical process, the automation functions, used platform and the well-established models, which are described in detail. On the other hand, challenges result from the circumstances at different points in the single phases of the life cycle of the automated system. The requirements for life-cycle-management, tools and the changeability during runtime are described in detail.

关键词： Automation Software Engineering Models Tools

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Baseline neutrophil-lymphocyte ratio and platelet-lymphocyte ratio appear predictive of immune treatment related toxicity in hepatocellular carcinoma

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World Journal of Gastrointestinal Oncology 2023年第11期15卷 1900-1912页

作者： Sirish Dharmapuri UmutÖzbek Hiren Jethra Tomi Jun Thomas U Marron Anwaar Saeed Yi-Hsiang Huang Mahvish Muzaffar Matthias Pinter Lorenz Balcar Claudia Fulgenzi Suneetha Amara Arndt Weinmann Nicola Personeni Bernhard Scheiner Tiziana Pressiani Musharraf Navaid Bertram Bengsch Sonal Paul Uqba Khan Dominik Bettinger Naoshi Nishida Yehia Ibrahim Mohamed Arndt vogel Anuhya Gampa James Korolewicz Antonella Cammarota Ahmed Kaseb peter R Galle Anjana Pillai Ying-Hong Wan Alessio Cortellini Masatoshi Kudo Antonio D’Alessio Lorenza Rimassa David James Pinato Celina Ang Tisch Cancer Institute Icahn School of Medicine at Mount SinaiNew YorkNY 10029United States Department of Population Health Science and Policy Icahn School of Medicine at Mount SinaiNew YorkNY 10029United States Department of Data Analytics Harrisburg Harrisburg University of Science and TechnologyHarrisburdPA 17101United States SEMA4 StamfordCT 06902United States Division of Medical Oncology Kansas University of Kansas Cancer CenterKansasMO 66160United States Division of Gastroenterology and Hepatology Taipei Veterans General HospitalTaipei 11217Taiwan Department of Internal Medicine Brody School of MedicineEast Carolina UniversityGreenvilleNC 27858United States Department of Internal Medicine III Division of Gastroenterology and HepatologyMedical University of ViennaVienna 1090Austria Department of Surgery and Cancer Imperial College LondonHammersmith Hospital LondonLondon W120HSUnited Kingdom Department of Hepatology Johannes Gutenberg-University Medical CentreNiedersachsen 30625Germany Medical Oncology Unit ASST GardaVia Lungomella ValsecchiBresciaManerbio 25025Italy Medical Oncology and Hematology Unit Humanitas Cancer CenterIRCCS Humanitas Research HospitalMilanRozzano 20089Italy Department of Medicine II Univ Med Ctr FreiburgHugstetter Str 55University Hospital FreiburgFreiburg D-79106Germany Department of Oncology Baltimore LifeBridge HealthBaltimoreMD 21215United States Division of Hematology and Oncology Weill Cornell Medical CollegeNY 10065United States Department of Gastroenterology and Hepatology Kindai University Faculty of MedicineOsaka 577-8502Japan Department of Gastrointestinal Medical Oncology The University of Texas MD Anderson Cancer CenterHoustonTX 77030United States Department of Gastroenterology Hepatology and Endocrinology HannoverArndt VogelMedical School HannoverCarl-Neubergstr.Hannover 30659Germany Department of Hepatology Rush University Medical Group 1725 W Harrison St Ste 158ChicagoIL 60612United States Department of Biomedica

BACKGROUND A well-recognized class effect of immune checkpoint inhibitors(ICI)is immune-related adverse events(IrAEs)ranging from low grade toxicities to life-threatening end organ damage requiring permanent discontinuation of *** are reported in300 was significantly associated with a lower incidence of grade≥2 IrAEs(OR=0.40;P=0.044).Similarly,a trend was observed between NLR>5 and lower incidence of grade≥2 IrAEs(OR=0.58;P=0.097).Multivariate analyses confirmed PLR>300 as an independent predictive marker of grade≥2 IrAEs(OR=0.26;P=0.011),in addition to treatment with programmed cell death ligand 1(PD-1)/cytotoxic T lymphocyte-associated protein-4(OR=2.57;P=0.037)and PD-1/tyrosine kinase inhibitor(OR=3.39;P=0.01)*** use was not associated with IrAE incidence(OR=1.02;P=0.954).Patients treated with steroids had a>2-fold higher incidence of gr

关键词： Neutrophil-lymphocyte ratio Platelet-lymphocyte ratio Inflammatory biomarkers Immunotherapy Immune toxicity

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WERITAS区域访客及红酒中心奥地利瓦格拉姆山麓基希贝格

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世界建筑导报 2018年第3期33卷 71-73页

作者： matthias raiger peter blasl laurenz vogel oliver gerner christian münster 无不详 gerner gerner plus

由于商铺、酒吧、餐馆云集,WERITAS成为奥地利红酒区核心区域。WERITAS采用钢筋混凝土结构,玻璃墙面,玻璃后的钢柱起支撑作用。可见区域均采用灰绿色纤维水泥墙面,运用陡坡地基切合当地地形,让整栋建筑自然的融入周边环境中。整栋建筑,... 详细信息

由于商铺、酒吧、餐馆云集,WERITAS成为奥地利红酒区核心区域。WERITAS采用钢筋混凝土结构,玻璃墙面,玻璃后的钢柱起支撑作用。可见区域均采用灰绿色纤维水泥墙面,运用陡坡地基切合当地地形,让整栋建筑自然的融入周边环境中。整栋建筑,像一个＂玻璃箱＂矗立在树丛中,作为地标十分显眼。后来证明在陡峭斜坡上选址十分科学,因为地下部分在夏天依然十分凉爽,减少了对空调的依赖。

关键词：奥地利红酒钢筋混凝土结构访客玻璃墙面支撑作用水泥墙面绿色纤维

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一座传授木材技术的木建筑

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世界建筑 2018年第7期 116-119页

作者： Marcel Meili Markus peter Zeno vogel Thomas Kühne Marc Loeliger Andreas Schmidt Thomas Schnabel Urs Schnenberger Othmar Villiger Meili Peter Architekten

在瑞士的汝拉山脚下,有一座人口约5万的小城市——比尔（Biel/Bienne）,自19世纪起就一直拥有繁荣的工业和手表制造业。与这座城市的工业氛围相得益彰的,是充满实用精神的应用科学教育。所属于伯尔尼应用科学大学（Bern University of A... 详细信息

在瑞士的汝拉山脚下,有一座人口约5万的小城市——比尔（Biel/Bienne）,自19世纪起就一直拥有繁荣的工业和手表制造业。与这座城市的工业氛围相得益彰的,是充满实用精神的应用科学教育。所属于伯尔尼应用科学大学（Bern University of Applied Sciences）的比尔高等木材技术学校（Higher Technical School of Wood Biel,HTS）即坐落于这里。

关键词：技术学校木材木建筑应用科学科学教育小城市制造业工业

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