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Partial external biliary diversion in bile salt export pump deficiency: Association between outcome and mutation

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World Journal of Gastroenterology 2017年第29期23卷 5295-5303页

作者： Philipp Ellinger Jan Stindt Carola Droge Katharina Sattler Claudia Stross Stefanie Kluge Diran Herebian Sander HJ Smits Martin Burdelski Sebastian Schulz-Jürgensen Antje Ballauff Jan Schulte am Esch Ertan Mayatepek Dieter Haussinger Ralf Kubitz lutz schmitt In-stitute of Biochemistry Hein-rich-Hein-e-Un-iversity Düsseldorf Medical Faculty Departmen-t of Gastroen-terology Hepatology an-d In-fectious Diseases Hein-rich-Hein-e-Un-iversity Düsseldorf Departmen-t of Gen-eral Pediatrics Neon-atology an-d Pediatric Cardiology Medical Faculty Hein-rich-Hein-e-Un-iversity Düsseldorf Departmen-t of Paediatrics Un-iversity Hospital Schleswig Holstein- Departmen-t of Paediatrics Helios Clin-ic Departmen-t of Gen-eral Surgery Hein-rich-Hein-e-Un-iversity

To investigate the relation of two different mutations to the outcome of partial external biliary diversion (PEBD) in severe bile salt export pump (BSEP) deficiency. METHODSMutations in the gene encoding BSEP leading to severe BSEP deficiency in two unrelated patients were identified by genomic sequencing. Native liver biopsies and transiently transfected human embryonic kidney (HEK) 293 cells expressing either wild-type or mutated BSEP were subjected to immunofluorescence analysis to assess BSEP transporter localization. Bile acid profiles of patient and control bile samples were generated by ultra-performance liquid chromatography-tandem mass spectrometry. Wild-type and mutant BSEP transport of [3H]-labeled taurocholate (TC) and taurochenodeoxycholate (TCDC) was assessed by vesicular transport assays. RESULTSA girl (at 2 mo) presented with pruritus, jaundice and elevated serum bile salts (BS). PEBD stabilized liver function and prevented liver transplantation. She was heterozygous for the BSEP deletion p.T919del and the nonsense mutation p.R1235X. At the age of 17 years relative amounts of conjugated BS in her bile were normal, while total BS were less than 3% as compared to controls. An unrelated boy (age 1.5 years) presenting with severe pruritus and elevated serum BS was heterozygous for the same nonsense and another missense mutation, p.G1032R. PEBD failed to alleviate pruritus, eventually necessitating liver transplantation. BS concentration in bile was about 5% of controls. BS were mainly unconjugated with an unusual low amount of chenodeoxycholate derivatives (< 5%). The patients’ native liver biopsies showed canalicular BSEP expression. Both BSEP p.T919del and p.G1032R were localized in the plasma membrane in HEK293 cells. In vitro transport assays showed drastic reduction of transport by both mutations. Using purified recombinant BSEP as quantifiable reference, per-molecule transport rates for TC and TCDC were determined to be 3 and 2 BS molecu

关键词： Familial intrahepatic cholestasis type 2 Partial external biliary diversion Bile salt export pump ATP binding cassette transporter Intrahepatic cholestasis

安全可靠地输送高温介质——新的排气技术提高了流程泵的可靠性

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流程工业 2012年第5期 90-90,92,93页

作者： Stephan Sylvain schmitt lutz Urban KSB公司

在很多流程工艺过程中都会用到导热油，正确的温度控制对保障最终产品的质量和平衡能源起着重要的作用。在现代化的流程工艺过程中，导热油输送循环过程扮演着重要的角色。Etanorm SYT系列流程泵的排气系统Ven Jet申请了专利保护，这一... 详细信息

在很多流程工艺过程中都会用到导热油，正确的温度控制对保障最终产品的质量和平衡能源起着重要的作用。在现代化的流程工艺过程中，导热油输送循环过程扮演着重要的角色。Etanorm SYT系列流程泵的排气系统Ven Jet申请了专利保护，这一排气系统能够安全可靠地输送高温介质。

关键词：热油输送排气系统高温介质安全可靠流程泵可靠性技术工艺过程

A force-compensated compliant MEMS-amplifier with electrostatic anti-springs

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Microsystems & Nanoengineering 2023年第3期9卷 235-244页

作者： Philip schmitt Martin Hoffmann Microsystems Technology Ruhr-Universität BochumUniversitätsstr.15044801 BochumGermany

In this paper,an electrostatic compliant mechanical amplifier intended for force-compensated displacement amplification in MEMS sensor applications is ***,mechanical transformers that enhance a small input displacement into a large output displacement generate large forces at the input of the *** microsystem proposed here allows for the reduction and compensation of the input stiffness of the amplifier and any mechanical components connected to it while providing a constant amplification ratio at the same *** amplifying mechanism features bidirectional electrostatic anti-springs enabling the control of the stiffness by applying a constant DC *** electrode design of the anti-springs and its influence on the force-displacement characteristic,the side instability and the maximal displacement are studied through analytical approaches and supported by FEA and by *** on the derived models,a compliant electromechanical amplifier is developed,featuring an amplification ratio of *** this amplifier the initial input stiffness of 422 N/m could be reduced to 6.8 N/m by applying a voltage of 100 *** an additional application,we show how the amplifier can be used as a mechanical force sensor with tuneable sensitivity,where the forces at the input are transformed into large output *** experiments,we show how the sensitivity can be adjusted and increased by a factor of 25 by applying a voltage at the anti-springs.

关键词： amplifier electrostatic spring

Spatial modeling issues in future smart cities

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Geo-Spatial Information Science 2013年第1期16卷 7-12页

作者： Gerhard schmitt Department of Architecture and Future Cities Laboratory ETH ZürichZürichSwitzerlandand ETH CREATESingapore

It is our goal to make today’s and future cities smart,sustainable and *** order to achieve this,it is fundamental to understand how each city works,to formalize the knowledge gained and to apply it to a city model as the base for simulations that can generate future scenarios with a high level of *** nature of this model,which must cover design,qualitative and quantitative aspects,has changed over *** this study,we focus on the role of the spatial dimension and of geometry in a city *** from being a dominating generative force in ancient cities,spatial modeling has developed into an underlying description language for present and future cities to define functions and properties of the city in space and *** example of the stocks and flows model applied to the city depicts where and how spatial modeling influences the design,construction and performance of the future Smart City.

关键词： future cities spatial modeling stocks and flows city models reality-based 3D models

In memoriamWalter Joseph Bock—a life dedicated to teaching and research in the biological sciences(20 November 1933-27 January 2022)

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Integrative Zoology 2022年第6期17卷 1215-1218页

作者： Michael schmitt John BUCKERIDGE Universität Greifswald Allgemeine&Systematische ZoologieGreifswaldGermany Professor Emeritus RMIT UniversityMelbourneVictoriaAustralia

Walter Joseph Bock(Fig.1)was born in Woodhaven,Queens County,New York,NY(USA),the second of 4 children of Paul and Anne Bock,née *** grew up in a German speaking family who discontinued speaking German only at the outset of *** 1947 to 1951 he attended Brooklyn Technical High School,where there was considerable emphasis on technological *** 1951,he entered Cornell University in Ithaca,NY,graduating with a Bachelor of Science in *** Cornell,he attended a short lecture series presented by the famed ethologist Konrad Lorenz,and began an association with the ornithologist Charles *** links provided Walter with a growing appreciation of the wonders of the world of biology.

关键词： teaching Walter continued

Mitophagy:From the dark into the spotlight

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Molecular Plant 2023年第10期16卷 1487-1489页

作者： Vivian schmitt Olivier Van Aken Department of Biology Lund UniversityLundSweden

Despite the generally placid appearance of plants,many dynamicprocesses take place inside a plant *** such process thathas garnered attention in recent years is termed"autophagy."While originally discovered in yeast,our understanding of autophagy in plants has improved *** is considered a cellular recycling system in which cellular components oreven entire organelles can be encapsulated by a membranestructure(autophagosome)and then transported to the vacuole(in plants)for degradation and re-use.

关键词： attention organelle spite

Molecular dynamics simulation of the Stribeck curve: Boundary lubrication, mixed lubrication, and hydrodynamic lubrication on the atomistic level

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Friction 2023年第12期11卷 2342-2366页

作者： Simon STEPHAN Sebastian schmitt Hans HASSE Herbert M.URBASSEK Laboratory of Engineering Thermodynamics(LTD) Department of Mechanical and Process EngieeringTU KaiserslauternRheinland-Pfalz 67663Germany Physics Department and Research Center(OPTIMAS) TU KaiserslauternRheinland-Pfalz 67663Germany

Lubricated contact processes are studied using classical molecular dynamics simulations for determining the entire range of the Stribeck ***,the lateral movement of two solid bodies at different gap height are *** each simulation,a rigid asperity is moved at constant height above a flat iron surface in a lubricating *** methane and decane are considered as *** three main lubrication regimes of the Stribeck curve and their transition regions are covered by the study:Boundary lubrication(significant elastic and plastic deformation of the substrate),mixed lubrication(adsorbed fluid layer dominates the process),and hydrodynamic lubrication(shear flow is set up between the surface and the asperity).We find the formation of a tribofilm in which lubricant molecules are immersed into the metal surface—not only in the case of scratching,but also for boundary lubrication and mixed *** formation of a tribofilm is found to have important consequences for the contact ***,the two fluids are found to show distinctly different behavior in the three lubrication regimes:For hydrodynamic lubrication(large gap height),decane yields a better tribological performance;for boundary lubrication(small gap height),decane shows a larger friction coefficient than methane,which is due to the different mechanisms observed for the formation of the tribofilm;the mixed lubrication regime can be considered as a transition regime between the two other ***,it is found that the nature of the tribofilm depends on the lubricant:While methane particles substitute substrate atoms sustaining mostly the crystalline structure,the decane molecules distort the substrate surface and an amorphous tribofilm is formed.

关键词： boundary lubrication mixed lubrication hydrodynamic lubrication molecular dynamics simulation tribofilm

Examining the effect of adverse geological conditions on jamming of a single shielded TBM in Uluabat tunnel using numerical modeling

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Journal of Rock Mechanics and Geotechnical Engineering 2017年第6期9卷 1112-1122页

作者： Rohola Hasanpour Jürgen schmitt Yilmaz Ozcelik Jamal Rostami Institute for Tunneling and Construction Management Ruhr-University Bochum Department of Civil Engineering Darmstadt University of Applied Science Department of Mining Engineering Hacettepe University Department of Mining Engineering Colorado School of Mines

Severe shield jamming events have been reported during excavation of Uluabat tunnel through adverse geological conditions, which resulted in several stoppages at advancing a single shielded tunnel boring machine(TBM). To study the jamming mechanism, three-dimensional(3D) simulation of the machine and surrounding ground was implemented using the finite difference code FLAC3D. Numerical analyses were performed for three sections along the tunnel with a higher risk for entrapment due to the combination of overburden and geological conditions. The computational results including longitudinal displacement contours and ground pressure profiles around the shield allow a better understanding of ground behavior within the excavation. Furthermore, they allow realistically assessing the impact of adverse geological conditions on shield jamming. The calculated thrust forces, which are required to move the machine forward, are in good agreement with field observations and measurements. It also proves that the numerical analysis can effectively be used for evaluating the effect of adverse geological environment on TBM entrapments and can be applied to prediction of loads on the shield and preestimating of the required thrust force during excavation through adverse ground conditions.

关键词： Single shielded tunnel boring machine(TBM) Numerical modeling Shield jamming Squeezing ground Uluabat tunnel

Interferometric measurement of injection nozzles using ultra-small fiber-optical probes

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Chinese Optics Letters 2011年第7期9卷 37-40页

作者： Tilo Pfeifer Robert schmitt Niels Knig Guilherme Francisco Mallmann Fraunhofer Institute for Production Technology Steinbachstr. 17 52074 Aachen Germany Laboratory for Machine Tools and Production Engineering of RWTH Aachen University

The measurement of boreholes with diameters smaller than 500 μm is a demanding task that cannot be performed using state-of-the-art production metrology. In this letter, a miniaturized fiber probe with a diameter of 80μm is presented. A probe is used for low-coherence interferometry to conduct highly precise measurements of form deviations of small boreholes. Measurements conducted in nozzles are also presented. The results prove the potential of the fiber-optical sensor for quality inspection of high-precision parts, such as injection nozzles, for common-rail diesel engines.

关键词： Charge coupled devices Fizeau interferometry Low coherence interferometry Metrology Michelson interferometers Optical fibers