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

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

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Chinese contributions to liver transplantation

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Hepatobiliary & Pancreatic Diseases International 2025年第1期24卷 1-5页

作者： jan Lerut Institute for Experimental and Clinical Research(IREC) Universitécatholique Louvain(UCL)

Thefirstlivertransplantation(LT)inhumanswasperformed by Thomas Starzl on March 1, 1963 [1, 2](Fig. 1). During the period 1963-1983, this challenging procedure was performed in four centers only, one in the USA and three in Western Europe:Denver,Cambridge,*** including only 540 cases (meaning 27(!) cases yearly) was the basis of the 1983 National Institutes of Health (NIH) development consensus conference leading to the conclusion that LT could become “a promising alternative to the current therapy in the managementofthelatephaseofseveralformsofseriousliverdisease…candidates are those who exhausted alternative medical and surgical treatments…and are approaching the terminal phase of their illness”. Patients presenting liver tumors and inherited,

关键词： thereby approaching conclusion

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Oligodendroglial heterogeneity in health,disease,and recovery:deeper insights into myelin dynamics

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Neural Regeneration Research 2025年第11期20卷 3179-3192页

作者： Pieter-jan Serneels Julie D.De Schutter Lies De Groef Lieve Moons Steven Bergmans KU Leuven Leuven Brain InstituteDepartment of BiologyAnimal Physiology and Neurobiology DivisionNeural Circuit Development&Regeneration Research GroupLeuvenBelgium KU Leuven Leuven Brain InstituteDepartment of BiologyAnimal Physiology and Neurobiology DivisionCellular Communication&Neurodegeneration Research GroupLeuvenBelgium

Decades of research asserted that the oligodendroglial lineage comprises two cell types:oligodendrocyte precursor cells and ***,recent studies employing single-cell RNA sequencing techniques have uncovered novel cell states,prompting a revision of the existing *** forward,the oligodendroglial lineage should be delineated into five distinct cell states:oligodendrocyte precursor cells,committed oligodendrocyte precursor cells,newly formed oligodendrocytes,myelin-forming oligodendrocytes,and mature *** new classification system enables a deeper understanding of the oligodendroglia in both physiological and pathological *** this uniform terminology will facilitate comparison and integration of data across ***,including the consolidation of findings from various demyelinating models,is essential to better understand the pathogenesis of demyelinating ***,comparing injury models across species with varying regenerative capacities can provide insights that may lead to new therapeutic strategies to overcome remyelination ***,by standardizing terminology and synthesizing data from diverse studies across different animal models,we can enhance our understanding of myelin pathology in central nervous system disorders such as multiple sclerosis,Alzheimer's disease,and amyotrophic lateral sclerosis,all of which involve oligodendroglial and myelin dysfunction.

关键词： animal models demyelination fish mammals multiple sclerosis myelin neurodegeneration oligodendrocytes oligodendroglial lineage remyelination

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Assessing XEN microstent’s one-year efficacy:independent of site variability

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International Journal of Ophthalmology(English edition) 2025年第1期18卷 86-93页

作者： Caroline Bormann Catharina Busch Matus Rehak Christian Thomas Scharenberg Focke Ziemssen jan Darius Unterlauft Department of Ophthalmology University of LeipzigLiebigstrasse 10-14Leipzig 04103Germany Department of Ophthalmology University of InnsbruckAnichstraBe 35Innsbruck 6020Austria Smile Eyes Augen-und Laserzentrum Leipzig LampestraBe 1Leipzig 04107Germany Department of Ophthalmology InselspitalUniversity of BernFreiburgstrasse 4Bern 3010Switzerland

AIM:To evaluate the short-term efficacy of XEN45 Gel Stent(XEN)implantation for primary open angle glaucoma(POAG)and pseudoexfoliation(PEX)glaucoma across two university eye clinics,aiming to assess the impact of varying center-specific protocols during the first postoperative ***:We retrospectively examined 282 patients(183 in center 1,99 in center 2),who underwent XEN microstent implantation for uncontrolled POAG or PEX *** including intraocular pressure(IOP),IOP-lowering medication count,best corrected visual acuity(BCVA),and postoperative complications were evaluated over ***:Post-implantation,center 1 reported a mean IOP reduction from 25.3±7.4 to 14.1±4.7 mm Hg(P<0.01)and a decrease in IOP-lowering medications from 3.2±1.2 to 1.0±1.3(P<0.01).Center 2 observed a similar reduction from 24.4±6.5 to 15.1±5.5 mm Hg(P<0.01)and medication decrease from 3.0±1.1 to 1.2±1.0(P<0.01).BCVA remained stable in both *** most common complications were hypotony(center 1:32;center 2:20)and choroidal detachment(center 1:22,center 2:15),with nearly identical needling rates(40% in center 1,41% in center 2).CONCLUSION:XEN implantation yields consistent reductions in IOP and medication use across different centers using comparable surgical and postoperative treatment *** findings underscore XEN’s shortterm effectiveness and suggest standardizable outcomes regardless of exact surgical procedure or treatment differences.

关键词： glaucoma XEN45 Gel Stent minimally invasive glaucoma surgery

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Fabrication and fracture behaviors of the continuous brittle fiber reinforced tungsten composites fabricated via field-assisted sintering technology

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Tungsten 2025年第1期7卷 172-182页

作者： Rui Shu Yi-Ran Mao jan W. Coenen Alexis Terra Stephan Sch?nen Johann Riesch Christian Linsmeier Christoph Broeckmann Juelich Research Center Institute of Energy and Climate Research Plasma Physics (IEK-4) Partner in the Trilateral Euregio Cluster Institute of Materials Applications in Mechanical Engineering (IWM) RWTH Aachen University Department of Nuclear Engineering & Engineering Physics University of Wisconsin Madison Central Institute of Engineering Electronics and Analytics Engineering and Technology (ZEA-1) Juelich Research Center Max Planck Institute for Plasma Physics Institute of Materials Applications in Mechanical Engineering IWM RWTH Aachen University

Tungsten（W） is a promising candidate material for the plasma-facing components in future fusion ***,it has issues regarding the intrinsic brittleness as well as operational *** fiber reinforced tungsten（W_f/W） composites overcome these issues by using extrinsic toughening *** to neutron irradiation and high-temperature recrystallization during fusion operation,the ductility of the fibers may degrade over a long period of ***,it is necessary to investigate the fracture behavior of W_f/W composites with brittle *** the present work,the tungsten fibers were embrittled by ***,the continuous brittle W_f/W composite with yttrium oxide（Y2O3） interface was fabricated by the field-assisted sintering technology *** microstructure of the prepared W_f/W composites was *** fracture behavior and toughening mechanisms were discussed in detail based on the experimental results of 3-point bending tests and the corresponding finite element *** composites show a pseudo-ductile fracture *** are hindered and deflected by the de-bonded fiber-matrix *** extrinsic toughening mechanisms of interface de-bonding,crack bridging,and fiber pull-out are *** indicates the reinforcement concept still works even though all components in the W_f/W are brittle.

关键词： Field-assisted sintering technology (FAST) Fracture behavior Interface Numerical simulation Wf/W composites

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Ghost species form an important component of the epiphytic lichens in temperate forests

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Forest Ecosystems 2025年第01期 20-28页

作者： jan Vondrák Ji?í Ko?nar Stanislav Svoboda Zdeněk Palice Jaroslav ?oun Ji?í Kubásek Pavel Ríha Ji?í Malí?ek jan Rydlo Jeňyk Hofmeister Institute of Botany of the Czech Academy of Sciences Department of Botany Faculty of ScienceUniversity of South Bohemia Museum of Dr.Bohuslav Horák in Rokycany Branch of Museum of West Bohemia Department of Experimental Plant Biology Faculty of ScienceUniversity of South Bohemia ReDpeupbalritcment of Forest Ecology Faculty of Forestry and Wood SciencesCzech University of Life Sciences in Prague Global Change Research Institute of the Czech Academy of Sciences

Sequencing of environmental samples has great potential for biodiversity research, but its application is limited by the lack of reliable DNA barcode databases for species identifications. Such a database has been created for epiphytic lichens of Europe, allowing us to compare the results of environmental sequencing with standard taxonomic surveys. The species undetected by taxonomic surveys(what we term the ghost component) amount to about half of the species actually present in hectare plots of Central European forests. Some of these, which currently occur only as diaspores or weakly developed thalli, are likely to be favoured in the course of global change. The ghost component usually represents a larger fraction in managed forests than in old-growth unmanaged forests. The total species composition of different plots is much more similar than suggested by taxonomic surveys alone. On a regional scale, this supports the well-known statement that“everything is everywhere,but, the environment selects”.

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A Genetic Algorithm Approach for Location-Specific Calibration of Rainfed Maize Cropping in the Context of Smallholder Farming in West Africa

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Agricultural Sciences 2025年第1期16卷 89-111页

作者： Moussa Waongo Patrick Laux jan Bliefernicht Amadou Coulibaly Seydou B. Traore Training and Research Department Agrhymet Reional Centre Niamey Niger Karlsruhe Institute of Technology (KIT) Campus Alpin Garmisch-Partenkirchen Germany Institute of Geography University of Augsburg Augsburg Germany

Smallholder farming in West Africa faces various challenges, such as limited access to seeds, fertilizers, modern mechanization, and agricultural climate services. Crop productivity obtained under these conditions varies significantly from one farmer to another, making it challenging to accurately estimate crop production through crop models. This limitation has implications for the reliability of using crop models as agricultural decision-making support tools. To support decision making in agriculture, an approach combining a genetic algorithm (GA) with the crop model AquaCrop is proposed for a location-specific calibration of maize cropping. In this approach, AquaCrop is used to simulate maize crop yield while the GA is used to derive optimal parameters set at grid cell resolution from various combinations of cultivar parameters and crop management in the process of crop and management options calibration. Statistics on pairwise simulated and observed yields indicate that the coefficient of determination varies from 0.20 to 0.65, with a yield deviation ranging from 8% to 36% across Burkina Faso (BF). An analysis of the optimal parameter sets shows that regardless of the climatic zone, a base temperature of 10˚C and an upper temperature of 32˚C is observed in at least 50% of grid cells. The growing season length and the harvest index vary significantly across BF, with the highest values found in the Soudanian zone and the lowest values in the Sahelian zone. Regarding management strategies, the fertility mean rate is approximately 35%, 39%, and 49% for the Sahelian, Soudano-sahelian, and Soudanian zones, respectively. The mean weed cover is around 36%, with the Sahelian and Soudano-sahelian zones showing the highest variability. The proposed approach can be an alternative to the conventional one-size-fits-all approach commonly used for regional crop modeling. Moreover, it has the potential to explore the performance of cropping strategies to adapt to changing climat

关键词： Smallholder Farming AquaCrop Genetics Algorithm Optimization Maize Burkina Faso

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The role of zonal flows in reactive fluid closures

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Plasma Science and Technology 2018年第7期20卷 41-49页

作者： jan weiland Chalmers University of Technology and EURATOM-VR Association SE-41296 GothenburgSweden

We will give an overview of results obtained by our reactive fluid model. It is characterised as a fluid model where all moments with sources in the experiment are kept. Furthermore, full account is taken for the highest moments appearing in unexpanded denominators also including full toroidicity. It has been demonstrated that the strength of zonal flows is dramatically larger in reactive fluid closures than in those which involve dissipation. This gives a direct connection between the fluid closure and the level of excitation of turbulence. This is because zonal flows are needed to absorb the inverse cascade in quasi 2D turbulence. This also explains the similarity in structure of the transport coefficients in our model with a reactive closure in the energy equation and models which have a reactive closure because of zero ion temperature such as the Hasegawa-Wakatani model. Our exact reactive closure unifies several well-known features of tokamak experiments such as the L-H transition, internal transport barriers and the nonlinear Dimits upshift of the critical gradient for onset of transport. It also gives transport of the same level as that in nonlinear gyrokinetic codes. Since these include the kinetic resonance this confirms the validity of the thermodynamic properties of our model. Furthermore, we can show that while a strongly nonlinear model is needed in kinetic theory a quasilinear model is sufficient in the fluid description. Thus our quasilinear fluid model will be adequate for treating all relevant problems in bulk transport. This is finally confirmed by the reproduction by the model of the experimental power scaling of the confinement time Te ~ P-2/3. This confirms the validity of our reactive fluid model. This also gives credibility to our ITER simulations including the H-mode barrier. A new result is here, that alpha heating strongly reduces the slope of the H-mode barrier. This should significantly reduce the effects of ELM＇s.

关键词： turbulent transport improved mixing length estimates zonal flows toroidicity

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Comparative study of electrostatic sensors with circular and probe electrodes for velocity measurement of pulverised coal

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仪器仪表学报 2007年第11期28卷 1921-1926页

作者： Krabicka jan Department of Electronics University of Kent

This paper presents recent progress on the velocity measurement of pulverised coal in pneumatic pipelines using electrostatic sensors in combination with correlation signal processing techniques.A comparative study of electrostatic sensors with circular and probe electrodes was conducted on a 94 mm bore horizontal pipeline in a 4 MW *** advantages and limitations of both sensors are *** results demonstrate that both sensors are capable of providing pulverised coal velocity measurement with excellent repeatability and dynamic response.

关键词：电话无线电电子仪器技术性能

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Grain Size Distribution of Soils within the Cordillera Blanca, Peru: An Indicator of Basic Mechanical Properties for Slope Stability Evaluation

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Journal of Mountain Science 2014年第3期11卷 563-577页

作者： jan NOVOTNY jan KLIME ARCADIS CZ a.s. Institute of Hydrogeology Engineering Geology and Applied Geophysics Charles University in Prague Albertov Department of Engineering Geology Institute of Rock Structure and Mechanics Academy of Sciences of the Czech Republic v.v.i

This paper presents results of a study on the mechanical properties of sandy and gravely soils within the Cordillera Blanca, Peru. The soils were divided into groups according to their origin(glacial, fluvial, or debris flow). The grain size distribution of forty three soil samples was used to classify the soils according to the scheme of the Unified Soil Classification System(USCS). These distributions have then been used to estimate shear strength and hydraulic properties of the soils. There are clear differences between the soils which reflect their divergent origins. The glacial soils normally fit within one of two distinctive groups according to the proportion of fines(Group A, 7%-21.5%; Group B, 21%-65%). The estimation of shear strength at constant volume friction angle and peak shear strength of the glacial sediments with low content of fines was made using published data relating to the measured shear strength characteristics of soils with similar origins and grain size distributions. The estimated values were supported by measurements of the angle of repose taken from fourteen samples from two moraines and by shear tests on samples from one locality. The results of the grain size distribution werealso used to estimate the average hydraulic conductivity using the empirical Hazen formula which results were verified by field infiltration tests at two localities.

关键词： Moraines Grain size distribution Shearstrength Hydraulic conductivity Cordillera Blanca Peru

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