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Exome sequencing identifies a likely causative variant in 53%of families with ciliopathy-related features on renal ultrasound after excluding NpHp1 deletions

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Genes & Diseases 2024年第5期11卷 40-43页

作者： Konstantin Deutsch Verena Klambt Thomas m.Kitzler Tilman Jobst-Schwan Ronen schneider Florian Buerger Steve Seltzsam Sherif El Desoky Jameela A.Kari Farkhanda Hafeez maria Szczepanska Loai A.Eid Hazem S.Awad muna Al-Saffar Neveen A.Soliman Velibor Tasic Camille Nicolas-Frank Kirollos Yousef Luca m.Schierbaum Sophia schneider Abdul Halawi Izzeldin Elmubarak Katharina Lemberg Shirlee Shril Shrikant m.mane Nancy Rodig Friedhelm Hildebrandt Division of Nephrology Department of PediatricsBoston Children’s HospitalHarvard Medical SchoolBostonMA 02215USA Department of pediatric Gastroenterology Nephrology and Metabolic DiseasesCharite Universitatsmedizin BerlinBerlin 13353Germany Berlin Institute of Health at Charitee Universitatsmedizin Berlin BIH Biomedical Innovation AcademyBIH Charite Clinician Scientist ProgramBerlin 10178Germany Department of Nephrology and Hypertension FriedrichAlexander-Universitat Erlangen-NurnbergErlangen 91054Germany Department of pediatrics Faculty of Medicine King Abdulaziz UniversityPediatric Nephrology Center of ExcellenceKing Abdulaziz University HospitalJeddah 21589Saudi Arabia Department of pediatric Nephrology The Children’s Hospital and Institute of Child HealthLahore 54000Pakistan Department of pediatrics Faculty of Medical Sciences in ZabrzeMedical University of Silesia in KatowiceKatowice 40-752Poland Dubai Hospital and Al-Jalila Children’s Specialty Hospital Kidney Center of ExcellenceDubai 4545United Arab Emirates Department of Genetics and Genomics UAE UniversityAbu Dhabi 15551United Arab Emirates Department of pediatrics Division of Genetics and GenomicsBoston Children’s HospitalHarvard Medical SchoolBostonMA 02215USA Department of pediatrics Center of Pediatric Nephrology and TransplantationKasr Al Ainy School of MedicineCairo UniversityCairo 11562Egypt Egypt Center for Research and Regenerative medicine(ECRRm) Cairo 11511Egypt medical Faculty Skopje University Children’s HospitalSkopje 1000North Macedonia Department of Genetics Yale University School of MedicineNew HavenCT 06520USA

Nephronophthisis-related ciliopathies(NpHp-RC)represent one of the most common causes of chronic kidney disease in the first three decades of life and are characterized by a broad genetic and clinical heterogeneity.1 To date,more than 90 genes have been identified that cause autosomalrecessive NpHp-RC if mutated,accounting for up to 60%of cases.1 Among these,homozygous deletions of NpHp1 are the most common *** genes localize to primary cilia,basal bodies,or the centrosome and lead to a primary ciliary disruption if mutated,thereby causing a broad phenotypical spectrum.1 patients that suffer from NpHp-RC can either have an isolated renal phenotype,such as polyuria,polydipsia,decreased urinary concentration ability,and secondary enuresis due to loss of tubular function,or present with extrarenal symptoms including retinal degeneration,cerebellar vermis hypoplasia,or hepatic *** are often diagnosed in early adolescence,reaching end-stage renal disease before 25 years of age,but early onset and rapidly progressive forms of NpHp-RC also exist.1 Renal ultrasound indicates kidneys of normal or reduced renal length with increased echogenicity and corticomedullary cysts.

关键词： degeneration urinary likely

miniSCIDOm: a scintillator-based tomograph for volumetric dose reconstruction of single laser-driven proton bunches

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High power Laser Science and Engineering 2024年第2期12卷 41-50页

作者： A.Corvino m.Reimold E.Beyreuther F.-E.Brack F.Kroll J.pawelke J.D.Schilz m.schneider U.Schramm m.E.p.Umlandt K.Zeil T.Ziegler J.metzkes-Ng Helmholtz-Zentrum Dresden–Rossendorf DresdenGermany Technische Universität Dresden DresdenGermany OncoRay–National Center for Radiation Research in Oncology Faculty of Medicine and University Hospital Carl Gustav CarusTUD Dresden University of TechnologyHelmholtz-Zentrum Dresden–RossendorfDresdenGermany Current affiliation:Institut Curie UniversitéPSLCNRS UMR3347OrsayFrance Current affiliation:Universitätsklinikum Freiburg FreiburgGermany

Laser plasma accelerators(LpAs)enable the generation of intense and short proton bunches on a micrometre scale,thus offering new experimental capabilities to research fields such as ultra-high dose rate radiobiology or material *** spectrally broadband,laser-accelerated proton bunches allow for tailored volumetric dose deposition in a sample via single bunches to excite or probe specific sample *** rising number of such experiments indicates a need for diagnostics providing spatially resolved characterization of dose distributions with volumes of approximately 1 cm^(3) for single proton bunches to allow for fast online *** we present the scintillator-based miniSCIDOm detector for online single-bunch tomographic reconstruction of dose distributions in volumes of up to approximately 1 cm^(3).The detector achieves a spatial resolution below 500μm and a sensitivity of 100 *** detector performance is tested at a proton therapy cyclotron and an LpA proton *** experiments’primary focus is the characterization of the scintillator’s ionization quenching behaviour.

关键词： beam monitoring detectors laser-driven proton beams scintillator-based diagnostics ultra-high dose rate

Demonstrating grating-based phase-contrast imaging of laser-driven shock waves

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matter and Radiation at Extremes 2024年第4期9卷 90-97页

作者： Leonard Wegert Stephan Schreiner Constantin Rauch Bruno Albertazzi paulina Bleuel Eric Frojdh michel Koenig Veronika Ludwig Artem Smartynenko pascal meyer Aldo mozzanica michael müller paul Neumayer markus schneider Angelos Triantafyllidis Bernhard Zielbauer Gisela Anton Thilo michel Stefan Funk plasma physics Department GSI Helmholtzzentrum fur SchwerionenforschungPlanckstraBe 164291 DarmstadtGermany Erlangen Centre for Astroparticle physics(ECAp) Friedrich-Alexander-Universität Erlangen-NurnbergNikolaus-Fiebiger-StraBe 291058 ErlangenGermany LULI-CNRS CEASorbonne UniversitésEcole PolytechniqueInstitut Polytechnique de ParisF-91120 Palaiseau CedexFrance paul Scherrer Institut(psi) Forschungsstrasse 1115232VilligenSwitzerland Karlsruhe Institute of Technology Institute of Microstructure TechnologyHermann-von-Helmholtz-Platz 176344 Eggenstein-LeopoldshafenGermany

Single-shot X-ray phase-contrast imaging is used to take high-resolution images of laser-driven strong shock *** a two-grating Talbot interferometer,we successfully acquire standard absorption,differential phase-contrast,and dark-field images of the shocked *** agreement is demonstrated between experimental data and the results of two-dimensional radiation hydrodynamics simulations of the laser-plasma *** main sources of image noise are identified through a thorough assessment of the interferometer’s *** acquired images demonstrate that grating-based phase-contrast imaging is a powerful diagnostic tool for high-energy-density *** addition,we make a novel attempt at using the dark-field image as a signal modality of Talbot interferometry to identify the microstructure of a foam target.

关键词： interaction shock phase

How completely are randomized controlled trials of non-pharmacological interventions following concussion reported? A systematic review

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Journal of Sport and Health Science 2024年第4期13卷 537-547页

作者： Jacqueline Josee van Ierssel Olivia Galea Kirsten Holte Caroline Luszawski Elizabeth Jenkins Jennifer O’Neil Carolyn A Emery Rebekah mannix Kathryn schneider Keith Owen Yeates Roger Zemek Children’s Hospital of Eastern Ontario Research Institute OttawaON K1H 8L1Canada The Centre for Health Activity and Rehabilitation ResearchUniversity of OtagoDunedin 9016New Zealand Sport Injury prevention Research Centre Faculty of KinesiologyUniversity of CalgaryCalgaryAB T2N 1N4Canada Alberta Children’s Hospital Research Institute University of Calgary CalgaryAB T2N 1N4Canada Hotchkiss Brain Institute University of CalgaryCalgaryAB T2N 4N1Canada Department of psychology University of CalgaryCalgaryAB T2N 1N4Canada g Faculty of MedicineUniversity of OttawaOttawaON K1H 8M5Canada School of Rehabilitation Sciences University of OttawaOttawaON K1H 8M5Canada Bruyere Research Institute OttawaON K1N 5C8Canada Departments of pediatrics and Emergency medicine Harvard Medical SchoolBostonMA 02115USA Departments of pediatrics and Emergency medicine University of OttawaOttawaON K1H 8M5Canada

purpose:The study aimed to examine the reporting completeness of randomized controlled trials(RCTs)of non-pharmacological interventions following ***:We searched mEDLINE,Embase,psycInfo,CINAHL,and Web of Science up to may *** reviewers independently screened studies and assessed reporting completeness using the Template for Intervention Description and Replication(TIDieR),Consensus on Exercise Reporting Template(CERT),and international Consensus on Therapeutic Exercise aNd Training(i-CONTENT)*** information was sought my study authors where reporting was *** of bias(ROB)was assessed with the Cochrane ROB-2 *** examining non-pharmacological interventions following ***:We included 89 RCTs(n=53 high ROB)examining 11 different interventions for concussion:sub-symptom threshold aerobic exercise,cervicovestibular therapy,physical/cognitive rest,vision therapy,education,psychotherapy,hyperbaric oxygen therapy,transcranial magnetic stimulation,blue light therapy,osteopathic manipulation,and head/neck *** scores were:TIDieR 9/12(75%;interquartile range(IQR)=5;range:5-12),CERT 17/19(89%;IQR=2;range:10-19),and i-CONTENT 6/7(86%;IQR=1;range:5-7).percentage of studies completely reporting all items was TIDieR 35%(31/89),CERT 24%(5/21),and i-CONTENT 10%(2/21).Studies were more completely reported after publication of TIDieR(t_(87)=2.08;p=0.04)and CERT(t_(19)=2.72;p=0.01).Reporting completeness was not strongly associated with journal impact factor(TIDieR:rs=0.27;p=0.01;CERT:r_(s)=-0.44;p=0.06;i-CONTENT:r_(s)=-0.17;p=0.48)or ROB(TIDieR:rs=0.11;p=0.31;CERT:rs=0.04;p=0.86;i-CONTENT:rs=0.12;p=0.60).Conclusion:RCTs of non-pharmacological interventions following concussion demonstrate moderate to good reporting completeness,but are often missing key components,particularly modifications,motivational strategies,and qualified *** completeness improved after TIDieR and CERT publication,but p

关键词： Concussion Intervention Randomized controlled trial Systematic review

The plastid Envelope CHLOROpLAST mANGANESE TRANSpORTER1 Is Essential for manganese Homeostasis in Arabidopsis

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molecular plant 2018年第7期11卷 955-969页

作者： marion Eisenhut Natalie Hoecker Sidsel Birkelund Schmidt Rubek merina Basgaran Samantha Flachbart peter Jahns Tabea Eser Stefan Geimer Seren Husted Andreas p.m. Weber Dario Leister Anja schneider Biochemie der pflanzen Cluster of Excellence on Plant Science (CEPLAS) Heinrich-Heine-University Di3sseldorf 40225 Desseldorf Germany molekularbiologie der pflanzen (Botanik) Department Biologie I Ludwig-Maximilians-University Mernchen 82152 Martinsried Germany Department of plant and Environmental Sciences and Copenhagen plant Science Centre (CpSC) Faculty of Science University of Copenhagen 1871 Frederiksberg Denmark Zellbiologie/Elektronenmikroskopie NW I/B1 Universit&t Bayreuth 95447 Bayreuth Germany These authors contributed equally to this article.

The transition metal manganese （mn） is indispensable for photoautotrophic growth since photosystem II （pSII） employs an inorganic mn4CaOs cluster for water splitting. Here, we show that the Arabidopsis membrane protein CHLOROpLAST mANGANESE TRANSpORTER1 （CmT1） is involved in chloroplast mn homeostasis. CmT1 is the closest homolog of the previously characterized thylakoid mn transporter pHOTOSYNTHESIS-AFFECTED mUTANT71 （p.m.1）. In contrast to p.m.1, CmT1 resides at the chloro- plast envelope and is ubiquitously expressed. Nonetheless, like p.m.1, the expression of CmT1 can also alleviate the mn-sensitive phenotype of yeast mutant qomrl. The cmtl mutant is severely suppressed in growth, chloroplast ultrastructure, and pSII activity owing to a decrease in the amounts of pigments and thylakoid membrane proteins. The importance of CmT1 for chloroplast mn homeostasis is demonstratedby the significant reduction in chloroplast mn concentrations in cmtl-1, which exhibited reduced mn binding in pSII complexes. moreover, CmT1 expression is downregulated in mn-surplus conditions. The p.m.1 cmtl-ldouble mutant resembles the cmtl-f single mutant rather than pare71 in most respects. Taken together, our results suggest that CmT1 mediates mn2 uptake into the chloroplast stroma, and that CmT1 and p.m.1 function sequentially in mn delivery to pSII across the chloroplast envelope and the thylakoid membrane.

关键词： manganese transporter envelope chloroplast photosynthesis Arabidopsis

production of composites with high relative permittivity using the spouted bed technique

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particuology 2019年第1期17卷 184-189页

作者： E. Eichner p.-K. Fischer S. Heinrich GA schneider Institute of Solids process Engineering and particle Technology Denickestr1521073 HamburgGermany Institute of Advanced Ceramics Hamburg University of TechnologyDenickestr1521073 HamburgGermany

This contribution presents a new application of the spouted bed technique. The developed production process involves coating fine copper particles with a polymer in a spouted bed plant and then hot pressing the produced granules to form a compact consisting of a copper–polymer composite with a high filling degree. The fabricated composites possess high relative permittivity, which is advantageous for the production of effective capacitors. With increasing filling degree of copper, the relative permittivity of the composites increased, with a maximum relative permittivity of 214 obtained for the composite containing 78.1 vol% copper. Such a high filling degree can be achieved with conductive particles without obtaining a conductive composite by using the spouted bed technology in the production process. The high relative permittivity results from the high filling degree of particles isolated by a polymer coating.

关键词： Spouted bed High relative permittivity Fine particles Coating metal-polymer composite

Growth of the tropical pinus kesiya as influenced by climate and nutrient availability along an elevational gradient

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Journal of plant Ecology 2020年第1期13卷 97-106页

作者： Le Tuan Ho Raimund schneider Frank m.Thomas Department of Geobotany University of TrierFaculty of Regional and Environmental SciencesBehringstraße 2154296 TrierGermany Department of Forest Resources management Faculty of ForestryNong Lam University HCMCThu Duc DistrictHo Chi Minh CityVietnam Department of Soil Science University of TrierFaculty of Regional and Environmental SciencesBehringstraße 2154296 TrierGermany

Aims In contrast to temperate regions,the reasons for growth reductions of individual tree species along elevational gradients in tropical mountain ranges are poorly known,especially for tropical *** aimed at testing whether climatic or edaphic conditions are responsible for the reduced growth of pinus kesiya,a widely distributed pine species of southern and south-eastern Asia,at higher *** We analysed the stem diameter increment and the isotope discrimination against ^(13)C(△^(13)C)in tree rings of *** along an elevational gradient of~900 to~2000 m *** the mountain ranges of South-Central Vietnam,and related growth to△^(13)C and to climatic and edaphic *** findings We found no consistent correlation patterns between the basal area increment(BAI)of the trees and temperature or *** contrast,across the elevational gradient,we obtained significantly negative correlations of BAI with△^(13)C and with the C/N ratios and theδ^(15)N signature of the upper mineral *** was positively correlated with the concentrations of plant-available phosphorus(p_(a))and of“base”cations(calcium,magnesium,potassium)in the *** conclude that lower temperatures at higher elevations exert an indirect effect on tree growth by inducing higher C/N ratios and by reducing the rate of nitrogen(N)and p.m.neralization,which may be further hampered by lower concentrations of“base”cations(upon enhanced leaching by precipitation)and a negative feedback from low availability of mineralized N and p at higher *** results may be transferable to the uppermost growth limit of *** and to other montane regions of the species’occurrence.

关键词： dendroecology pine pointer year soil nutrient stable isotope

Thinking about worry:A systematic review and meta-analysis on the assessment of metacognitions in children and adolescents

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World Journal of psychiatry 2021年第9期11卷 635-658页

作者： Laura m Kocher Kai schneider Hanna Christiansen Department of psychology University of MarburgMarburg 35037Germany Department of Clinical Child and Adolescent psychology University of LandauLandau 76829Germany

BACKGROUND The metacognitive model of generalized anxiety disorder identifies three forms of metacognition:positive metacognitive beliefs about worry(pOS),negative metacognitive beliefs about worry(NEG),and *** this model was originally developed relying on adult samples,it has since been applied to children and youth in different studies,and results mostly support its validity for this *** the roles of pOS,meta-worry,and age-effects do not appear to be fully clarified for children and adolescents yet,an integration of studies on children and adolescents and the metacognitive model is both timely and *** To summarize the current research on relationships,age-effects,and measurements for pOS,NEG,and meta-worry in childhood and *** We carried out a literature search in the electronic databases psycINFO,pubmed,pSYNDEX,and ERIC in 2017 and updated in *** research in German or English language on metacognition was included with child and adolescent samples diagnosed with anxiety disorders or healthy controls if pOS,NEG,or meta-worry were *** were included for meta-analysis if they reported correlations between these metacognitions and anxiety or *** rating for eligibility was done for 20.89% of full-texts with 90.32% *** of bias was assessed with the appraisal tool for cross-sectional studies and consensus rating of appraisal tool for cross-sectional studies for 20.83% of included studies attaining agreement of intraclass correlation=***,correlations between metacognitions,anxiety and worry were calculated with Revman 5.4.1,assuming random-effects ***-regressions with mean age as the covariate were performed via the online tool metamar ***-ID:*** Overall,k=763 records and k=78 additional records were *** those,k=48 studies with 12839 participants were included and of those,k=24 studies were included for *** studie

关键词： metacognition Anxiety Child Adolescent measures meta-analysis

Insights into manganese transport: A matter of phosphorylation

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molecular plant 2022年第3期15卷 385-387页

作者： Anja schneider plant Science Faculty of BiologyLudwig-Maximilians-Universität MünchenMunichGermany

manganese(mn)is an essential element required for the growth of all known living *** plants acquire mn from the rhizosphere,and its translocation into,and distribution within,the plant involves a multitude of transporters(Alejandro et al.,2020).In the aerial part of the green plant,the most prominent reaction that depends on mn is the water-splitting reaction of photosystem II,which employs a tetra-mn cluster located in the thylakoid lumen of ***,no less important reactions take place in mitochondria,where a mn superoxide dismutase acts as a scavenger of reactive oxygen species,and in the Golgi apparatus,where mn-dependent glycosyltransferases are involved in cell wall biosynthesis.

关键词： manganese apparatus splitting