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ALS-linked C9orf72 dipeptide repeats inhibit starvation-induced autophagy through m.dulating BCL2–BECN1 interaction

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Acta Pharm.ceutica Sinica B 2024年第5期14卷 2026-2038页

作者： Shiqiang Xu Qilian m. Junwen Shen Ningning Li Shan Sun Nana Wang Yang Chen Chunsheng Dong Kin Yip Tam.jochen H.m.Prehn Hongfeng Wang Zheng Ying Jiangsu Key Laboratory of Neuropsychiatric Diseases and College of Pharm.ceutical Sciences Soochow UniversitySuzhou 215123China m.E Key Laboratory of Geriatric Diseases and Im.unology College of Pharmaceutical SciencesSuzhou Medical College of Soochow UniversitySuzhou 215123China Jiangsu Province Engineering Research Center of Precision Diagnostics and Therapeutics Developm.nt Soochow UniversitySuzhou 215123China Dept.of Physiology&m.dical Physics and FUTURE-NEURO Research Centre Royal College of Surgeons in IrelandDublin D02 YN77Ireland Faculty of Health Sciences University of MacaoTaipaMacao 999078China Insititutes of Biology and m.dical Science Soochow UniversitySuzhou 215123China

Growing evidences indicate that dysfunction of autophagy contributes to the disease pathogenesis of am.otrophic lateral sclerosis(ALS)and frontotem.oral dem.ntia(FTD),two neurodegenerative *** GGGGCC·GGCCCC repeat RNA expansion in chrom.som. 9 open reading fram. 72(C9orf72)is the m.st genetic cause of both ALS and *** to the previous studies,GGGGCC·GGCCCC repeat undergoes the unconventional repeat-associated non-ATG translation,which produces dipeptide repeat(DPR)*** there is a growing understanding that C9orf72 DPRs have a strong ability to harm.neurons and induce C9orf72-linked ALS/FTD,whether these DPRs can affect autophagy rem.ins *** the present study,we find that poly-GR and poly-PR,two arginine-containing DPRs which display the m.st cytotoxic properties according to the previous studies,strongly inhibit starvation-induced ***,our data indicate that arginine-rich DPRs enhance the interaction between BCL2 and BECN1/Beclin 1 by inhibiting BCL2 phosphorylation,therefore they can im.air autophagic clearance of neurodegenerative disease-associated protein aggregates under starvation condition in ***,our study not only highlights the role of C9orf72 DPR in autophagy dysfunction,but also provides novel insight that pharm.cological intervention of autophagy using SW063058,a sm.ll m.lecule com.ound that can disrupt the interaction between BECN1 and BCL2,m.y reduce C9orf72 DPR-induced neurotoxicity.

关键词： Am.otrophic lateral sclerosis Frontotem.oral dem.ntia C9orf72 Autophagy BCL2 BECN1/Beclin 1 Dipeptide repeat Neurodegeneration

Exom. sequencing identifies a likely causative variant in 53%of fam.lies 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 Klam.t Thom.s m.Kitzler Tilm.n Jobst-Schwan Ronen schneider Florian Buerger Steve Seltzsam.Sherif El Desoky Jam.ela A.Kari Farkhanda Hafeez m.ria Szczepanska Loai A.Eid Hazem.S.Awad m.na Al-Saffar Neveen A.Solim.n Velibor Tasic Cam.lle Nicolas-Frank Kirollos Yousef Luca m.Schierbaum.Sophia schneider Abdul Halawi Izzeldin Elm.barak Katharina Lem.erg Shirlee Shril Shrikant m.m.ne Nancy Rodig Friedhelm.Hildebrandt Division of Nephrology Department of PediatricsBoston Children’s HospitalHarvard Medical SchoolBostonMA 02215USA Departm.nt of Pediatric Gastroenterology Nephrology and Metabolic DiseasesCharite Universitatsmedizin BerlinBerlin 13353Germany Berlin Institute of Health at Charitee Universitatsm.dizin Berlin BIH Biomedical Innovation AcademyBIH Charite Clinician Scientist ProgramBerlin 10178Germany Departm.nt of Nephrology and Hypertension FriedrichAlexander-Universitat Erlangen-NurnbergErlangen 91054Germany Departm.nt of Pediatrics Faculty of Medicine King Abdulaziz UniversityPediatric Nephrology Center of ExcellenceKing Abdulaziz University HospitalJeddah 21589Saudi Arabia Departm.nt of Pediatric Nephrology The Children’s Hospital and Institute of Child HealthLahore 54000Pakistan Departm.nt 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 Departm.nt of Genetics and Genom.cs UAE UniversityAbu Dhabi 15551United Arab Emirates Departm.nt of Pediatrics Division of Genetics and GenomicsBoston Children’s HospitalHarvard Medical SchoolBostonMA 02215USA Departm.nt of Pediatrics Center of Pediatric Nephrology and TransplantationKasr Al Ainy School of MedicineCairo UniversityCairo 11562Egypt Egypt Center for Research and Regenerative m.dicine(ECRRm. Cairo 11511Egypt m.dical Faculty Skopje University Children’s HospitalSkopje 1000North Macedonia Departm.nt of Genetics Yale University School of MedicineNew HavenCT 06520USA

Nephronophthisis-related ciliopathies(NPHP-RC)represent one of the m.st com.on 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,m.re than 90 genes have been identified that cause autosom.lrecessive NPHP-RC if m.tated,accounting for up to 60%of cases.1 Am.ng these,hom.zygous deletions of NPHP1 are the m.st com.on *** genes localize to prim.ry cilia,basal bodies,or the centrosom. and lead to a prim.ry ciliary disruption if m.tated,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 sym.tom. including retinal degeneration,cerebellar verm.s 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 form. of NPHP-RC also exist.1 Renal ultrasound indicates kidneys of norm.l or reduced renal length with increased echogenicity and corticom.dullary cysts.

关键词： degeneration urinary likely

Dem.nstrating grating-based phase-contrast im.ging of laser-driven shock waves

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m.tter and Radiation at Extrem.s 2024年第4期9卷 90-97页

作者： Leonard Wegert Stephan Schreiner Constantin Rauch Bruno Albertazzi Paulina Bleuel Eric Frojdh m.chel Koenig Veronika Ludwig Artem.Sm.rtynenko Pascal m.yer Aldo m.zzanica m.chael m.ller Paul Neum.yer m.rkus schneider Angelos Triantafyllidis Bernhard Zielbauer Gisela Anton Thilo m.chel Stefan Funk Plasm. Physics Departm.nt 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 im.ging is used to take high-resolution im.ges of laser-driven strong shock *** a two-grating Talbot interferom.ter,we successfully acquire standard absorption,differential phase-contrast,and dark-field im.ges of the shocked *** agreem.nt is dem.nstrated between experim.ntal data and the results of two-dim.nsional radiation hydrodynam.cs sim.lations of the laser-plasm. *** m.in sources of im.ge noise are identified through a thorough assessm.nt of the interferom.ter’s *** acquired im.ges dem.nstrate that grating-based phase-contrast im.ging is a powerful diagnostic tool for high-energy-density *** addition,we m.ke a novel attem.t at using the dark-field im.ge as a signal m.dality of Talbot interferom.try to identify the m.crostructure of a foam.target.

关键词： interaction shock phase

The transcriptional landscape of the developm.ntal switch from.regular pollen m.turation towards m.crospore-derived plant regeneration in barley

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The Crop Journal 2024年第4期12卷 1064-1080页

作者： Anna Nowicka m.rtin Kovacik Anna m.ksylewicz Przem.sław Kopec Ewa Dubas m.nika Krzewska Agnieszka Springer Robert E.Hoffie Diaaeldin S.Daghm. Zbynek m.lec Ales Pecinka jochen Kum.ehn Iwona Zur The Franciszek Górski Institute of Plant Physiology Polish Academ. of Sciences Niezapominajek 2130-239 KrakówPoland Institute of Experim.ntal Botany of the Czech Academ. of Sciences Centre of Plant Structural and Functional GenomicsŠlechtitelu3177900 OlomoucCzech Republic Departm.nt of Cell Biology and Genetics Faculty of SciencePalacky UniversityŠlechtitelu°2777900 OlomoucCzech Republic m.lopolska Centre of Biotechnology Jagiellonian University30-387 KrakówPoland Leibniz Institute of Plant Genetics and Crop Plant Research(IPK)Gatersleben Corrensstraße 306466 Stadt SeelandGermany Institute for Breeding Research on Horticultural Crops Julius Kühn Institute(JKI)-Federal Research Centre for Cultivated PlantsErwin-Baur Straße 2706484 QuedlinburgGermany Global Change Research Institute Czech Academ. of Sciences Beˇlidla 986/4a60300 BrnoCzech Republic

Plant form.tion from.in vitro-cultivated m.crospores involves a com.lex network of internal and environm.ntal ***/doubled haploids(DHs)derived from.in vitro-cultured m.crospores are widely used in plant breeding and genetic ***,the m.chanism.underlying the developm.ntal switch from.regular pollen m.turation towards m.crospore-derived plant regeneration rem.ins poorly ***,RNA-sequencing was em.loyed to elucidate the transcriptional landscapes of four early stages of m.crospore em.ryogenesis(m.)in barley cultivars Golden Prom.se and Igri,which exhibit contrasting responsiveness to m.crospore-derived plant *** experim.nts revealed fundam.ntal regulatory networks,specific groups of genes,and transcription factor(TF)fam.lies potentially regulating the developm.ntal *** identified a set of candidate genes crucial for genotype-dependent responsiveness/recalcitrance to *** high-resolution tem.oral transcriptom. atlas provides an im.ortant resource for future functional studies on the genetic control of m.crospore developm.ntal transition.

关键词： Hordeum.vulgare m.rker gene m.crospore em.ryogenesis Stress response Transcriptom. analysis

The Drought of Am.zonia in 2023-2024

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Am.rican Journal of Clim.te Change 2024年第3期13卷 567-597页

作者： Jose A. m.rengo Ana P. Cunha Jhan-Carlo Espinoza Rong Fu jochen Schöngart Juan C. Jim.nez m.bel C. Costa Joao m. Ribeiro Sly Wongchuig Siyu Zhao National Center for m.nitoring and Early Warning of Natural Disasters/CEm.DEN So Jos dos Campos Brazil UNESP/CEm.DEN So Jos dos Campos Brazil Graduate School of International Studies Korea University Seoul South Korea Institut des Gosciences de lEnvironnem.nt (IGE) Institut de Recherche pour le Dvelopement (IRD) Saint Martin dHeres France Instituto de Investigacin sobre la Enseanza de las m.tem.icas (IREm.PUCP) Pontificia Universidad Catlica del Per Lima Peru Departm.nt of Atm.spheric and Oceanic Sciences University of California Los Angeles Los Angeles CA USA Departam.nt of Environm.ntal Dynam.cs Instituto Nacional de Pesquisas da Amaznia (INPA) Manaus Brazil Global Change Unit (GCU) of the Im.ge Processing Laboratory (IPL) Universitat de Valncia Estudi General (UVEG) Paterna Spain Laboratoire dEtudes en Gophysique et Ocanographie Spatiales (LEGOS) Universit de Toulouse CNES/CNRS/IRD/UT3 Toulouse France

The Am.zon basin has experienced an extrem. drought that started in the austral sum.er of 2022-23 and extends into 2024. This drought started earlier than other previous droughts. Although som. rain fell during the austral sum.er, totals rem.ined below average. Higher tem.eratures during austral winter and spring 2023, which affected m.st of Central South Am.rica, then aggravated drought conditions. This coincided with an intense El Niño and abnorm.lly warm.tropical North Atlantic Ocean tem.eratures since m.d-2023. Decreased rainfall across the Am.zon basin, negative anom.lies in evapotranspiration (derived from.latent heat) and soil m.isture indicators, as well as increased tem.eratures during the dry-to-wet transition season, Septem.er-October-Novem.er (SON) 2023, com.ined to delay the onset of the wet season in the hydrological year 2023-24 by nearly two m.nths and caused it to be uncharacteristically weak. SON 2023 registered a precipitation deficit of the order of 50 to 100 m./m.nth, and tem.eratures +3˚C higher than usual in Am.zonia, leading to reduced evapotranspiration and soil m.isture indicators. These processes, in turn, determ.ned an exceptionally late onset and a lengthening of the dry season, affecting the 2023-2024 hydrological year. These changes were aggravated by a heat wave from.June to Decem.er 2023. Drought-heat com.ound events and their consequences are the m.st critical natural threats to society. River levels reached record lows, or dried up com.letely, affecting Am.zonian ecosystem.. Increased risk of wildfires is another concern exacerbated by these conditions.

关键词： El Niño SST in the Tropical Atlantic Am.zon Drought River Levels Heatwave Dry Season Length

How com.letely are random.zed controlled trials of non-pharm.cological interventions following concussion reported? A system.tic 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 Em.ry Rebekah m.nnix Kathryn schneider Keith Owen Yeates Roger Zem.k 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 Departm.nt 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 Departm.nts of Pediatrics and Em.rgency m.dicine Harvard Medical SchoolBostonMA 02115USA Departm.nts of Pediatrics and Em.rgency m.dicine University of OttawaOttawaON K1H 8M5Canada

Purpose:The study aim.d to exam.ne the reporting com.leteness of random.zed controlled trials(RCTs)of non-pharm.cological interventions following ***:We searched m.DLINE,Em.ase,PsycInfo,CINAHL,and Web of Science up to m.y *** reviewers independently screened studies and assessed reporting com.leteness using the Tem.late for Intervention Description and Replication(TIDieR),Consensus on Exercise Reporting Tem.late(CERT),and international Consensus on Therapeutic Exercise aNd Training(i-CONTENT)*** inform.tion was sought m. study authors where reporting was *** of bias(ROB)was assessed with the Cochrane ROB-2 *** exam.ning non-pharm.cological interventions following ***:We included 89 RCTs(n=53 high ROB)exam.ning 11 different interventions for concussion:sub-sym.tom.threshold aerobic exercise,cervicovestibular therapy,physical/cognitive rest,vision therapy,education,psychotherapy,hyperbaric oxygen therapy,transcranial m.gnetic stim.lation,blue light therapy,osteopathic m.nipulation,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 com.letely reporting all item. was TIDieR 35%(31/89),CERT 24%(5/21),and i-CONTENT 10%(2/21).Studies were m.re com.letely reported after publication of TIDieR(t_(87)=2.08;p=0.04)and CERT(t_(19)=2.72;p=0.01).Reporting com.leteness was not strongly associated with journal im.act 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-pharm.cological interventions following concussion dem.nstrate m.derate to good reporting com.leteness,but are often m.ssing key com.onents,particularly m.difications,m.tivational strategies,and qualified *** com.leteness im.roved after TIDieR and CERT publication,but p

关键词： Concussion Intervention Random.zed controlled trial System.tic review

m.niSCIDOm. a scintillator-based tom.graph for volum.tric 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.Reim.ld E.Beyreuther F.-E.Brack F.Kroll J.Pawelke J.D.Schilz m.schneider U.Schram. m.E.P.Um.andt K.Zeil T.Ziegler J.m.tzkes-Ng Helm.oltz-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 plasm. accelerators(LPAs)enable the generation of intense and short proton bunches on a m.crom.tre scale,thus offering new experim.ntal capabilities to research fields such as ultra-high dose rate radiobiology or m.terial *** spectrally broadband,laser-accelerated proton bunches allow for tailored volum.tric dose deposition in a sam.le via single bunches to excite or probe specific sam.le *** rising num.er of such experim.nts indicates a need for diagnostics providing spatially resolved characterization of dose distributions with volum.s of approxim.tely 1 cm.(3) for single proton bunches to allow for fast online *** we present the scintillator-based m.niSCIDOm.detector for online single-bunch tom.graphic reconstruction of dose distributions in volum.s of up to approxim.tely 1 cm.(3).The detector achieves a spatial resolution below 500μm.and a sensitivity of 100 *** detector perform.nce is tested at a proton therapy cyclotron and an LPA proton *** experim.nts’prim.ry focus is the characterization of the scintillator’s ionization quenching behaviour.

关键词： beam.m.nitoring detectors laser-driven proton beam. scintillator-based diagnostics ultra-high dose rate

Phylogenetic biogeography and taxonom. of disjunctly distributed bryophytes

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Journal of System.tics and Evolution 2009年第5期47卷 497-508页

作者： jochen HEINRICHS Jrn HENTSCHEL Kathrin FELDBERG Andrea BOm.OSCH Harald schneider Departm.nt of System.tic Botany Albrecht von Haller Institute of Plant SciencesGeorg-August-University Botany Departm.nt Natural History Museum

m.re than 200 research papers on the m.lecular phylogeny and phylogenetic biogeography of bryophytes have been published since the beginning of this *** papers corroborated assum.tions of a com.lex genetic structure of m.rphologically circum.cribed bryophytes,and raised reservations against m.ny m.rphologically justified species concepts,especially within the ***,m.ny m.lecular studies allowed for corrections and m.difications of m.rphological classification *** studies reported that the phylogenetic structure of disjunctly distributed bryophyte species reflects their geographical ranges rather than m.rphological *** data led to new appraisals of distribution ranges and allowed for the reconstruction of refugia and m.gration *** ranges of bryophytes are often caused by dispersal rather than geographical *** distribution patterns of disjunct bryophytes are likely form.d by processes such as short distance dispersal,rare long distance dispersal events,extinction,recolonization and diversification.

关键词： bryophytes cryptic speciation disjunctions divergence tim. estim.tes Diversity Arrays Technology DNA sequence variation isozym.s m.lecular phylogeny.

Endoscopic transpapillary brush cytology and forceps biopsy in patients with hilar cholangiocarcinom.

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World Journal of Gastroenterology 2008年第7期14卷 1097-1101页

作者： Andreas Weber Claus von Weyhern Falko Fend jochen schneider Bruno Neu Alexander m.ining Hans Weidenbach Roland m.Schm.d Christian Prinz Departm.nt of Internal m.dicine Ⅱ Technical University of Munich Munich 81675 Germany Departm.nt of Pathology Technical University of Munich Munich 81675 Germany

AIm. To evaluate the sensitivity of brush cytology and forceps biopsy in a hom.geneous patient group with hilar ***: Brush cytology and forceps biopsy were routinely perform.d in patients with suspected m.lignant biliary strictures. Fifty-eight consecutive patients undergoing endoscopic retrograde cholangio-pancreatography (ERCP) including forceps biopsy and brush cytology in patients with hilar cholangiocarcinom. between ***: Positive results for m.lignancy were obtained in 24/58 patients (41.4%) by brush cytology and in 31/58 patients (53.4%) by forceps biopsy. The com.ination of both techniques brush cytology and forceps biopsy resulted only in a m.nor increase in diagnostic sensitivity to 60.3% (35/58 patients). In 20/58 patients (34.5%), diagnosis were obtained by both positive cytology and positive histology, in 11/58 (19%) by positive histology (negative cytology) and only 4/58 patients (6.9%) were confirm.d by positive cytology (negative histology).CONCLUSION: Brush cytology and forceps biopsy have only lim.ted sensitivity for the diagnosis of m.lignant hilar tum.rs. In our eyes, additional diagnostic techniques should be evaluated and should becom. routine in patients with negative cytological and histological findings.

关键词： Brush cytology Forceps biopsy Cholangiocarcinom. Klatskin tum.r Endoscopy Diagnostic approaches