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Refractory very early-onset inflammatory bowel disease associated with cytosolic isoleucyl-tRNA synthetase deficiency:A case report

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World Journal of Gastroenterology 2020年第15期26卷 1841-1846页

作者： Andrew Fagbemi William G Newman Stuart G Tangye Stephen M Hughes Edmund Cheesman peter d arkwright department of Paediatric Gastroenterology Royal Manchester Children’s HospitalManchester M199WLUnited Kingdom department of Medical Genetics Manchester University NHS Foundation TrustManchester M139WLUnited Kingdom Evolution and Genomic Sciences University of ManchesterManchester M139WLUnited Kingdom Garvan Institute of Medical Research DarlinghurstNSW 2010Australia St Vincent’s Clinical School Faculty of MedicineUNSW AustraliaSydneyNSW 2010Australia department of Paediatric Allergy and Immunology Royal Manchester Children’s HospitalManchester M139WLUnited Kingdom department of Paediatric Histopathology St Mary’s HospitalManchester M139WLUnited Kingdom Lydia Becker Institute of Immunology and Inflammation University of ManchesterManchester M139WLUnited Kingdom

BACKGROUNd Aminoacyl tRNA synthetases/ligases(ARSs)are highly conserved enzymes involved in attaching amino acids to tRNA promoting protein *** deficiencies of ARSs localized to the mitochondria classically present with neuropathology,the clinical features of cytosolic ARS deficiencies are more *** have previously been associated with neonatal hepatitis,but never with early-onset inflammatory bowel *** SUMMARY A nine-year-old Bangladeshi boy presented with neonatal liver failure and deranged clotting,transaminitis and *** parents were first *** older brothers and a sister were *** patient suffered from loose stools from early infancy which became more troublesome and persistent from five years old with ten bloody motions a *** endoscopies showed persistent pancolitis,which was refractory to mesalazine,corticosteroids,azathioprine,sirolimus and anti-TNF(adalimumab)therapy,but has improved recently with subcutaneous *** Genome Sequencing revealed a novel pathogenic missense variant(c.290A>G)in the cytosolic isoleucyl-tRNA synthetase gene,leading to an amino acid substitution(***97Gly).Pathogenic variants in other genes associated with inflammatory bowel disease(IBd)(AdAM17,EGFR,FOXP3,IL10RA,IL10RB,IL21R,NCF4,STAT3)were *** assays demonstrated markedly elevated IL-2,IL-5,IL-13,IL-9 and IL-10 by the patient’s Cd4+T-cells,while IL-17A,IL-17F,IFNβwere lower,and TNFαnot significantly different when compared to healthy *** This case report provides evidence that recessive mutations in cytosolic isoleucyltRNA synthetase are a novel monogenic cause of IBd,which should be considered,particularly in infants and children with a history of neonatal hepatitis and very early-onset IBd poorly responsive to treatment.

关键词： Inflammatory bowel disease Hepatitis Gene Cytosolic isoleucine tRNA synthase

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A product version of the Hilton-Milner-Frankl theorem

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Science China Mathematics 2024年第2期67卷 455-474页

作者： peter Frankl Jian Wang Alfréd Rényi Institute of Mathematics Hungarian Academy of SciencesBudapest H-1053Hungary department of Mathematics Taiyuan University of TechnologyTaiyuan 030024China

Two families F and G of k-subsets of {1, 2,..., n} are called non-trivial cross t-intersecting if|F ∩ G|≥t for all F ∈ F, G ∈ G and | ∩ {F : F ∈ F}| < t, | ∩ {G : G ∈ G}| < t. In the present paper,we determine... 详细信息

Two families F and G of k-subsets of {1, 2,..., n} are called non-trivial cross t-intersecting if|F ∩ G|≥t for all F ∈ F, G ∈ G and | ∩ {F : F ∈ F}| determine the maximum product of the sizes of two non-trivial cross t-intersecting families of k-subsets of{1, 2,..., n} for n≥4(t + 2)2k2, k≥5, which is a product version of the Hilton-Milner-Frankl theorem.

关键词： extremal set theory cross t-intersecting family product version non-trivial

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Benchmarking visual SLAM methods in mirror environments

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Computational Visual Media 2024年第2期10卷 215-241页

作者： peter Herbert Jing Wu Ze Ji Yu-Kun Lai School of Computer Science and Informatics Cardiff UniversityAbacws BuildingSenghennydd RdCardiff CF244AGUK School of Engineering Cardiff UniversityQueen’s BuildingsThe ParadeCardiff CF243AAUK

Visual simultaneous localisation and mapping(vSLAM)finds applications for indoor and outdoor navigation that routinely subjects it to visual complexities,particularly mirror *** effect of mirror presence(time visible and its average size in the frame)was hypothesised to impact localisation and mapping performance,with systems using direct techniques expected to perform ***,a dataset,MirrEnv,of image sequences recorded in mirror environments,was collected,and used to evaluate the performance of existing representative *** ORB-SLAM3 and BundleFusion appear to show moderate degradation of absolute trajectory error with increasing mirror duration,whilst the remaining results did not show significantly degraded localisation *** mesh maps generated proved to be very inaccurate,with real and virtual reflections colliding in the reconstructions.A discussion is given of the likely sources of error and robustness in mirror environments,outlining future directions for validating and improving vSLAM performance in the presence of planar *** MirrEnv dataset is available at https://***/10.17035/d.2023.0292477898.

关键词： visual simultaneous localisation and mapping(vSLAM) mirror localisation mapping reflection datase

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Multiple sclerosis drug repurposing for neuroregeneration

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Neural Regeneration Research 2024年第3期19卷 507-508页

作者： peter Göttle Michael dietrich Patrick Küry department of Neurology Medical FacultyHeinrich-Heine UniversityDüsseldorfGermany

Multiple sclerosis(MS)is an autoimmune disorder of the central nervous system(CNS)and is primarily characterized by immune cell infiltration leading to relapses followed by remission phases and a disease course turning progressive over time with neurodegenerative processes taking over(Amin and Hersh,2023).Of note,beyond relapse-associated worsening early in disease progression independent of relapse activity may arise independently of relapse activity and can occur in all ***-mediated damage of myelin sheaths and the subsequent loss of mature oligodendrocytes are resulting in impaired axonal integrity,neurodegeneration and accounts for irreversible neuronal damage(Kuhlmann et al.,2023).The current landscape of available disease-modifying therapies comprises mainly immunomodulatory drugs that effectively diminish relapses and slow down progression at the onset form of the disease,namely relapsing MS(RMS).In this regard,a number of drugs have been approved as disease-modifying therapies for MS by US Food and drug Administration and European Medicines Agencies(Box 1).

关键词： drugs impaired irreversible

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Breakdown of a Commonly Practiced Technique in Quantum Mechanics

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Journal of Applied Mathematics and Physics 2024年第5期12卷 1919-1929页

作者： Pirooz Mohazzabi William d. Parker peter Kveton department of Mathematics and Physics University of Wisconsin-Parkside Kenosha USA

The dynamically shifted oscillator is investigated quantum mechanically, both analytically and numerically. It is shown that the commonly used method of solving the Schrödinger equation using power series results in i... 详细信息

关键词： dynamic Shift Oscillator Quantum Schrödinger Solution

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Assessment of molecular markers and marker-assisted selection for drought tolerance in barley(Hordeum vulgare L.)

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Journal of Integrative Agriculture 2024年第1期23卷 20-38页

作者： Akmaral Baidyussen Gulmira Khassanova Maral Utebayev Satyvaldy Jatayev Rystay Kushanova Sholpan Khalbayeva Aigul Amangeldiyeva Raushan Yerzhebayeva KulpashBulatova Carly Schramm peter Anderson Colin L.d.Jenkins Kathleen LSoole Yuri Shavrukov Faculty of Agronomy S.Seifullin Kazakh AgroTechnical Research UniversityAstana 010000Kazakhstan A.I.Barayev Research and Production Centre of Grain Farming Shortandy 021601Kazakhstan Kazakh Research Institute of Agriculture and Plant Growing AlmalybakAlmaty District 040909Kazakhstan College of Science and Engineering Biological SciencesFlinders UniversityAdelaideSA 5042Australia

This review updates the present status of the field of molecular markers and marker-assisted selection(MAS),using the example of drought tolerance in *** accuracy of selected quantitative trait loci(QTLs),candidate genes and suggested markers was assessed in the barley genome *** common strategies are described for molecular marker development,candidate gene identification and verification,and their possible applications in MAS to improve the grain yield and yield components in barley under drought *** strategies are based on the following five principles:(1)Molecular markers are designated as genomic‘tags’,and their‘prediction’is strongly dependent on their distance from a candidate gene on genetic or physical maps;(2)plants react differently under favourable and stressful conditions or depending on their stage of development;(3)each candidate gene must be verified by confirming its expression in the relevant conditions,e.g.,drought;(4)the molecular marker identified must be validated for MAS for tolerance to drought stress and improved grain yield;and(5)the small number of molecular markers realized for MAS in breeding,from among the many studies targeting candidate genes,can be explained by the complex nature of drought stress,and multiple stress-responsive genes in each barley genotype that are expressed differentially depending on many other factors.

关键词： barley candidate genes drought tolerance gene verification via expression grain yield marker-assisted selection(MAS) molecular markers quantitative trait loci(QTLs) strategy for MAS

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Investigation of WZ Sge-type dwarf Nova ASASSN-19oc: Optical Spectroscopy and Multicolor Light Curve Analysis

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Research in Astronomy and Astrophysics 2024年第8期24卷 22-33页

作者： Viktoriia Krushevska Sergey Shugarov Paolo Ochner Yuliana Kuznyetsova Mykola Petrov peter Kroll Astronomical Institute of the Slovak Academy of Sciences Main Astronomical Observatory of NAS of Ukraine INAF—Osservatorio Astronomico di Padova department of Physics and Astronomy University of Padova Max Planck Computing and data Facility (MPCdF) Sonneberg Observatory

In this study,we present an investigation of the newly discovered dwarf nova ASASSN-19oc during its superoutburst on 2019 June *** carried out detailed UBVRcIc-photometric observations and also obtained a spectrum on day 7 of the outburst,which shows the presence of hydrogen absorption lines commonly found in dwarf nova *** of photometric data reveals the occurrence of early superhumps in the initial days of observations,followed by ordinary and late *** have accurately calculated the period of the ordinary superhumps as Pord=0.05681（10） days and determined the periods at different stages,as well as the rate of change of the superhump period (Pdot=（5）P/P=8.1×10-5).Additionally,we have derived the mass ratio of the components （q=0.09）,and estimated the color temperature during the outburst as～11,000 K,the distance to the system （d=560 pc） and absolute magnitude of the system in outburst （MV=5.3）.We have shown that outbursts of this star are very rare:based on brightness measurements on 600 archival photographic plates,we found only one outburst that occurred in *** fact,as well as the properties listed above,convincingly shows that the variable ASASSN-19oc is a dwarf nova of WZ Sge type.

关键词： (stars:) binaries (including multiple): close (stars:) novae cataclysmic variables stars: dwarf novae

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Hepatic angiotensin-converting enzyme 2 expression in metabolic dysfunction-associated steatotic liver disease and in patients with fatal COVId-19

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World Journal of Gastroenterology 2024年第31期30卷 3705-3716页

作者： Angus K Jacobs Steven d Morley Kay Samuel Katie Morgan Lyndsey Boswell Timothy J Kendall david A dorward Jonathan A Fallowfield peter C Hayes John N Plevris Hepatology Laboratory University of EdinburghEdinburgh EH164SBUnited Kingdom Scottish National Blood Transfusion Service Jack Copland CentreEdinburgh EH144BEUnited Kingdom Institute for Regeneration and Repair University of EdinburghEdinburgh EH164UUUnited Kingdom Edinburgh Pathology University of EdinburghEdinburgh EH164SBUnited Kingdom

BACKGROUNd Metabolic dysfunction-associated steatotic liver disease(MASLd),characterised by hepatic lipid accumulation,causes inflammation and oxidative stress accompanied by cell damage and *** injury(LI)is also frequently reported in patients hospitalised with coronavirus disease 2019(COVId-19),while preexisting MASLd increases the risk of LI and the development of COVId-19-associated *** of injury at the cellular level remain unclear,but it may be significant that severe acute respiratory syndrome coronavirus 2(SARS-CoV-2)which causes COVId-19,uses angiotensin-converting expression enzyme 2(ACE2),a key regulator of the‘anti-inflammatory’arm of the renin-angiotensin system,for viral attachment and host cell *** To determine if hepatic ACE2 levels are altered during progression of MASLd and in patients who died with severe *** ACE2 protein levels and localisation,and histological fibrosis and lipid droplet accumulation as markers of MASLd were determined in formalin-fixed liver tissue sections across the MASLd pathological spectrum(isolated hepatocellular steatosis,metabolic dysfunction-associated steatohepatitis(MASH)+/-fibrosis,end-stage cirrhosis)and in post-mortem tissues from patients who had died with severe COVId-19,using ACE2 immunohistochemistry and haematoxylin and eosin and picrosirius red staining of total collagen and lipid droplet areas,followed by quantification using machine learning-based image pixel *** ACE2 staining is primarily intracellular and concentrated in the cytoplasm of centrilobular hepatocytes and apical membranes of bile duct ***,ACE2 protein levels are elevated in non-fibrotic MASH compared to healthy controls but not in the progression to MASH with fibrosis and in ***2 protein levels and histological fibrosis are not associated,but ACE2 and liver lipid droplet content are significantly correlated across the MASLd *** A

关键词： Metabolic dysfunction-associated steatotic liver disease Angiotensin-converting enzyme 2 Immunohistochemistry COVId-19 COVId-19-associated cholangiopathy

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Adaptation in brain structure and respiratory and olfactory structures across environmental gradients in African and North American muroid rodents

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Integrative Zoology 2024年第1期19卷 165-181页

作者： peter J.TAYLOR Aluwani NENGOVHELA Christiane dENYS Graham R.SCOTT Catherine M.IVY department of Zoology School of Natural and Mathematical SciencesUniversity of VendaThohoyandouSouth Africa Afromontane Unit Department of Zoology and EntomologyUniversity of the Free StatePhuthaditjhabaSouth Africa department of Mammalogy National MuseumBloemfonteinSouth Africa Institut de Systématique EvolutionBiodiversité(ISYEB)Muséum National d’Histoire NaturelleCNRSSorbonne UniversitéEPHEUniversitéDes AntillesParisFrance department of Biology McMaster UniversityHamiltonOntarioCanada Guglielmo and Shoemaker Labs Advanced Facility for Avian ResearchUniversity of Western OntarioLondonOntarioCanada

Morphometric studies of 3d micro CT-scanned images can provide insights into the evolution of the brain and sensory structures but such data are still scarce for the most diverse mammalian order of *** reviewed and new data,we tested for convergence to extreme aridity and high elevation in the sensory and brain morphology of rodents,from morphometric data from micro-CT X-ray scans of 174 crania of 16 species of three distantly re-lated African murid(soft-furred mice,Praomyini,laminate-toothed rats,Otomyini,and gerbils,Gerbillinae)clades and one North American cricetid(deer mice and white-footed mice,Peromyscus)*** studies demon-strated convergent evolution acting on the oval window area of the cochlea(enlarged in extremely arid-adapted species of Otomyini and Gerbillinae)and on endocranial volume(reduced in high elevation taxa of Otomyini and Peromyscus).However,contrary to our predictions,we did notfind evidence of convergence in brain struc-ture to aridity,or in the olfactory/respiratory system(turbinate bones)to high *** structure differed,particularly in the petrosal lobules of the cerebellum and the olfactory bulbs,between Otomyini and Gerbillinae,with extreme arid-adapted species in each clade being highly divergent(not convergent)from other species in the same *** observed greater“packing”of the maxillary turbinate bones,which have important respiratory functions,in Peromyscus mice from high and low elevations compared to the high-elevation African Praomyini,but more complex patterns within Peromyscus,probably related to trade-offs in respiratory physiology and heat exchange in the nasal epithelium associated with high-elevation adaptation.

关键词： 3d morphometrics Africa brain size North America Rodentia sensory evolution

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Wear-resistant CoCrNi multi-principal element alloy at cryogenic temperature

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Science Bulletin 2024年第2期69卷 227-236页

作者： Yue Ren Qing Zhou dongpeng Hua Zhuobin Huang Yulong Li Qian Jia peter Gumbsch Christian Greiner Haifeng Wang Weimin Liu State Key Laboratory of Solidification Processing Center of Advanced Lubrication and Seal MaterialsNorthwestern Polytechnical UniversityXi’an 710072China Institute for Applied Materials Karlsruhe Institute of TechnologyKarlsruhe 76131Germany IAM-ZM MicroTribology CenterμTC Karlsruhe Institute of TechnologyKarlsruhe 76131Germany Fraunhofer IWM Freiburg 79108Germany State Key Laboratory of Solid Lubrication Lanzhou Institute of Chemical PhysicsChinese Academy of SciencesLanzhou 730000China

Traditional high strength engineering alloys suffer from serious surface brittleness and inferior wear performance when servicing under sliding contact at cryogenic ***,we report that the recently emerging CoCrNi multi-principal element alloy defies this trend and presents dramatically enhanced wear resistance when temperature decreases from 273 to 153 K,surpassing those of cryogenic austenitic *** temperature-dependent structure characteristics and deformation mechanisms influencing the cryogenic wear resistance of CoCrNi are clarified through microscopic observation and atomistic *** is found that sliding-induced subsurface structures show distinct scenarios at different deformation *** cryogenic condition,significant grain refinement and a deep plastic zone give rise to an extended microstructural gradient below the surface,which can accommodate massive sliding deformation,in direct contrast to the strain localization and delamination at 273 ***,the temperature-dependent cryogenic deformation mechanisms(stacking fault networks and phase transformation)also provide additional strengthening and toughening of the subsurface *** features make the CoCrNi alloy particularly wear resistant at cryogenic conditions and an excellent candidate for safety–critical applications.

关键词： Multi-principal element alloy Cryogenic temperature Wear CoCrNi

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