The isothermal single-component multi-phase lattice Boltzmann method(LBM)combined with the particle motion model is used to simulate the detailed process of liquid film rupture induced by a single spherical particle.T...The isothermal single-component multi-phase lattice Boltzmann method(LBM)combined with the particle motion model is used to simulate the detailed process of liquid film rupture induced by a single spherical particle.The entire process of the liquid film rupture can be divided into two stages.In Stage 1,the particle contacts with the liquid film and moves into it due to the interfacial force and finally penetrates the liquid film.Then in Stage 2,the upper and lower liquid surfaces of the thin film are driven by the capillary force and approach to each other along the surface of the particle,resulting in a complete rupture.It is found that a hydrophobic particle with a contact angle of 106.7°shows the shortest rupture duration when the liquid film thickness is less than the particle radius.When the thickness of the liquid film is greater than the immersed depth of the particle at equilibrium,the time of liquid film rupture caused by a hydrophobic particle will be increased.On the other hand,a moderately hydrophilic particle can form a bridge in the middle of the liquid film to enhance the stability of the thin liquid film.展开更多
The effects of the acid site in main channels of MOR zeolites on their product selectivity and deactivation in the MTO(methanol to olefin) reactions were investigated. The catalytic analysis demonstrates that the py...The effects of the acid site in main channels of MOR zeolites on their product selectivity and deactivation in the MTO(methanol to olefin) reactions were investigated. The catalytic analysis demonstrates that the pyridine modified MOR zeolite yielded high selectivity(> 65.3%) of C;-C;, although the conversion dropped from 100% to 54%. Furthermore, both the catalytic lifetime of MOR and the stability of yielding the lower olefins were increased from less than 30 min to more than 120 min after the modification with pyridine.1H MAS NMR on MOR and modified MOR shows that the acid sites in main channel do not benefit the productivity of lower olefins and catalysts’ lifetime. It can be concluded from ex-situ;C CP MAS NMR that the deposit species during the MTO reaction depend on the pore sizes, and the formation of large alkyl aromatic species more likely occurs in the 12-ring main channels rather than the 8-ring side pocket.展开更多
CGG repeat expansions in LOC642361/NUTM2B-AS1 have recently been identified as a cause of oculopharyngeal myopathy with leukoencephalopathy.However,since only three patients from a single family were reported,it remai...CGG repeat expansions in LOC642361/NUTM2B-AS1 have recently been identified as a cause of oculopharyngeal myopathy with leukoencephalopathy.However,since only three patients from a single family were reported,it remains unknown whether their clinicopathological features are typical for CGG repeat expansions in LOC642361/NUTM2B-AS1.Here,using repeat-primed-polymerase chain reaction and long-read sequencing,we identify 12 individuals from 3 unrelated families with CGG repeat expansions in LOC642361/NUTM2B-AS1,typically presenting with oculopharyngodistal myopathy.The CGG repeat expansions range from 161 to 669 repeat units.Most of the patients present with ptosis,restricted eye movements,dysphagia,dysarthria,and diffuse limb muscle weakness.Only one patient shows T2-weighted hyperintensity in the cerebellar white matter surrounding the deep cerebellar nuclei on brain magnetic resonance imaging.Muscle biopsies from three patients show a myopathic pattern and rimmed vacuoles.Analyses of muscle biopsies suggest that CGG repeat expansions in LOC642361/NUTM2B-AS1 may deleteriously affect aggrephagic capacity,suggesting that RNA toxicity and mitochondrial dysfunction may contribute to pathogenesis.Our study thus expands the phenotypic spectrum for the CGG repeat expansion of LOC642361/NUTM2B-AS1 and indicates that this genetic variant typically manifests as oculopharyngodistal myopathy with chronic myopathic changes with rimmed vacuoles and filamentous intranuclear inclusions in muscle fibers.展开更多
Duchenne muscular dystrophy(DMD)is a severe neuromuscular disorder caused by mutations in the dystrophin gene,which encodes the essential protein dystrophin.This genetic condition,affecting approximately 1 in 5000 mal...Duchenne muscular dystrophy(DMD)is a severe neuromuscular disorder caused by mutations in the dystrophin gene,which encodes the essential protein dystrophin.This genetic condition,affecting approximately 1 in 5000 male births worldwide(Birnkrant et al.,2018),is characterized by progressive muscle weakness and wasting,leading to premature death in the second or third decade of life(Farini et al.,2016).The absence of dystrophin protein causes instability in the sarcolemma,which predisposes individuals to myonecrosis and activation of inflammatory signaling cascades.Unfortunately,there is currently no effective cure for DMD,and glucocorticoid steroids are commonly used in clinical settings to delay symptom development(Merlini et al.,2003).展开更多
CRISPR-mediated genome editing is a revolutionary technology for genome manipulation that uses the CRISPR-Cas systems and base editors.Currently,poor efficiency and off-target problems have impeded the application of ...CRISPR-mediated genome editing is a revolutionary technology for genome manipulation that uses the CRISPR-Cas systems and base editors.Currently,poor efficiency and off-target problems have impeded the application of CRISPR systems.The on-target efficiency has been improved in several advanced versions of CRISPR systems,whereas the off-target detection still remains a key challenge.Here,we outline the different versions of CRISPR systems and off-target detection strategies,discuss the merits and limitations of off-target detection methods,and provide potential implications for further gene editing research.展开更多
Solar,terrestrial,and supernova neutrino experiments are subject to muon-induced radioactive background.The China Jinping Underground Laboratory(CJPL),with its unique advantage of a 2400 m rock coverage and long dista...Solar,terrestrial,and supernova neutrino experiments are subject to muon-induced radioactive background.The China Jinping Underground Laboratory(CJPL),with its unique advantage of a 2400 m rock coverage and long distance from nuclear power plants,is ideal for MeV-scale neutrino experiments.Using a 1-ton prototype detector of the Jinping Neutrino Experiment(JNE),we detected 3 43 high-energy cosmic-ray muons and(7.86±3.97)muon-induced neutrons from an 820.28-day dataset at the first phase of CJPL(CJPL-I).Based on the muon-induced neutrons,we measured the corresponding muon-induced neutron yield in a liquid scintillator to be(3.44±1.86_(stat.)±0.76_(syst.))×10^(-4) μ^(-1)g^(-1)cm^(2) at an average muon energy of 340 GeV.We provided the first study for such neutron background at CJPL.A global fit including this measurement shows a power-law coefficient of(0.75±0.02) for the dependence of the neutron yield at the liquid scintillator on muon energy.展开更多
基金supported by the National Key Technologies Research and Development(R&D)Program by the Ministry of Science and Technology of China(No.2017YFB0406100)the National Natural Science Foundation of China(No.51676123)。
文摘The isothermal single-component multi-phase lattice Boltzmann method(LBM)combined with the particle motion model is used to simulate the detailed process of liquid film rupture induced by a single spherical particle.The entire process of the liquid film rupture can be divided into two stages.In Stage 1,the particle contacts with the liquid film and moves into it due to the interfacial force and finally penetrates the liquid film.Then in Stage 2,the upper and lower liquid surfaces of the thin film are driven by the capillary force and approach to each other along the surface of the particle,resulting in a complete rupture.It is found that a hydrophobic particle with a contact angle of 106.7°shows the shortest rupture duration when the liquid film thickness is less than the particle radius.When the thickness of the liquid film is greater than the immersed depth of the particle at equilibrium,the time of liquid film rupture caused by a hydrophobic particle will be increased.On the other hand,a moderately hydrophilic particle can form a bridge in the middle of the liquid film to enhance the stability of the thin liquid film.
基金supported by the Ministry of Science and Technology of China(2012CB224806)
文摘The effects of the acid site in main channels of MOR zeolites on their product selectivity and deactivation in the MTO(methanol to olefin) reactions were investigated. The catalytic analysis demonstrates that the pyridine modified MOR zeolite yielded high selectivity(> 65.3%) of C;-C;, although the conversion dropped from 100% to 54%. Furthermore, both the catalytic lifetime of MOR and the stability of yielding the lower olefins were increased from less than 30 min to more than 120 min after the modification with pyridine.1H MAS NMR on MOR and modified MOR shows that the acid sites in main channel do not benefit the productivity of lower olefins and catalysts’ lifetime. It can be concluded from ex-situ;C CP MAS NMR that the deposit species during the MTO reaction depend on the pore sizes, and the formation of large alkyl aromatic species more likely occurs in the 12-ring main channels rather than the 8-ring side pocket.
文摘CGG repeat expansions in LOC642361/NUTM2B-AS1 have recently been identified as a cause of oculopharyngeal myopathy with leukoencephalopathy.However,since only three patients from a single family were reported,it remains unknown whether their clinicopathological features are typical for CGG repeat expansions in LOC642361/NUTM2B-AS1.Here,using repeat-primed-polymerase chain reaction and long-read sequencing,we identify 12 individuals from 3 unrelated families with CGG repeat expansions in LOC642361/NUTM2B-AS1,typically presenting with oculopharyngodistal myopathy.The CGG repeat expansions range from 161 to 669 repeat units.Most of the patients present with ptosis,restricted eye movements,dysphagia,dysarthria,and diffuse limb muscle weakness.Only one patient shows T2-weighted hyperintensity in the cerebellar white matter surrounding the deep cerebellar nuclei on brain magnetic resonance imaging.Muscle biopsies from three patients show a myopathic pattern and rimmed vacuoles.Analyses of muscle biopsies suggest that CGG repeat expansions in LOC642361/NUTM2B-AS1 may deleteriously affect aggrephagic capacity,suggesting that RNA toxicity and mitochondrial dysfunction may contribute to pathogenesis.Our study thus expands the phenotypic spectrum for the CGG repeat expansion of LOC642361/NUTM2B-AS1 and indicates that this genetic variant typically manifests as oculopharyngodistal myopathy with chronic myopathic changes with rimmed vacuoles and filamentous intranuclear inclusions in muscle fibers.
基金supported by grants from National Natural Science Foundation of China(82202052,82230039,U2005201,81870902,82001217,31925016,and 82021001)Major Scientific Research Project for Middle-age and Youth of Fujian Provincial Health Commission grant(2021ZQNZD003)+2 种基金Major Scientific Research Project of Fujian Provincial Health Commission grant(2022ZD01002)Joint Funds for the Innovation of Science and Technology of Fujian Province(2018Y9082)the Youth Scientific Research。
文摘Duchenne muscular dystrophy(DMD)is a severe neuromuscular disorder caused by mutations in the dystrophin gene,which encodes the essential protein dystrophin.This genetic condition,affecting approximately 1 in 5000 male births worldwide(Birnkrant et al.,2018),is characterized by progressive muscle weakness and wasting,leading to premature death in the second or third decade of life(Farini et al.,2016).The absence of dystrophin protein causes instability in the sarcolemma,which predisposes individuals to myonecrosis and activation of inflammatory signaling cascades.Unfortunately,there is currently no effective cure for DMD,and glucocorticoid steroids are commonly used in clinical settings to delay symptom development(Merlini et al.,2003).
基金supported by the grants 81771230(W.C.),31922048(E.Z.)and 31522037(H.Y.)from the National Natural Science Foundation of China.
文摘CRISPR-mediated genome editing is a revolutionary technology for genome manipulation that uses the CRISPR-Cas systems and base editors.Currently,poor efficiency and off-target problems have impeded the application of CRISPR systems.The on-target efficiency has been improved in several advanced versions of CRISPR systems,whereas the off-target detection still remains a key challenge.Here,we outline the different versions of CRISPR systems and off-target detection strategies,discuss the merits and limitations of off-target detection methods,and provide potential implications for further gene editing research.
基金Supported in part by the National Natural Science Foundation of China (11620101004,11475093,12127808)the Key Laboratory of Particle&Radiation Imaging(Tsinghua University)+2 种基金the CAS Center for Excellence in Particle Physics (CCEPP)Guangdong Basic and Applied Basic Research Foundation(2019A1515012216)Portion of this work performed at Brookhaven National Laboratory is supported in part by the United States Department of Energy (DESC0012704)。
文摘Solar,terrestrial,and supernova neutrino experiments are subject to muon-induced radioactive background.The China Jinping Underground Laboratory(CJPL),with its unique advantage of a 2400 m rock coverage and long distance from nuclear power plants,is ideal for MeV-scale neutrino experiments.Using a 1-ton prototype detector of the Jinping Neutrino Experiment(JNE),we detected 3 43 high-energy cosmic-ray muons and(7.86±3.97)muon-induced neutrons from an 820.28-day dataset at the first phase of CJPL(CJPL-I).Based on the muon-induced neutrons,we measured the corresponding muon-induced neutron yield in a liquid scintillator to be(3.44±1.86_(stat.)±0.76_(syst.))×10^(-4) μ^(-1)g^(-1)cm^(2) at an average muon energy of 340 GeV.We provided the first study for such neutron background at CJPL.A global fit including this measurement shows a power-law coefficient of(0.75±0.02) for the dependence of the neutron yield at the liquid scintillator on muon energy.