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Rare loss-of-function variants in FLNB cause non-syndromic orofacial clefts

Journal of Genetics and Genomics 2024年第2期51卷 222-229页

作者： Wenbin Huang Shiying Zhang Jiuxiang Lin Yi Ding Nan Jiang Jieni Zhang Huaxiang Zhao Feng chen Department of Orthodontics Peking University School and Hospital of StomatologyBeijing100081China Key Laboratory of Shaanxi Province for Craniofacial Precision Medicine Research College of StomatologyXi’an Jiaotong UniversityXi’anShaanxi710004China Guangdong Provincial High-level Clinical Key Specialty Guangdong Province Engineering Research Center of Oral Disease Diagnosis and TreatmentDepartment of OrthodonticsStomatological CenterPeking University Shenzhen HospitalShenzhen Peking University-The Hong Kong University of Science and Technology Medical CenterShenzhenGuangdong 518036China Department of Physiology and Pathophysiology School of Basic Medical SciencesXi'an Jiaotong UniversityXi'anShaanxi710049China National Center for Stomatology National Clinical Research Center for Oral DiseasesNational Engineering Laboratory for Digital and Material Technology of StomatologyBeijing Key Laboratory for Digital StomatologyResearch Center of Engineering and Technology for Computerized Dentistry Ministry of HealthNMPA Key Laboratory for Dental MaterialsBeijing100101China Central Laboratory Peking University School and Hospital of StomatologyBeijing100081China

Orofacial clefts (OFCs) are the most common congenital craniofacial disorders, of which the etiology is closely related to rare coding variants. Filamin B (FLNB) is an actin-binding protein implicated in bone formation. FLNB mutations have been identified in several types of syndromic OFCs and previous studies suggest a role of FLNB in the onset of non-syndromic OFCs (NSOFCs). Here, we report two rare heterozygous variants (p.P441T and p.G565R) in FLNB in two unrelated hereditary families with NSOFCs. Bioinformatics analysis suggests that both variants may disrupt the function of FLNB. In mammalian cells, p.P441T and p.G565R variants are less potent to induce cell stretches than wild type FLNB, suggesting that they are loss-of-function mutations. Immunohistochemistry analysis demonstrates that FLNB is abundantly expressed during palatal development. Importantly, Flnb^(−/−) embryos display cleft palates and previously defined skeletal defects. Taken together, our findings reveal that FLNB is required for development of palates in mice and FLNB is a bona.fide causal gene for NSOFCs in humans.

关键词： Or ofacial clefts FLNB Loss-of-function mutati on Cleft palate Filamin B Actin flannent Knockout mouse

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Pivotal role for long noncoding RNAs in zygotic genome activation in mice

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Science China(Life Sciences) 2024年第5期67卷 958-969页

作者： Kang chen Wenju Liu Jiang Zhu Xiaochen Kou Yanhong Zhao Hong Wang Cizhong Jiang Shaorong Gao Lan Kang Institute for Regenerative Medicine Shanghai East HospitalShanghai Key Laboratory of Signaling and Disease ResearchSchool of Life Sciences and TechnologyTongji UniversityShanghai 200120China Key Laboratory of Spine and Spinal Cord Injury Repair and Regeneration of Ministry of Education Orthopaedic Department of Tongji HospitalShanghai Key Laboratory of Signaling and Disease ResearchSchool of Life Sciences and TechnologyTongji UniversityShanghai 200092China Frontier Science Center for Stem Cell Research Tongji UniversityShanghai 200092China Clinical and Translation Research Center of Shanghai First Maternity&Infant Hospital School of Life Sciences and TechnologyTongji UniversityShanghai 200092China Institute of Biophysics Chinese Academy of SciencesBeijing 100101China University of Chinese Academy of Sciences Beijing 100049China

Vertebrate life begins with fertilization,and then the zygote genome is activated after transient silencing,a process termed zygotic genome activation(ZGA).Despite its fundamental role in totipotency and the initiation of life,the precise mechanism underlying ZGA initiation remains *** existence of minor ZGA implies the possible critical role of noncoding RNAs in the initiation of ***,we delineate the expression profile of long noncoding RNAs(lncRNAs)in early mouse embryonic development and elucidate their critical role in minor *** with protein-coding genes(PCGs),lncRNAs exhibit a stronger correlation with minor *** H3K9me3 profiles can be observed between lncRNA genes and PCGs,and the enrichment of H3K9me3 before ZGA might explain the suspended expression of major ZGA-related PCGs despite possessing PolII ***,we identified the presence of PolII-enriched MuERV-L around the transcriptional start site of minor ZGA-related lncRNAs,and these repeats are responsible for the activation of minor ZGA-related lncRNAs and subsequent embryo *** study suggests that MuERV-L mediates minor ZGA lncRNA activation as a critical driver between epigenetic reprogramming triggered by fertilization and the embryo developmental program,thus providing clues for understanding the regulatory mechanism of totipotency and establishing bona.fide totipotent stem cells.

关键词： ZGA lncRNA MuERV-L

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Numerical comparison of two modes of gas-solid riser operation： Fluid catalytic cracking vs CFB combustor

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Particuology 2017年第2期15卷 42-48页

作者： Yifeng Mei Mingzhao Zhao bona.Lu Sheng chen Wei Wang Stare Key Laboratory afMultiphase Complex Systems Institute of Process Engineering Chinese Academy of Sciences Beijing 100190 China School of Chemical Engineering Sichuan University Chengdu 610065 China Stare Key Laboratory of Heavy Oil Processing China University of Petroleum Beijing 102249 China University of Chinese Academy of Sciences Beijing 100049 China

Two modes of gas-solid riser operation, i.e., fluid catalytic cracking （FCC） and circulating fluidized bed combustor （CFBC）, have been recognized in literature; particularly in the understanding of choking phenomena. This work compares these two modes of operation through computational fluid dynamics （CFD） simulation. In CFD simulations, the different operations are represented by fixing appropriate boundary conditions： solids flux or solids inventory. It is found that the FCC and CFBC modes generally have the same dependence of solids flux on the mean solids volume fraction or solids inventory. However, during the choking transition, the FCC mode of operation needs more time to reach a steady state; thus the FCC system may have insufficient time to respond to valve adjustments or flow state change, leading to the choking. The difference between FCC and CFBC systems is more pronounced for the systems with longer risers. A more detailed investigation of these two modes of riser operation may require a three-dimensional full loop simulation with dynamic valve adjustment.

关键词： Fluidization Computational fluid dynamics Simulation Fluid catalytic cracking Circulating fluidized bed Choking

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有限公司中夫妻股权单方处分的效力认定及其救济

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广东社会科学 2024年第4期 261-272,288页

作者：冉克平陈丹怡武汉大学法学院武汉430072

夫妻股权单方处分问题的核心在于平衡交易安全与婚姻财产安全保护之价值冲突。夫妻股权共有应当限于对股权权属的共有。股权处分权行使主体的认定在公司法或婚姻家庭法下会产生不同的适用结果,为完成两者之间的衔接,需以夫妻共同财产管... 详细信息

夫妻股权单方处分问题的核心在于平衡交易安全与婚姻财产安全保护之价值冲突。夫妻股权共有应当限于对股权权属的共有。股权处分权行使主体的认定在公司法或婚姻家庭法下会产生不同的适用结果,为完成两者之间的衔接,需以夫妻共同财产管理权规则作为制度工具,基于对《民法典》第1060条和1062条的解释重构,建立起以共同管理为原则,各方单独管理和推定一方单独管理为例外的管理权规则。显名方单方处分夫妻股权原则上应被推定为显名方单独管理而构成有权处分,但需受到善意相对人要件的限制。相对人明知显名方无权单独处分或与显名方恶意串通的,以及显名方以不合理的低价或无偿处分股权的,处分行为皆属无效。隐名方对于显名方滥用其推定管理权的单方处分行为,可通过婚内或离婚时的共同财产分割以及侵权制度进行救济。

关键词：夫妻股权股权共有单方处分外观主义管理权规则善意相对人

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Synthesis of chain-like MoS_2 nanoparticles in W/O reverse microemulsion and application in photocatalysis

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Chinese Science Bulletin 2012年第30期57卷 3862-3866页

作者： LIU MeiYing LI XiaoQian XU ZhiLing LI bona.chen LinLin SHAN NanNan Institute of Chemistry.for Functionalized Materials Faculty of Chemistry and Chemical Engineering Liaoning Normal UniversityDalian 116029China

Chain-like MoS2assemblies consisting of hexagonal MoS2nanoparticles(20-60 nm) have been successfully synthesized in a Triton X-100/cyclohexane/hexanol/water W/O reverse microemulsion in the presence of(NH 4)2MoS 4 as the molybdenum source and NH2OH·HCl as the reducing *** products were characterized by X-ray powder diffraction(XRD),transmission electron microscopy(TEM) and UV-vis diffuse reflectance absorption *** influence of synthetic parameters such as acidity,water/oil ratio(0),aging time and annealing temperature on the formation of MoS2assemblies was *** analysis showed that these synthetic factors played important roles in controlling the size of MoS2nanoparticles and the length of the chain-like *** analysis indicated that the well-crystallized MoS2nanoparticles could be obtained by annealing the precursors at 700 C for2h under a flow of *** addition,the as-prepared chain-like MoS2nanoparticles exhibited excellent photocatalytic H2activity in Ru(bpy) 3 2+-MoS2-H2A three-component molecular systems under visible light irradiation.

关键词：微乳液合成二硫化钼纳米粒子反相微乳液光催化 O型链状 X射线粉末衍射

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房屋征收实物补偿下物上代位与登记的衔接

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河南财经政法大学学报 2024年第4期39卷 123-135页

作者：陈佳琳北京大学法学院北京100871

房屋征收实物补偿下的物上代位,属非因法律行为产生的物权变动,抵押权人对置换房所享有的抵押权不受登记生效主义的制约。但权利外观的缺失将招致两重现实风险。一是置换房被后设立且先登记的其他抵押权人优先受偿。法定抵押权的绝对优... 详细信息

房屋征收实物补偿下的物上代位,属非因法律行为产生的物权变动,抵押权人对置换房所享有的抵押权不受登记生效主义的制约。但权利外观的缺失将招致两重现实风险。一是置换房被后设立且先登记的其他抵押权人优先受偿。法定抵押权的绝对优先理论、以成立时间确定顺位理论、物上代位中的顺位保留理论均存缺陷,或无法实质解决问题,或将冲击交易安全。二是发生不动产权利负担的消极善意取得,阻却抵押权的追及效力。据此,有必要在置换房移转后及时衔接抵押登记。通知义务的配置是推进登记的前提,除抵押人外,处于风险控制源头、负有权属调查义务的征收人,也应就产权置换事实对抵押权人承担通知义务。具体规则上,可对置换房抵押权设10日的登记期限,并配置债权法定加速到期规定;或借鉴代位申请制度,由抵押权人直接代位抵押人申请置换房转移登记,同时一并办理抵押登记,以此兼顾物权保护与交易安全。

关键词：物上代位实物补偿抵押权征收登记

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委托代持担保物权的法律效力

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苏州大学学报（法学版） 2024年第2期11卷 123-136页

作者：高圣平陈南成中国人民大学法学院

在特定的交易模式中,担保物权可由他人代为持有。为使金融交易方式更加灵活便捷,应缓和担保物权设立上的从属性,允许为将来债权提供担保,在担保物权可得实现之时,主债权真实存在即可。在权利归属认定中,法院不宜恪守于权利登记内容,在... 详细信息

在特定的交易模式中,担保物权可由他人代为持有。为使金融交易方式更加灵活便捷,应缓和担保物权设立上的从属性,允许为将来债权提供担保,在担保物权可得实现之时,主债权真实存在即可。在权利归属认定中,法院不宜恪守于权利登记内容,在不影响第三人权益的前提下,应尊重当事人间的真实意思表示,认定债权人为实际权利人。倘若名义权利人将登记系统上所登记的债权及担保物权无权处分给第三人,善意取得规则自有适用空间。在担保物权可得实现之时,原则上债权人和受托人均可行使担保物权。在特殊情形下,担保物权的行使主体可由当事人约定。

关键词：委托代持从属性登记公示无权处分准共有

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善意取得在直播打赏中的适用

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安徽商贸职业技术学院学报 2024年第1期23卷 56-60页

作者：杨艺凡陈旭玲南京理工大学知识产权学院南京210094 安徽师范大学法学院安徽芜湖241000

网络用户因直播打赏行为存在效力瑕疵而请求主播返还打赏时,主播可以利用善意取得制度以维护自身合法利益。在网络直播打赏中,善意取得适用条件的认定与一般交易模式中对其认定有所区别。此外,善意取得的适用有时还会受到公序良俗原则... 详细信息

网络用户因直播打赏行为存在效力瑕疵而请求主播返还打赏时,主播可以利用善意取得制度以维护自身合法利益。在网络直播打赏中,善意取得适用条件的认定与一般交易模式中对其认定有所区别。此外,善意取得的适用有时还会受到公序良俗原则和社会整体效益的影响。

关键词：直播打赏善意取得合理对价无权处分

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A FLASH pipeline for arrayed CRISPR library construction and the gene function discovery of rice receptor-like kinases

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Molecular Plant 2022年第2期15卷 243-257页

作者： Kaiyuan chen Runnan Ke Manman Du Yuqing Yi Yache chen Xiaochun Wang Lu Yao Hao Liu Xin Hou Lizhong Xiong Yinong Yang Kabin Xie National Key Laboratory of Crop Genetic Improvement and National Center of Plant Gene Research(Wuhan) Hubei Hongshan LaboratoryHuazhong Agricultural UniversityNo.1 Shizishan StreetHongshan DistrictWuhan 430070China Hubei Key Laboratory of Plant Pathology Huazhong Agricultural UniversityWuhan 430070China State Key Laboratory of Hybrid Rice College of Life SciencesWuhan UniversityWuhan 430072China Department of Plant Pathology and Environmental Microbiology The Huck Institutes of Life SciencesThe Pennsylvania State UniversityUniversity ParkPA 16802USA Shenzhen Institute of Nutrition and Health Huazhong Agricultural UniversityWuhan 430070China

Clustered regularly interspaced short palindromic repeats(CRISPR)-CRISPR-associated protein 9(Cas9)-mediated gene editing is revolutionizing plant research and crop ***,we present an effective and streamlined pipeline for arrayed CRISPR library construction and demonstrate it is suitable for small-to large-scale genome editing in *** pipeline introduces artificial PCR fragment-length markers for distinguishing guide RNAs(gRNAs)(FLASH),and a group of 12 constructs harboring different FLASH tags are co-transformed into plants each *** identities of gRNAs in Agrobacterium mixtures and trans-genic plants can therefore be read out by detecting the FLASH tags,a process that requires only conven-tional PCR and gel electrophoresis rather than *** generated an arrayed CRISPR library target-ing all 1,072 members of the receptor-like kinase(RLK)family in ***-shot transformation generated a mutant population that covers gRNAs targeting 955 RLKs,and 74.3%(710/955)of the target genes had three or more independent T0 *** results indicate that the FLASH tags act as bona.fide surrogates for the gRNAs and are tightly(92.1%)associated with frameshift mutations in the target *** addition,the FLASH pipeline allows for rapid identification of unintended editing events without corresponding T-DNA integrations and generates high-order mutants of closely related RLK ***,we showed that the RLK mutant library enables rapid discovery of defense-related RLK *** study introduces an effective pipeline for arrayed CRISPR library construction and provides genome-wide rice RLK mutant resources for functional genomics.

关键词： CRISPR library gene editing high throughput receptor-like kinaserice

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IFT140^(+)/K14^(+) cells function as stem/progenitor cells in salivary glands

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International Journal of Oral Science 2022年第4期14卷 561-571页

作者： Xueming Zhang Ji Zhou Xinyu Wang Jiangyu Geng Yubei chen Yao Sun Department of Oral and Maxillofacial Surgery Stomatological Hospital and Dental School of Tongji UniversityShanghai Engineering Research Center of Tooth Restoration and RegenerationNo.399YanChang Middle RoadShanghaiChina Department of Implantology Stomatological Hospital and Dental School of Tongji UniversityShanghai Engineering Research Center of Tooth Restoration and RegenerationNo.399YanChang Middle RoadShanghaiChina

Stem/progenitor cells are important for salivary gland development, homeostasis maintenance, and regeneration following ***-14^(+)(K14^(+)) cells have been recognized as bona.fide salivary gland stem/progenitor cells. However, K14 is also expressed in terminally differentiated myoepithelial cells;therefore, more accurate molecular markers for identifying salivary stem/progenitor cells are required. The intraflagellar transport(IFT) protein IFT140 is a core component of the IFT system that functions in signaling transduction through the primary cilia. It is reportedly expressed in mesenchymal stem cells and plays a role in bone formation. In this study, we demonstrated that IFT140 was intensively expressed in K14^(+)stem/progenitor cells during the developmental period and early regeneration stage following ligation-induced injuries in murine submandibular glands. In addition, we demonstrated that IFT140^(+)/K14^(+)could self-renew and differentiate into granular duct cells at the developmental stage in vivo. The conditional deletion of Ift140 from K14^(+)cells caused abnormal epithelial structure and function during salivary gland development and inhibited regeneration. IFT140 partly coordinated the function of K14^(+)stem/progenitor cells by modulating ciliary membrane trafficking. Our investigation identified a combined marker, IFT140^(+)/K14^(+), for salivary gland stem/progenitor cells and elucidated the essential role of IFT140 and cilia in regulating salivary stem/progenitor cell differentiation and gland regeneration.

关键词： salivary progenitor injuries

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