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Clinical and molecular characterization of 10 Chinese children with congenital adrenal hyperplasia due to 11beta‑hydroxylase deficiency

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World Journal of Pediatrics 2024年第4期20卷 422-433页

作者： Wen‑li Lu Xiao‑Yu Ma Jiao Zhang Jun‑Qi Wang Ting‑Ting Zhang Lei Ye Yuan Xiao Zhi‑Ya Dong Wei Wang Shou‑Yue Sun chuan‑yin li Rong‑Gui Hu Guang Ning li‑Dan Zhang Department of Pediatrics Ruijin HospitalShanghai Jiao Tong University School of MedicineNo.197Ruijin 2nd RdHuangpu DistrictShanghai 200025China Department of Pediatric Genetic and Metabolic Endocrinology West China Second University HospitalSichuan UniversityNo.20Section 3Renmin South RoadSichuan 610041China Department of Endocrine and Metabolic Diseases Shanghai Clinical Center for Endocrine and Metabolic DiseasesRuijin HospitalShanghai Jiao Tong University School of MedicineShanghai 200025China Cancer Center School of MedicineShanghai Tenth People’s HospitalTongji UniversityYanchang Zhong Lu 301St RdJing’an DistrictShanghai 200031China

Background The clinical manifestations of nonclassical 11beta-hydroxylase deficiency are very similar to those of nonclassical 21-hydroxylase *** this study,we investigated the relationship between the clinical and molecular features of congenital adrenal hyperplasia caused by 11beta-hydroxylase deficiency and reviewed the related literature,which are expected to provide assistance for the clinical diagnosis and analysis of congenital adrenal *** Clinical data for 10 Chinese patients diagnosed with congenital adrenal hyperplasia in our hospital from 2018 to 2022 were retrospectively *** examined the effects of gene mutations on protease activity and constructed threedimensional structure prediction models of *** We describe 10 patients with 11beta-hydroxylase gene mutations(n=5,46,XY;n=5,46,XX),with 10 novel mutations were *** patients received treatment at an early stage,with an average age of 2.08±1.66 years,whereas male patients received treatment significantly later,at an average age of 9.77±3.62 *** most common CYP11B1 pathogenic variant in the Chinese population was found to be c.1360C>*** mutations lead to spatial conformational changes that affect protein *** Our study found that there was no significant correlation between each specific mutation and the severity of clinical *** patients with the same gene pathogenic variant may have mild or severe clinical *** correlation between genotype and phenotype needs further ***-dimensional protein simulations may provide additional support for the physiopathological mechanism of genetic mutations.

关键词： 11β-hydroxylase deficiency CYP11B1 Enzymatic activity Phenotype-genotype correlation Threedimensional protein simulations

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Immediate implantation of ultrafine fiber slow-release system based on cell electrospinning to induce osteogenesis of mesenchymal stem cells

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Regenerative Biomaterials 2024年第2期3卷 76-87页

作者： Tao Lu Long Yang Zhuoyang li yin liu Shun'en Xu chuan Ye Department of Orthopaedics The Affliated Hospital of Guizhou Medical UniversityGuiyang 550004China Department of Orthopaedics The First People's Hospital of GuiyangGuiyang 550004China Center for Tissue Engineering and Stem Cell Research Guizhou Medical UniversityGuiyang 550004China Department of Otolaryngology Head and Neck Surgery Xinqiao HospitalArmy Medical University(Third Military Medical University)Chongqing 400037China Department of Dental implant Stomatological Hospital of GuiyangGuiyang 550000China

This study presents the development and evaluation of a poly(3-hydroxybutyrate-co-4-hydroxybutyrate)(P34HB)ultrafine fiber slow-release system for in vivo osteogenic induction of human umbilical cord mesenchymal stem cells(HUCMSCs).Utilizing dual-nozzle and cell electrospinning techniques,the system encapsulates L-ascorbic acid-2-phosphate magnesium(AsP),βB-glycerophosphate sodium and dexamethasone(DEX)within the'fbers,ensuring sustained osteogenic *** scaffold's morphology,characterization,hydrophilicity,mechanical properties and cellular behavior were *** subcutaneous implantation in rabbits was con-ducted to observe its ectopic osteogenic induction *** fabricated P34HB ultrafine fiber slow-release *** ization *** distribution of HUCMSCs and inducing components within the scaffold,with no chemical reactions affecting the active *** vitro tests showcased a prolonged release of DEX and ASP,while biocompatibility assays highlighted the scaffold's suitability for cellular *** Red,type I collagen,and osteopontin(OPN)staining verifed the scaffold's potent osteogenic induction effect on ***,immediate implantation into New Zealand White rabbits led to significant new bone formation within 8 *** findings underscore the system's potential for immediate in vivo implantation without prior in vitro induction,marking a promising advancement in bone tissue engineering.

关键词： bone tissue engineering cell electrospinning in vivo induction immediate implantation biomaterials

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TRIM72‑mediated degradation of the short form of p62/SQSTM1 rheostatically controls selective autophagy in human cells

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军事医学研究:英文版 2023年第4期10卷 559-562页

作者： Cheng‑Cheng Wang Hong Peng Zi Wang Jiao Yang Rong‑Gui Hu chuan‑yin li Wu‑Jun Geng Cancer Center Shanghai Tenth People’s HospitalSchool of MedicineTongji UniversityShanghai 200040China Guizhou University School of Medicine Guiyang 550025China MOE Key Laboratory of Tropical Disease Control Centre for Infection and Immunity Study(CIIS)School of MedicineSun Yat-Sen UniversityShenzhen 510275GuangdongChina Institute for Frontierlife and Medical Sciences Kyoto UniversityKyoto 606‑8501Japan State Key Laboratory of Molecular Biology Shanghai Institute of Biochemistry and Cell BiologyCenter for Excellence in Molecular Cell ScienceChinese Academy of SciencesShanghai 200031China Department of Anesthesiology Wenzhou Key Laboratory of Perioperative Medicinethe First Affiliated Hospital of Wenzhou Medical UniversityWenzhou 325000ZhejiangChina

Dear Editor,Autophagy is an evolutionarily conserved catabolic process that involves the sequestration and transport of organelles,macromolecules,or invading microorganisms to lysosomes for degradation[1].Sequestosome 1(p62/SQSTM1)was the first protein shown to bind target-associated ubiquitin(Ub)and LC3 conjugated to the phagophore membrane,thus,acting as an important autophagy receptor for ubiquitinated targets[2].

关键词： p62S Alternative splicing Ubiquitination TRIM72 Autophagy

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Alkyl Radical Initiated Cyclization/Cascade for Synthesizing Lactam-Substituted Alkyl Sulfones

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Chinese Journal of Chemistry 2024年第16期42卷 1853-1859页

作者： li-Jun Wu Kai-Yi Zhang Pei Yang chuan-Chong Peng Jin-Hui liu Qing li Da-Zhi Sun Shuangfeng yin College of Sciences Central South University of Forestry and TechnologyChangshaHunan410004 China School of Chemistry and Chemical Engineering Inner Mongolia UniversityHohhotInner Mongolia010021 China

An alkyl radical initiated cyclization/tandem reaction of alkyl bromides and alkyl electrophiles by using potassium metabisulphite(K_(2)S_(2)O_(5))as a connector is developed for the synthesis of various lactam-substituted alkyl ***,this process does not require a metal catalyst or metal powder reductant,highlighting its environmentally friendly *** reaction demonstrates outstanding substrate adaptability and a high tolerance towards diverse functional ***,the biologically active molecules and commercially available drugs with a late-stage modification are also highly compatible with this *** studies revealed that the reaction proceeds through a single-step process involving intramolecular radical cyclization,"SO_(2)"insertion,and external alkyl incorporation.

关键词： Alkyl radical Metal-free catalyzed Cyclization/cascade Potassium metabisulphite Electrophiles Alkyl sulfones Lactams Alkyl bromides Sodium formate

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适于机载环境对地目标跟踪的粒子滤波设计

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光学精密工程 2014年第4期22卷 1037-1047页

作者：宋策张葆尹传历中国科学院长春光学精密机械与物理研究所中国科学院航空光学成像与测量重点实验室吉林长春130033 中国科学院大学北京100039

为提高机载环境对地面强机动性目标跟踪的鲁棒性,本文以粒子滤波为跟踪框架,研究了它的动态模型与观测模型。针对机载环境的特点与跟踪目标的强机动性,提出了基于Kristan双步动态模型结构的加速度双步动态模型(TSA)。根据Yilmaz等人提... 详细信息

为提高机载环境对地面强机动性目标跟踪的鲁棒性,本文以粒子滤波为跟踪框架,研究了它的动态模型与观测模型。针对机载环境的特点与跟踪目标的强机动性,提出了基于Kristan双步动态模型结构的加速度双步动态模型(TSA)。根据Yilmaz等人提出的非对称核函数思想,针对实际工程中目标变化特点与实时性要求,提出利用Snake算法提取目标轮廓,以轮廓信息构造非对称核函数的方法。最后,依据上述方法提出了TSA-AK粒子滤波跟踪算法。利用提出的算法对机载环境对地目标跟踪的视频进行了测试,结果表明,本文算法可实现对大幅度变速运动目标的稳定跟踪,正确跟踪率为98%;对大小为25pixel×30pixel的目标的处理帧率为26frame/s。

关键词：目标跟踪粒子滤波动态模型核函数 Snake算法

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Trace formula of the integro-differential operator on a quantum graph

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Applied Mathematics(A Journal of Chinese Universities) 2024年第2期39卷 311-318页

作者： WEI li-xiao YANG chuan-fu School of Mathematics and Statistics Nanjing University of Science and TechnologyNanjing 210094China.

In this paper we study the eigenvalue problem for integro-differential operators on a lasso *** trace formula of the operator is established by applying the residual technique in complex analysis.

关键词： integro-differential operators eigenvalue trace formula

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Physiological and pathological functions of circular RNAs in the nervous system

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Neural Regeneration Research 2024年第2期19卷 342-349页

作者： Min Zhou Shi li chuan Huang School of life Sciences Chongqing UniversityChongqingChina

Circular RNAs(circRNAs)are a class of covalently closed single-stranded RNAs that are expressed during the development of specific cells and *** play crucial roles in physiological and pathological processes by sponging microRNAs,modulating gene transcription,controlling the activity of certain RNA-binding proteins,and producing functional peptides.A key focus of research at present is the functionality of circRNAs in the nervous system and several advances have emerged over the last 2 ***,the precise role of circRNAs in the nervous system has yet to be comprehensively *** this review,we first summarize the recently described roles of circRNAs in brain development,maturity,and ***,we focus on the involvement of circRNAs in various diseases of the central nervous system,such as brain cancer,chronic neurodegenerative diseases,acute injuries of the nervous system,and neuropathic pain.A better understanding of the functionality of circRNAs will help us to develop potential diagnostic,prognostic,and therapeutic strategies to treat diseases of the nervous system.

关键词： Alzheimer’s disease amyotrophic lateral sclerosis brain development circRNAs neuropathic pain Parkinson’s disease

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A Deep Mantle Source for the Late Neoarchean Metamorphosed Basalts in Eastern Hebei,North China Craton:Insights from Whole-Rock Geochemistry and Sm-Nd Isotopes,and Zircon U-Pb-Hf Isotopes

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Journal of Earth Science 2024年第1期35卷 29-40页

作者： Zhuang li Chunjing Wei chuan Yang Xi Zhang College of Geosciences China University of PetroleumBeijing 102249China MOE Key Laboratory of Orogenic Belts and Crustal Evolution School of Earth and Space SciencesPeking UniversityBeijing 100871China China Transport Telecommunications and Information Center Beijing 100011China

The basalts within the greenstone belt worldwide serve as an ideal target to decipher the nature of Archean mantle sources and further to extend the understanding of the early stages of Earth's *** provide important insights into the issues,we carried out a detailed investigation of whole-rock geochemistry and Sm-Nd isotopes,and zircon U-Pb-Hf isotopes for the Late Neoarchean metamorphosed basalts in eastern Hebei,North China Craton.U-Pb isotopic dating using the LA-ICPMS on zircons reveals that the basalts in eastern Hebei erupted at ca.2.48-2.51 Ga and subsequently experienced multiple regional metamorphic events at 2477 and 1798 Ma,*** metamorphosed basalts are featured by low SiO_(2),MgO,K_(2)O+Na_(2)O,and high Fe O contents,endowed with the subalkaline and high-Fe tholeiitic *** radiogenic initial Nd and Hf isotope values and correlations among V,Ni and Cr contents strongly imply that the basalts experienced significant clinopyroxene and olivine fractionation and minor crustal contamination during magma *** are also characterized by the relatively low total REE contents and exhibit significant depletions to moderate enrichments in the LREE contents,indicating the derivation from a deep mantle source in an Archean proto-mantle plume setting.

关键词： zircon U-Pb-Hf isotopes geochemistry Late Neoarchean basaltic magmatism North China Craton Archean proto-mantle plume

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Cambrian integrative stratigraphy,biotas,and paleogeographical evolution of the Qinghai-Tibetan Plateau and its surrounding areas

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Science China Earth Sciences 2024年第4期67卷 950-970页

作者： Zhixin SUN Lang SUN Fangchen ZHAO Bing PAN Malik Muhammad Saud Sajid KHAN Shehryar AHMED chuan YANG Lanyun MIAO Zongjun yin Guoxiang li Maoyan ZHU State Key Laboratory of Palaeobiology and Stratigraphy Nanjing Institute of Geology and PalaeontologyChinese Academy of SciencesNanjing 210008China College of Earth and Planetary Sciences University of Chinese Academy of SciencesBeijing 100049China

The Qinghai-Tibetan Plateau and its surrounding areas have a long and complex tectonic evolutionary *** and blocks,such as northern India,Lhasa,Qiangtang,Qaidam and Central Qilian,and their in-between orogenic belts constitute the main part of the Qinghai-Tibetan *** the Cambrian Period,most of these cratons and blocks were on the northwestern periphery of Gondwana,and were associated with the surrounding blocks,***,Central Iran,Afghanistan,Tarim,Alxa,North China,South China and Sibumasu through the Proto-Tethys *** Cambrian stratigraphic sequences on these stable blocks are composed of mixed siliciclastic and carbonate rocks deposited in the shallow-water marine environments,and contain the trilobite assemblages of shelf *** Cambrian stratigraphic sequences in the Qilian tectonic belts,however,are characterized by the intermediate-basic igneous rocks and silicates formed in the Proto-Tethys Ocean,and contain the trilobite assemblages of deep-water slope *** with previous data,field observations and newly discovered fossils through funding by the Second Tibetan Plateau Scientific Expedition and Research,the general characteristics of the Cambrian strata in different tectonic units of the Qinghai-Tibetan Plateau and its surrounding areas have been summarized in this ***,efforts have been made to subdivide and correlate the Cambrian strata across these areas by utilizing available biostratigraphic and geochronological *** a result,a comprehensive litho-and biostratigraphy chart has been ***,from the biogeographic perspective,this paper also provides a brief overview of the Cambrian paleogeographical reconstruction of the major tectonic blocks,and discusses the problems associated with the evolution of the ProtoTethys tectonic belt.

关键词： Qinghai-Tibetan Plateau Cambrian Stratigraphy Paleogeography Biota Gondwana Proto-Tethys Ocean

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Migration and distribution characteristics of soil heavy metal(loid)s at a lead smelting site

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Journal of Environmental Sciences 2024年第1期135卷 600-609页

作者： Chuxuan li Mu li Jiaqing Zeng Shanxin Yuan Xinghua Luo chuan Wu Shengguo Xue School of Metallurgy and Environment Central South UniversityChangsha 410083China Chinese National Engineering Research Center for Control and Treatment of Heavy Metal Pollution Central South UniversityChangsha 410083China

Heavy metal(loid)s contamination is a constant issue at smelting *** is essential to investigate the spatial distribution and migration characteristics of heavy metal(loid)s in the soil for environmental management and remediation strategies of non-ferrous smelting *** this study,203 soil samples from 57 sites were collected in a typical lead smelting *** findings demonstrated that there were significant Pb,Zn,Cd,and As contamination in soil *** spatial distribution of heavy metal(loid)s showed strong spatial heterogeneity,the contaminated soil areas of Pb,As,Cd,and Zn were 99.5%,98.9%,85.3%,and 72.4%,***,Cd,and As contamination of the soil reached a depth of 5 m,which migrated from the surface to deep soil *** leaching contents of Zn,Pb,and As decreased obviously in 3-4 m soil layer,but the leaching content of Cd was still high,which indicated the high migration of *** the increase of depth,the proportion of acid soluble fraction of heavy metal(loid)s decreased,and the residual fraction *** acid soluble fraction of Cd accounted for a higher proportion,and As mainly existed in reducible and residual fractions in *** to the calculation of the migration factor,the migration of heavy metal(loid)s in soils were ordered as Cd>Zn>Pb>*** outcomes are advantageous for risk reduction and site remediation for non-ferrous metal smelting sites.

关键词： Smelting sites Heavy metal(loid)s Spatial distribution Chemical speciation Migration characteristics

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