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Characterization of two novel knock-in mouse models of syndromic retinal ciliopathy carrying hypomorphic Sdccag8 mutations

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Zoological Research 2022年第3期43卷 442-456页

作者： Zhi-lin Ren Hou-Bin Zhang lin Li zheng-lin yang Li Jiang Department of Laboratory Medicine Sichuan Provincial People's HospitalSchool of MedicineUniversity of Electronic Science and Technology of ChinaChengduSichuan 610072China Sichuan Provincial Key Laboratory for Human Disease Gene Study Sichuan Provincial People's HospitalUniversity of Electronic Science and Technology of ChinaChengduSichuan 610072China Research Unit for Blindness Prevention of Chinese Academy of Medical Sciences(2019RU026) Sichuan Academy of Medical SciencesChengduSichuan 610072China

Mutations in serologically defined colon cancer autoantigen protein 8(SDCCAG8)were first identified in retinal ciliopathy families a decade ago with unknown *** investigate the pathogenesis of SDCCAG8-associated retinal ciliopathies in vivo,we employed CRISPR/Cas9-mediated homology-directed recombination(HDR)to generate two knock-in mouse models,Sdccag8^(Y236X/Y236X) and Sdccag8^(E451GfsX467/E451GfsX467),which carry truncating mutations of the mouse Sdccag8,corresponding to mutations that cause Bardet-Biedl syndrome(BBS)and Senior-L?ken syndrome(SLS)(c.696T>G p.Y232X and c.1339-1340ins G p.E447GfsX463)in humans,*** two mutant Sdccag8 knock-in mice faithfully recapitulated human SDCCAG8-associated BBS phenotypes such as rod-cone dystrophy,cystic renal disorder,polydactyly,infertility,and growth retardation,with varied age of onset and severity depending on the hypomorphic strength of the Sdccag8 *** the best of our knowledge,these knock-in mouse lines are the first BBS mouse models to present with the polydactyly *** phototransduction protein mislocalization was also observed outside the outer segment after initiation of photoreceptor *** cilia were observed in the mutant photoreceptors,renal epithelial cells,and mouse embryonic fibroblasts derived from the knock-in mouse embryos,suggesting that SDCCAG8 plays an essential role in ciliogenesis,and cilium defects are a primary driving force of SDCCAG8-associated retinal ciliopathies.

关键词： SDCCAG8 Primary cilia Retinal ciliopathy Bardet-Biedl syndrome(BBS) Senior-L?ken syndrome(SLS) Nephronophthisis(NPHP) Polydactyly

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Inhibition of mTOR signaling by rapamycin protects photoreceptors from degeneration in rd1 mice

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Zoological Research 2021年第4期42卷 482-486页

作者： Jia-Liang yang Tong-Dan Zou Fang yang zheng-lin yang Hou-Bin Zhang Key Laboratory for Human Disease Gene Study of Sichuan Province and Institute of Laboratory Medicine Sichuan Provincial People’s HospitalUniversity of Electronic Science and Technology of ChinaChengduSichuan 610072China Institute of Chengdu Biology Sichuan Translational Medicine HospitalChinese Academy of SciencesChengduSichuan 610072China Research Unit for Blindness Prevention of Chinese Academy of Medical Sciences(2019RU026) Sichuan Academy of Medical Sciences&Sichuan Provincial People’s HospitalChengduSichuan 610072China

Retinitis pigmentosa(RP)is an inherited retinal degenerative disease that begins with defective rod photoreceptor function,followed by impaired cone function,and complete blindness in its late *** date,however,there is no effective treatment for *** carrying a nonsense mutation in the Pde6b gene,rd1 mice display elevated cGMP in conjunction with higher intracellular Ca2+in their rod photoreceptors,resulting in fast retinal ***2+has been linked to activation of the mammalian target of rapamycin(mTOR)*** mTOR pathway integrates extracellular and intracellular signals to sense the supply of nutrients and plays a central role in regulating protein and lipid synthesis as well as apoptosis and *** the present study,we showed that mTOR and phosphorylated mTOR(p-mTOR,activated form of mTOR)are up-regulated in rd1 photoreceptors at postnatal day 10(P10),a pre-degenerative ***,the downstream effectors of mTOR,such as pS6K and S6K,are also increased,suggesting activation of the mTOR signaling *** administration of rapamycin,a negative regulator of mTOR,inhibits the mTOR pathway in rd1 ***,the progression of retinal degeneration is slower and retinal function is enhanced,possibly mediated by activation of autophagy in the *** together,these results highlight rapamycin as a potential therapeutic avenue for retinal degeneration.

关键词： degeneration rapamycin impaired

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Identification and Gene Mapping of a Novel Mutant supernumerary lodicules(snl) in Rice

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Journal of Integrative Plant Biology 2010年第3期52卷 265-272页

作者： Nan Wang Xian-Chun Sang Yun-Feng Li zheng-lin yang Fang-Ming Zhao Ying-Hua ling zheng-Sheng Zhang Guang-Hua He Rice Research Institute Key Laboratory of Biotechnology and Crop Quality Improvement Ministry of Agriculture Southwest University Chongqing 400715 China

In order to gain a better understanding of rice flower development, a rice flower mutant supernumerary Iodicules （snl）, which was identified from ethyl methane sulfonate （EMS）-treated Jinhui10 （Oryza sativa L. ssp. indica） was used in the present study. In the snl mutant, the palea obtained lemma identity, additional glume-like organs formed, Iodicules increased and elongated, stamens decreased, and a few aberrant carpels formed. These phenotypes suggest that SNL is involved in the entire rice flower development. SNL was mapped between two simple sequence repeat markers RM3512 and RM1342 on chromosome 2, an approximate 800 kb region, and it co-segregated with SSR215. We conclude that SNL is a novel gene involved in flower development in rice. The present study will be useful for further cloning of the SNL gene, which will contribute to the elucidation of rice flower development.

关键词： GENE mapping RICE ETHYL methanesulfonate STAMEN CARPEL

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Diagnosis of the accurate genotype of HKαα carriers in patients with thalassemia using multiplex ligation-dependent probe amplification combined with nested polymerase chain reaction

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Chinese Medical Journal 2020年第10期133卷 1175-1181页

作者： Dong-Mei Chen Shi Ma Xiang-Lan Tang Ji-Yun yang zheng-lin yang Clinical Medical School Southwest Medical UniversityLuzhouSichuan 646000China The Key Laboratory for Human Disease Gene Study of Sichuan Province and Institute of Laboratory Medicine Sichuan Provincial People's HospitalUniversity of Electronic Science and Technology of ChinaChengduSichuan 610072China Research Unit for Blindness Prevention of Chinese Academy of Medical Sciences(2019RU026) Sichuan Academy of Medical SciencesChengduSichuan 610072China Natural Products Research Center Institute of Chengdu BiologySichuan Translational Medicine HospitalChinese Academy of SciencesChengduSichuan 610072China.

Background:Patients carrying the HongKongαα(HKαα)allele and-α3.7/αααanti-4.2 could be misdiagnosed as-α3.7/ααby the current conventional thalassemia detection methods,leading to inaccurate genetic counseling and an incorrect prenatal *** study was aimed to accurately analyze the genotypes of HKααcarriers and-α3.7/ααα***::Samples were collected in our hospital from July 2017 to October ***-four common types of Chinese thalassemia were screened by gap-polymerase chain reaction(Gap-PCR)and reverse dot blot(RDB).Anti-4.2 multiplex-PCR was used to confirm carriers of theαααanti-4.2 duplication with-α3.7 ***-round nested PCR and multiplex ligation-dependent probe amplification(MLPA)were applied to accurately identify and confirm their *** data analysis,we used descriptive statistics and Fisher’s exact ***::Two thousand five hundred and forty-four cases were identified as thalassemia in 5488 peripheral blood *** results showed thatα,β,andαβcompound thalassemia were identified in 1190(46.78%),1286(50.55%),and 68(2.67%)cases,respectively.A total of 227 samples from thalassemia patients were identified as-α3.7/ααby Gap-PCR,and the genotypes of two samples were *** was a difference between Gap-PCR and combined groups(Gap-PCR combined with nested PCR and MLPA)in detecting HKαα(Pling can be provided in the clinic using nested PCR combined

关键词： Thalassemia HongKongαα Nested polymerase chain reaction Multiplex ligation-dependent probe amplification Gene dosage

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Multi-level Evaluation of Load Supply Adequacy for Transmission Network

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Energy and Power Engineering 2013年第4期5卷 728-732页

作者： zheng-lin yang Jian-guo Yao Jian Geng China Electric Power Research Institute Nanjing China

Considering sharp increase of central integration scale of intermittent energy generation, power system couldn’t rely on the determined model based operation schedule to assure its reliable load supply. Then, this article presents a new load supply adequacy evaluation method, which based on continuous security constrained generation optimization, and provides the functional architecture design of online multi-level short term load supply adequacy evaluation system.

关键词： Load Supply Adequacy Operation Schedule Security Analysis Continuous Security Constrained Generation Optimization Unit Commitment Economic Dispatch

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Pyroptosis,ferroptosis,and autophagy in spinal cord injury:regulatory mechanisms and therapeutic targets

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Neural Regeneration Research 2025年第10期20卷 2787-2806页

作者： Qingcong zheng Du Wang Rongjie lin Weihong Xu Department of Spinal Surgery the First Affiliated Hospital of Fujian Medical UniversityFuzhouFujian ProvinceChina Arthritis Clinical and Research Center Peking University People’s HospitalBeijingChina Department of Orthopedic Surgery Fujian Medical University Union HospitalFuzhouFujian ProvinceChina

Regulated cell death is a form of cell death that is actively controlled by *** studies have shown that regulated cell death plays a key role after spinal cord *** and ferroptosis are newly discovered types of regulated cell deaths that have been shown to exacerbate inflammation and lead to cell death in damaged spinal ***,a complex form of cell death that is interconnected with various regulated cell death mechanisms,has garnered significant attention in the study of spinal cord *** injury triggers not only cell death but also cellular survival *** signaling pathways play pivotal roles in influencing the processes of both deterioration and repair in spinal cord injury by regulating pyroptosis,ferroptosis,and ***,this review aims to comprehensively examine the mechanisms underlying regulated cell deaths,the signaling pathways that modulate these mechanisms,and the potential therapeutic targets for spinal cord *** analysis suggests that targeting the common regulatory signaling pathways of different regulated cell deaths could be a promising strategy to promote cell survival and enhance the repair of spinal cord ***,a holistic approach that incorporates multiple regulated cell deaths and their regulatory pathways presents a promising multi-target therapeutic strategy for the management of spinal cord injury.

关键词： autophagy cell death ferroptosis inflammation pathological mechanisms pyroptosis regulated cell death regulatory pathways spinal cord injury therapeutic targets

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Multi boron-doping effects in hard carbon toward enhanced sodium ion storage

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Journal of Energy Chemistry 2025年第1期100卷 730-738页

作者： Peng zheng Wang Zhou Ying Mo Biao zheng Miaomiao Han Qin Zhong Wenwen yang Peng Gao Lezhi yang Jilei Liu College of Materials Science and Engineering Hunan Joint International Laboratory of Advanced Materials and Technology for Clean Energy Hunan Province Key Laboratory for Advanced Carbon Materials and Applied Technology Hunan University Greater Bay Area Institute for Innovation Hunan University Changsha Research Institute of Mining & Metallurgy Co. LTD.

Hard carbon （HC） has been considered as promising anode material for sodium-ion batteries （SIBs）.The optimization of hard carbon’s microstructure and solid electrolyte interface （SEI） property are demonstrated effective in enhancing the Na+storage capability,however,a one-step regulation strategy to achieve simultaneous multi-scale structures optimization is highly ***,we have systematically investigated the effects of boron doping on hard carbon’s microstructure and interface chemistry.A variety of structure characterizations show that appropriate amount of boron doping can increase the size of closed pores via rearrangement of carbon layers with improved graphitization degree,which provides more Na+storage ***-situ Fourier transform infrared spectroscopy/electrochemical impedance spectroscopy （FTIR/EIS） and X-ray photoelectron spectroscopy （XPS） analysis demonstrate the presence of more BC3and less B–C–O structures that result in enhanced ion diffusion kinetics and the formation of inorganic rich and robust SEI,which leads to facilitated charge transfer and excellent rate *** a result,the hard carbon anode with optimized boron doping content exhibits enhanced rate and cycling *** general,this work unravels the critical role of boron doping in optimizing the pore structure,interface chemistry and diffusion kinetics of hard carbon,which enables rational design of sodium-ion battery anode with enhanced Na+storage performance.

关键词： X ray photoelectron spectroscopy

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Synergistic promotion strategy of “dual-site” and “dual-path” to enhance the C–C coupling between CO2 and HCHO driven by photoelectrocatalysis

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Journal of Energy Chemistry 2025年第1期100卷 39-49页

作者： Hongqiang yang Jianfeng zheng Guoyu Wang Yifei Gao Yatao yang Jia Li Xiaojin Han Yan Cui Zhanggen Huang National Key Laboratory of High Efficiency and Low Carbon Utilization of Coal Institute of Coal Chemistry Chinese Academy of Science University of Chinese Academy of Science Shenyang University of Chemical Technology

Photoelectrocatalytic coupling CO2and volatile organic compounds （VOCs） is a promising green strategy for the synergistic conversion of the two carbon-containing resources to *** catalytic efficiency is always at the mercy of chemical inertness of CO2and the competitive hydrogen evolution of ***,a modified g-C3N4/ZnAl-LDH Z-scheme heterojunction catalyst with dual reaction site was rationally designed and precisely *** Faraday efficiency of ethanol reached 68.67%with a corresponding formation rate of 227.3μmol *** revealed by in-situ characterizations and density functional theory calculations,CO2and HCHO were absorbed at Zn site and N site,***,*CO generated from CO2and HCHO was converted to*CH3O and*CHO on the dual-active-site *** detailed reaction mechanism experiments indicated that C–C coupling only occurred between*CO and*CH3O in electrocatalysis *** from the“*CO+*CH3O”path,another“*CO+*CHO”coupling path was also detected in photoelectrocatalytic *** selectivity of ethanol was significantly enhanced due to the synthesis of dual-site catalyst and the dual-path coupling path between CO2and HCHO simultaneously driven by light and electricity.

关键词： Ethanol

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Cholesterol metabolism: physiological versus pathological aspects in intracerebral hemorrhage

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Neural Regeneration Research 2025年第4期20卷 1015-1030页

作者： Ruoyu Huang Qiuyu Pang Lexin zheng Jiaxi lin Hanxi Li lingbo Wan Tao Wang Department of Forensic Science School of Basic Medicine and Biological SciencesSuzhou Medicine College of Soochow UniversitySuzhouJiangsu ProvinceChina

Cholesterol is an important component of plasma membranes and participates in many basic life functions,such as the maintenance of cell membrane stability,the synthesis of steroid hormones,and *** plays a key role in the establishment and maintenance of the central nervous *** brain contains 20%of the whole body’s cholesterol,80%of which is located within myelin.A huge number of processes(e.g.,the sterol regulatory element-binding protein pathway and liver X receptor pathway)participate in the regulation of cholesterol metabolism in the brain via mechanisms that include cholesterol biosynthesis,intracellular transport,and *** brain injuries or diseases involving crosstalk among the processes above can affect normal cholesterol metabolism to induce detrimental ***,we hypothesized that cholesterol-related molecules and pathways can serve as therapeutic targets for central nervous system *** hemorrhage is the most severe hemorrhagic stroke subtype,with high mortality and *** cholesterol levels are associated with the risk of intracerebral ***,secondary pathological changes after intracerebral hemorrhage are associated with cholesterol metabolism dysregulation,such as neuroinflammation,demyelination,and multiple types of programmed cell *** cholesterol accumulation in the brain has been found after intracerebral *** this paper,we review normal cholesterol metabolism in the central nervous system,the mechanisms known to participate in the disturbance of cholesterol metabolism after intracerebral hemorrhage,and the links between cholesterol metabolism and cell *** also review several possible and constructive therapeutic targets identified based on cholesterol metabolism to provide cholesterol-based perspectives and a reference for those interested in the treatment of intracerebral hemorrhage.

关键词： cell death cholesterol metabolism intracerebral hemorrhage myelination therapeutic target

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Hypoxia-preconditioned bone marrow-derived mesenchymal stem cells protect neurons from cardiac arrest-induced pyroptosis

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Neural Regeneration Research 2025年第4期20卷 1103-1123页

作者： Xiahong Tang Nan zheng Qingming lin Yan You zheng Gong yangping Zhuang Jiali Wu Yu Wang Hanlin Huang Jun Ke Feng Chen Shengli Clinical Medical College of Fujian Medical University Fujian Medical UniversityFuzhouFujian ProvinceChina Department of Emergency Fujian Provincial HospitalFuzhouFujian ProvinceChina Fujian Provincial Key Laboratory of Emergency Medicine FuzhouFujian ProvinceChina The Second Department of Intensive Care Unit Fujian Provincial Hospital South BranchFuzhouFujian ProvinceChina

Cardiac arrest can lead to severe neurological impairment as a result of inflammation,mitochondrial dysfunction,and post-cardiopulmonary resuscitation neurological *** preconditioning has been shown to improve migration and survival of bone marrow–derived mesenchymal stem cells and reduce pyroptosis after cardiac arrest,but the specific mechanisms by which hypoxia-preconditioned bone marrow–derived mesenchymal stem cells protect against brain injury after cardiac arrest are *** this end,we established an in vitro co-culture model of bone marrow–derived mesenchymal stem cells and oxygen–glucose deprived primary neurons and found that hypoxic preconditioning enhanced the protective effect of bone marrow stromal stem cells against neuronal pyroptosis,possibly through inhibition of the MAPK and nuclear factor κB ***,we transplanted hypoxia-preconditioned bone marrow–derived mesenchymal stem cells into the lateral ventricle after the return of spontaneous circulation in an 8-minute cardiac arrest rat model induced by *** results showed that hypoxia-preconditioned bone marrow–derived mesenchymal stem cells significantly reduced cardiac arrest–induced neuronal pyroptosis,oxidative stress,and mitochondrial damage,whereas knockdown of the liver isoform of phosphofructokinase in bone marrow–derived mesenchymal stem cells inhibited these *** conclude,hypoxia-preconditioned bone marrow–derived mesenchymal stem cells offer a promising therapeutic approach for neuronal injury following cardiac arrest,and their beneficial effects are potentially associated with increased expression of the liver isoform of phosphofructokinase following hypoxic preconditioning.

关键词： bone marrow–derived mesenchymal stem cells cardiac arrest cardiac resuscitation hypoxic preconditioning liver isoform of phosphofructokinase mitochondria neuroinflammation oxidative stress pyroptosis reactive oxygen species

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