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Gene therapy for spinal muscular atrophy:perspectives on the possibility of optimizing SMN1 delivery to correct all neurological and systemic perturbations

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Neural Regeneration Research 2025年第7期20卷 2011-2012页

作者： Sharon J.Brown Rafael J.Yáñez-muñoz Heidi R.Fuller School of Pharmacy and Bioengineering Keele UniversityKeeleUK Wolfson Center for Inherited Neuromuscular Disease TORCH BuildingRJAH Orthopaedic HospitalOswestryUK *** Center of Gene and Cell TherapyDepartment of Biological SciencesSchool of Life Sciences and the EnvironmentRoyal Holloway University of LondonEghamUK

Spinal muscular atrophy(SMA)is a genetic condition that results in selective lower motor neuron loss with concomitant muscle weakness and *** genetic cause of SMA was understood in 1995 when loss or impairment of the survival motor neuron 1(SMN1)gene was identified as the main contributing factor(Lefebvre et al.,1995).This,in combination with the discovery that humans have a“back-up”gene,SMN2,which can produce low levels(approximately 10%)of the full-length functional SMN protein,has led to the generation of SMA-specific gene *** was traditionally classified according to age of symptom onset and developmental milestones achieved,with life expectancy and severity varying between ***,SMN2 copy number is used as a proxy for the prediction of disease severity,with higher SMN2 copy number typically being associated with reduced severity of SMA,although this relationship is not absolute:some individuals with low SMN2 copy number have less severe SMA phenotypes and vice ***,the etiology of SMA is further complicated by other factors,such as non-typical nucleotide variants and SMN2-independent modifiers of disease severity.

关键词： atrophy traditionally absolute

Pancreatic stent improves the success rate of needle-knife papillotomy in patients with difficult biliary cannulation

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World Journal of Gastroenterology 2025年第1期31卷 58-67页

作者： mu-Hsien Lee Cheng-Hui Lin Chi-Huan Wu Yung-Kuan Tsou Kai-Feng Sung Sheng-Fu Wang Nai-Jen Liu Department of Gastroenterology and Hepatology Chang Gung Memorial Hospital and Chang Gung University College of MedicineTaoyuan 33305Taiwan School of Medicine Chang Gung UniversityTaoyuan 33305Taiwan

BACKGROUND Needle-knife precut papillotomy(NKP)is typically performed ***,it remains unclear whether pancreatic stent(PS)placement can improve the outcomes of *** To explore whether PS placement improves the success rate of NKP in patients with difficult biliary *** This single-center retrospective study included 190 patients who underwent NKP between January 2017 and December 2021 after failed conventional biliary *** cases with incidental pancreatic duct cannulation during conventional biliary cannulation,the decision for pre-NKP PS placement was made at the endoscopist's *** primary outcome was the difference in the NKP success rate between patients with and without PS placement;the secondary outcome was the adverse event *** Among the 190 participants,82 received pre-NKP PS(PS-NKP group)whereas 108 did not[freehand or freehand NKP(FH-NKP)group].Post-NKP selective biliary cannulation was successful in 167(87.9%)patients,and the PS-NKP had a significantly higher success rate than the FH-NKP group(93.9%vs 83.3%,P=0.027).The overall adverse event rates were 7.3%and 11.1%in the PS-NKP and FH-NKP groups,respectively(P=0.493).A periampullary diverticulum(PAD)and significant intraoperative bleeding during NKP were independently associated with NKP failure;however,a pre-NKP PS was the only predictor of NKP *** the 44 participants with PADs,the PS-NKP group had a non-significantly higher NKP success rate than the FH-NKP group(87.5%and 65%,respectively;P=0.076).CONCLUSION PS significantly improved the success rate of NKP in patients with difficult biliary cannulation.

关键词： Difficult biliary cannulation Endoscopic retrograde cholangiopancreatography Needle-knife papillotomy Pancreatic stent Selective biliary cannulation

Müller cells are activated in response to retinal outer nuclear layer degeneration in rats subjected to simulated weightlessness conditions

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Neural Regeneration Research 2025年第7期20卷 2116-2128页

作者： Yuxue mu Ning Zhang Dongyu Wei Guoqing Yang Lilingxuan Yao Xinyue Xu Yang Li Junhui Xue Zuoming Zhang Tao Chen Aerospace Clinical Medical Center School of Aerospace MedicineAir Force Medical UniversityXi’anShaanxi ProvinceChina Department of Aviation Medicine Xijing HospitalAir Force Medical UniversityXi’anShaanxi ProvinceChina Department of Emergency Medicine Wuhan No.1 HospitalWuhanHubei ProvinceChina Third Regiment School of Basic MedicineAir Force Medical UniversityXi’anShaanxi ProvinceChina Fourth Regiment School of Basic MedicineAir Force Medical UniversityXi’anShaanxi ProvinceChina

A microgravity environment has been shown to cause ocular damage and affect visual acuity,but the underlying mechanisms remain ***,we established an animal model of weightlessness via tail suspension to examine the pathological changes and molecular mechanisms of retinal damage under *** 4 weeks of tail suspension,there were no notable alterations in retinal function and morphology,while after 8 weeks of tail suspension,significant reductions in retinal function were observed,and the outer nuclear layer was thinner,with abundant apoptotic *** investigate the mechanism underlying the degenerative changes that occurred in the outer nuclear layer of the retina,proteomics was used to analyze differentially expressed proteins in rat retinas after 8 weeks of tail *** results showed that the expression levels of fibroblast growth factor 2(also known as basic fibroblast growth factor)and glial fibrillary acidic protein,which are closely related to Müller cell activation,were significantly *** addition,Müller cell regeneration and Müller cell gliosis were observed after 4 and 8 weeks,respectively,of simulated *** findings indicate that Müller cells play an important regulatory role in retinal outer nuclear layer degeneration during weightlessness.

关键词： glial fibrous acidic protein gliosis Müller cells nerve growth factor neural differentiation neurodegeneration proteomic retinal degeneration retinal outer nuclear layer simulated weightlessness

EZH2-dependent myelination following sciatic nerve injury

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Neural Regeneration Research 2025年第8期20卷 2382-2394页

作者： Hui Zhu Li mu Xi Xu Tianyi Huang Ying Wang Siyuan Xu Yiting Wang Wencong Wang Zhiping Wang Hongkui Wang Chengbin Xue Research Center of Clinical Medicine Affiliated Hospital of Nantong UniversityKey Laboratory of Neuroregeneration of Jiangsu and Ministry of EducationCo-innovation Center of NeuroregenerationNMPA Key Laboratory for Research and Evaluation of Tissue Engineering Technology ProductsNantong UniversityNantongJiangsu ProvinceChina Medical School of Nantong University NantongJiangsu ProvinceChina Department of Neurology Affiliated Hospital of Nantong UniversityNantongJiangsu ProvinceChina Department of Rehabilitation Medicine Affiliated Hospital of Nantong UniversityNantongJiangsu ProvinceChina Department of Critical Care Medicine Nantong Fourth People’s HospitalNantongJiangsu ProvinceChina

Demyelination and remyelination have been major focal points in the study of peripheral nerve regeneration following peripheral nerve ***,the gene regulatory network of regenerated myelin differs from that of native *** of enhancer of zeste homolog 2(EZH2)hinders the differentiation,maturation,and myelination of Schwann cells in *** further determine the role of EZH2 in myelination and recovery post-peripheral nerve injury,conditional knockout mice lacking Ezh2 in Schwann cells(Ezh2^(fl/fl);Dhh-Cre and Ezh2^(fl/fl);Mpz-Cre)were *** results show that a significant proportion of axons in the sciatic nerve of Ezh2-depleted mice remain *** highlights the crucial role of Ezh2 in initiating Schwann cell ***,we observed that 21 days after inducing a sciatic nerve crush injury in these mice,most axons had remyelinated at the injury site in the control nerve,while Ezh2^(fl/fl);Mpz-Cre mice had significantly fewer remyelinated axons compared with their wild-type *** suggests that the absence of Ezh2 in Schwann cells impairs myelin formation and *** conclusion,EZH2 has emerged as a pivotal regulatory factor in the process of demyelination and myelin regeneration following peripheral nerve *** EZH2 activity during these processes may offer a promising therapeutic target for the treatment of peripheral nerve injuries.

关键词： demyelination EZH2 myelination peripheral nerve injury PRC2 remyelination Schwann cells sciatic nerve crush sciatic nerve transection

CNKSR2 interactome analysis indicates its association with the centrosome/microtubule system

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Neural Regeneration Research 2025年第8期20卷 2420-2432页

作者： Lin Yin Yalan Xu Jie mu Yu Leng Lei Ma Yu Zheng Ruizhi Li Yin Wang Peifeng Li Hai Zhu Dong Wang Jing Li Institute for Translational Medicine The Affiliated Hospital of Qingdao UniversityMedical CollegeQingdao UniversityQingdaoShandong ProvinceChina School of Basic Medicine Qingdao UniversityQingdaoShandong ProvinceChina College of Life Sciences and School of PharmacyMedical CollegeQingdao UniversityQingdaoShandong ProvinceChina Department of Urology Qingdao Municipal Hospital Affiliated to Qingdao UniversityQingdaoShandong ProvinceChina

The protein connector enhancer of kinase suppressor of Ras 2(CNKSR2),present in both the postsynaptic density and cytoplasm of neurons,is a scaffolding protein with several protein-binding *** of the CNKSR2 gene have been implicated in neurodevelopmental disorders,particularly intellectual disability,although the precise mechanism involved has not yet been fully *** has demonstrated that CNKSR2 plays a role in facilitating the localization of postsynaptic density protein complexes to the membrane,thereby influencing synaptic signaling and the morphogenesis of dendritic ***,the function of CNKSR2 in the cytoplasm remains to be *** this study,we used immunoprecipitation and high-resolution liquid chromatography-mass spectrometry to identify the interactors of *** a combination of bioinformatic analysis and cytological experiments,we found that the CNKSR2 interactors were significantly enriched in the proteome of the *** also showed that CNKSR2 interacted with the microtubule protein DYNC1H1 and with the centrosome marker *** colocalization analysis confirmed the centrosomal localization of *** we downregulated CNKSR2 expression in mouse neuroblastoma cells(Neuro 2A),we observed significant changes in the expression of numerous centrosomal *** manipulation also affected centrosome-related functions,including cell size and shape,cell proliferation,and ***,we found that CNKSR2 interactors were highly enriched in de novo variants associated with intellectual disability and autism spectrum *** findings establish a connection between CNKSR2 and the centrosome,and offer new insights into the underlying mechanisms of neurodevelopmental disorders.

关键词： autism spectrum disorder centrosome CNKSR2 intellectual disability interactome mass spectrometry microtubule neurodevelopmental disease protein complexes protein-protein interactions

Increasingly important role of numerical modeling in oceanic observation design strategy: A review

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Science China Earth Sciences 2020年第11期63卷 1678-1690页

作者： Kun ZHANG mu mu Qiang WANG CAS Key Laboratory of Ocean Circulation and Waves Institute of OceanologyChinese Academy of Sciences and Pilot National Laboratory for Marine Science and Technology(Qingdao)Qingdao 266071China Center for Ocean Mega-Science Chinese Academy of SciencesQingdao 266071China Department of Atmospheric and Oceanic Sciences and Institute of Atmospheric Sciences Fudan UniversityShanghai 200438China CMA-FDU Joint Laboratory of Marine Meteorology Shanghai 200438China Innovation Center of Ocean and Atmosphere System Zhuhai Fudan Innovation Research InstituteZhuhai 518057China Key Laboratory of Marine Hazards Forecasting Ministry of Natural ResourcesHohai UniversityNanjing 210098China College of Oceanography Hohai UniversityNanjing 210098China

Oceanic observation design is of considerable significance and has made remarkable progress during the past several *** study addresses the critical role of numerical modeling in oceanic observation *** a brief introduction of the characteristics of existing oceanic observation design studies,we present the advantages of the model-based observation design strategy and further review its decisive *** demonstrate the effectiveness of this strategy,the targeted observation applications using the conditional nonlinear optimal perturbation(CNOP)approach for improving the Kuroshio predictions are ***,the authors present their consideration for correcting model errors by targeted observations of sensitive model parameters and mitigating the model-dependency problem by utilizing multiple modeling *** on using observing system simulation experiments to validate the designed observations and extending the model-based observation design strategy into observing oceanic climatological mean states are also discussed.

关键词： Observation design Numerical modeling Targeted observation CNOP

A useful approach to sensitivity and predictability studies in geophysical fluid dynamics: conditional non-linear optimal perturbation

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National Science Review 2020年第1期7卷 214-223页

作者： Qiang Wang mu mu Guodong Sun CAS Key Laboratory of Ocean Circulation and Waves Institute of Oceanology Chinese Academy of Sciences Pilot National Laboratory for Marine Science and Technology(Qingdao) LASG Institute of Atmospheric Physics Chinese Academy of Sciences Center for Ocean Mega-Science Chinese Academy of Sciences University of Chinese Academy of Sciences Department of Atmospheric and Oceanic Sciences Institute of Atmospheric Sciences Fudan University

In atmospheric and oceanic studies, it is important to investigate the uncertainty of model solutions. The conditional non-linear optimal perturbation(CNOP) method is useful for addressing the uncertainty. This paper reviews the development of the CNOP method and its computational aspects in recent ***, the CNOP method was first proposed to investigate the effects of the optimal initial perturbation on atmosphere and ocean model results. Then, it was extended to explore the influences of the optimal parameter perturbation, model tendency perturbation and boundary condition perturbation. To obtain solutions to these optimal perturbations, four kinds of optimization approaches were developed: the adjoint-based method, the adjoint-free method, the intelligent optimization method and the unconstrained optimization method. We illustrate the calculation process of each method and its advantages and disadvantages. Then, taking the Zebiak–Cane model as an example, we compare the CNOPs related to initial conditions(CNOP-Is) calculated by the above four methods. It was found that the dominant structures of the CNOP-Is for different methods are similar, although some differences in details ***, we discuss the necessity and possible direction for designing a more effective optimization approach related to the CNOP in the future.

关键词： CNOP non-linear optimization atmosphere ocean

The predictability of atmospheric and oceanic motions:Retrospect and prospects

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Science China Earth Sciences 2017年第11期60卷 2001-2012页

作者： mu mu DUAN WanSuo TANG YouMin Institute of Atmospheric Sciences Fudan UniversityShanghai 200433China Institute of Oceanology Chinese Academy of SciencesQingdao 266071China Institute of Atmospheric Physics Chinese Academy of SciencesBeijing 100029China College of Earth Sciences University of Chinese Academy of SciencesBeijing 100049China Second Institute of Oceanography State Oceanic AdministrationHangzhou 310012China

This paper reviews the historic understanding of the predictability of atmospheric and oceanic motions, and interprets it in a general framework. On this basis, the existing challenges and unsolved problems in the study of the intrinsic predictability limit(IPL) of weather and climate events of different spatio-temporal scales are summarized. Emphasis is also placed on the structure of the initial error and model parameter errors as well as the associated targeting observation issue. Finally, the predictability of atmospheric and oceanic motion in the ensemble-probabilistic methods widely used in current operational forecasts are *** necessity of considering IPLs in the framework of stochastic dynamic systems is also addressed.

关键词： Atmosphere-ocean Predictability Intrinsic predictability limit Ensemble forecast

Influence of the Arctic on the Predictability of Eurasian Winter Extreme Weather Events

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Advances in Atmospheric Sciences 2020年第4期37卷 307-317页

作者： Guokun DAI mu mu Department of Atmospheric and Oceanic Sciences and Institute of Atmospheric Sciences Fudan UniversityShanghai 200438China

The linkage between the Arctic and midlatitudes has received much attention recently due to the rapidly changing *** investigations have been conducted to reveal the relationship between the Arctic and Eurasian extreme events from the perspective of climatological *** a prediction source for extreme events in Eurasia,Arctic conditions are crucial for extreme event ***,it is urgent to explore the Arctic influence on the predictability of Eurasian extreme events due to the large uncertainties in Arctic *** the sensitivity and nonlinearity of the atmospheric circulations in midlatitude to Arctic conditions,it is necessary to investigate the Arctic influences on Eurasian extreme weather events in case studies at weather time *** studies indicate that only perturbations in specific patterns have fast ***,the conditional nonlinear optimal perturbation approach is recommended for exploring the uncertainties in Arctic initial and boundary conditions and their synergistic effect on Eurasian extreme ***,the mechanism for extreme event formation may differ in different ***,more extreme cases should be investigated to reach robust conclusions.

关键词： Arctic uncertainties Eurasian extreme weather events synergistic effect optimization algorithms